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1. Timmers HJ, Brouwers FM, Hermus AR, Sweep FC, Verhofstad AA, Verbeek AL, Pacak K, Lenders JW: Metastases but not cardiovascular mortality reduces life expectancy following surgical resection of apparently benign pheochromocytoma. Endocr Relat Cancer; 2008 Dec;15(4):1127-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastases but not cardiovascular mortality reduces life expectancy following surgical resection of apparently benign pheochromocytoma.
  • The treatment of choice for non-metastatic pheochromocytoma is surgical resection.
  • Data on long-term mortality and morbidity after pheochromocytoma surgery are limited.
  • We here report a retrospective study on the long-term outcome after surgery for apparently benign pheochromocytoma at the Radboud University Nijmegen Medical Centre.
  • Two patients experienced a benign recurrence. Mean+/-s.d. follow-up was 10.2+/-7.5 (median 9, range 1-38) years.
  • In conclusion, compared with the general population patients have a reduced life expectancy following pheochromocytoma surgery, due to their risk of developing metastatic disease.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Cardiovascular Diseases / mortality. Life Expectancy. Pheochromocytoma / secondary. Pheochromocytoma / surgery

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  • (PMID = 18824558.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Catecholamines
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2. Brouwers FM, Petricoin EF 3rd, Ksinantova L, Breza J, Rajapakse V, Ross S, Johann D, Mannelli M, Shulkin BL, Kvetnansky R, Eisenhofer G, Walther MM, Hitt BA, Conrads TP, Veenstra TD, Mannion DP, Wall MR, Wolfe GM, Fusaro VA, Liotta LA, Pacak K: Low molecular weight proteomic information distinguishes metastatic from benign pheochromocytoma. Endocr Relat Cancer; 2005 Jun;12(2):263-72
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  • [Title] Low molecular weight proteomic information distinguishes metastatic from benign pheochromocytoma.
  • Metastatic lesions occur in up to 36% of patients with pheochromocytoma.
  • Currently there is no way to reliably detect or predict which patients are at risk for metastatic pheochromocytoma.
  • Thus, the discovery of biomarkers that could distinguish patients with benign disease from those with metastatic disease would be of great clinical value.
  • Using surface-enhanced laser desorption ionization protein chips combined with high-resolution mass spectrometry, we tested the hypothesis that pheochromocytoma pathologic states can be reflected as biomarker information within the low molecular weight (LMW) region of the serum proteome.
  • LMW protein profiles were generated from the serum of 67 pheochromocytoma patients from four institutions and analyzed by two different bioinformatics approaches employing pattern recognition algorithms to determine if the LMW component of the circulatory proteome contains potentially useful discriminatory information.
  • Both approaches were able to identify combinations of LMW molecules which could distinguish all metastatic from all benign pheochromocytomas in a separate blinded validation set.
  • In conclusion, for this study set low molecular mass biomarker information correlated with pheochromocytoma pathologic state using blinded validation.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Biomarkers, Tumor / blood. Neoplasm Proteins / blood. Pheochromocytoma / diagnosis. Proteome / analysis
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Molecular Weight. Neoplasm Metastasis. Proteomics

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  • (PMID = 15947101.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins; 0 / Proteome
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3. Ohta S, Lai EW, Pang AL, Brouwers FM, Chan WY, Eisenhofer G, de Krijger R, Ksinantova L, Breza J, Blazicek P, Kvetnansky R, Wesley RA, Pacak K: Downregulation of metastasis suppressor genes in malignant pheochromocytoma. Int J Cancer; 2005 Mar 10;114(1):139-43
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  • [Title] Downregulation of metastasis suppressor genes in malignant pheochromocytoma.
  • There is no reliable method currently available to predict malignant potential of pheochromocytoma based on conventional histology or genetic, molecular or immunohistochemical markers.
  • Metastasis suppressor genes affect the spread of several cancers and, therefore, may provide promise as prognostic markers or therapeutic targets for malignant pheochromocytoma.
  • We hypothesized that the downregulation of metastasis suppressor genes in malignant pheochromocytoma may play a role in malignant behavior.
  • The study included 15 benign and 10 malignant pheochromocytomas.
  • Six metastasis suppressor genes (nm23-H1, TIMP-4, BRMS-1, TXNIP, CRSP-3 and E-Cad) were downregulated significantly in malignant compared to benign pheochromocytoma (p < 0.05, Mann-Whitney U-test).
  • We applied a non-linear rule using median malignant value (MMV) as a threshold to use metastasis suppressor genes to distinguish malignant from benign samples.
  • After cross-validation, the non-linear rule produced no errors in 10 malignant samples and 3 errors in the 15 benign samples, with an overall error rate of 12%.
  • These results suggest that downregulation of metastasis suppressor genes reflect malignant pheochromocytoma with a high degree of sensitivity.
  • Thus, we conclude that altered function of these metastasis suppressor gene pathways may play an important role in the malignant behavior of pheochromocytoma.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Genes, Tumor Suppressor. Pheochromocytoma / metabolism
  • [MeSH-minor] Adrenal Gland Neoplasms / metabolism. Adult. Aged. Cadherins / metabolism. Down-Regulation. Female. Humans. Male. Mediator Complex. Middle Aged. NM23 Nucleoside Diphosphate Kinases. Nucleoside-Diphosphate Kinase / metabolism. Polymerase Chain Reaction. Prognosis. Tissue Inhibitor of Metalloproteinases / metabolism. Trans-Activators / metabolism

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  • (PMID = 15523699.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cadherins; 0 / MED23 protein, human; 0 / Mediator Complex; 0 / NM23 Nucleoside Diphosphate Kinases; 0 / Tissue Inhibitor of Metalloproteinases; 0 / Trans-Activators; 0 / tissue inhibitor of metalloproteinase-4; EC 2.7.4.6 / NME1 protein, human; EC 2.7.4.6 / Nucleoside-Diphosphate Kinase
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4. Koeth O, Mark B, Cornelius B, Senges J, Zeymer U: [Cardiogenic shock after adrenalectomy for pheochromocytoma]. Internist (Berl); 2007 Feb;48(2):189-93
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  • [Title] [Cardiogenic shock after adrenalectomy for pheochromocytoma].
  • A 56 year old patient underwent left-sided adrenalectomy for a benign pheochromocytoma.
  • Catecholamine release during a left-sided adrenalectomy for a benign pheochromocytoma was associated with Takotsubo cardiomyopathy complicated by cardiogenic shock.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Adrenalectomy / adverse effects. Cardiomyopathies / etiology. Pheochromocytoma / complications. Shock, Cardiogenic / etiology


5. Waldmann J, Fendrich V, Holler J, Buchholz M, Heinmöller E, Langer P, Ramaswamy A, Samans B, Walz MK, Rothmund M, Bartsch DK, Slater EP: Microarray analysis reveals differential expression of benign and malignant pheochromocytoma. Endocr Relat Cancer; 2010 Sep;17(3):743-56
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  • [Title] Microarray analysis reveals differential expression of benign and malignant pheochromocytoma.
  • The diagnosis of a malignant pheochromocytoma (PC) can only be established by the presence of distant metastases, but a subset of apparently benign PCs develop metastases.
  • We have employed a microarray analysis to identify a typical gene expression profile which distinguishes malignant from benign PC.
  • Total RNA was isolated from fresh-frozen tissue of five benign and five malignant PCs.
  • The reference consisted of laser microdissected tissue from normal adrenal medulla.
  • The analysis revealed a more than twofold difference in expression between benign and malignant PCs in 132 genes: 19 were up-regulated and 113 were down-regulated.
  • Comparative analysis by microarray of all ten PCs (benign/malignant) versus normal adrenal medulla revealed a more than twofold expression difference in 455/539 and 491/671 genes respectively.
  • Several of these genes are known to participate on adrenal tumorigenesis, potential tumor suppressor genes, and oncogenes.
  • Comprehensive gene expression analysis of malignant and benign PCs revealed different gene profiles, which could be used to discriminate between malignant and benign PCs.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Adrenal Medulla / metabolism. Biomarkers, Tumor / metabolism. Gene Expression Profiling. Pheochromocytoma / metabolism


6. Lai EW, Perera SM, Havekes B, Timmers HJ, Brouwers FM, McElroy B, Adams KT, Ohta S, Wesley RA, Eisenhofer G, Pacak K: Gender-related differences in the clinical presentation of malignant and benign pheochromocytoma. Endocrine; 2008 Aug-Dec;34(1-3):96-100
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  • [Title] Gender-related differences in the clinical presentation of malignant and benign pheochromocytoma.
  • In this study, we evaluated 23 male and 35 female pheochromocytoma patients for symptoms and signs of pheochromocytoma with special regard to gender-related differences in presentation.
  • Subgroup analyses and multiple regression analysis revealed gender differences to be irrespective of benign or malignant disease, use of adrenoceptor-blockade, age and biochemical phenotype.
  • We conclude female patients have significantly more self-reported pheochromocytoma signs and symptoms than male patients irrespective of biochemical phenotype and tumor presentation which may be related to distinct catecholamine receptor sensitivity.
  • Clinicians should be aware of these complaints in female pheochromocytoma patients and offer adequate treatment if indicated.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis. Sex Characteristics

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  • (PMID = 18982461.001).
  • [ISSN] 1355-008X
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers
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7. Lehrfeld T, Natale R, Sharma S, Mendoza PJ, Schwab Ii CW, Lee DI: Robot-assisted excision of a retroperitoneal mass between the left renal artery and vein. JSLS; 2010 Jul-Sep;14(3):447-9
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  • BACKGROUND: Extra-adrenal pheochromocytomas are rare.
  • The pathology demonstrated a benign pheochromocytoma.
  • To our knowledge, this is the first report of a peri-hilar excision of a pheochromocytoma using this approach.
  • CONCLUSION: Extra-adrenal pheochromocytomas are rare and can present in difficult locations.

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  • (PMID = 21333207.001).
  • [ISSN] 1086-8089
  • [Journal-full-title] JSLS : Journal of the Society of Laparoendoscopic Surgeons
  • [ISO-abbreviation] JSLS
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3041050
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8. Opocher G, Schiavi F, Iacobone M, Toniato A, Sattarova S, Erlic Z, Martella M, Mian C, Merante Boschin I, Zambonin L, De Lazzari P, Murgia A, Pelizzo MR, Favia G, Mantero F: Familial nonsyndromic pheochromocytoma. Ann N Y Acad Sci; 2006 Aug;1073:149-55
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  • [Title] Familial nonsyndromic pheochromocytoma.
  • The molecular basis for the familial pheochromocytoma has been largely elucidated and the role of germline mutation of the VHL, RET, SDHB, and SDHD genes has been established.
  • The proband of family 1 is a man who had a bilateral pheochromocytoma at the age of 32 and a local recurrence at the age of 48 years.
  • His brother died of malignant pheochromocytoma and his nephew died suddenly of an undiagnosed pheochromocytoma.
  • The proband of family 2 is a female who had a 5-cm benign adrenal pheochromocytoma at the age of 34 years, while her cousin (maternal branch) had a monolateral pheochromocytoma at the age of 42 years.
  • The proband of family 3 is a female who had a bilateral pheochromocytoma at the age of 66 years.
  • Her sister had a bilateral pheochromocytoma and breast cancer at the age of 54 years.
  • In family 4, the proband was a female who had a bilateral pheochromocytoma at the age of 46 years and a local recurrence a few years later, with liver metastases from the pheochromocytoma.
  • Her brother had a monolateral benign pheochromocytoma.
  • This case revealed a VHL sequence variant IVS2+43 A>G, which was also found in one other unrelated sporadic pheochromocytoma.
  • In family 5, the proband was a female who had a right adrenal pheochromocytoma at the age of 50 years and a breast cancer at 49 years of age.
  • Her mother had had a right adrenal pheochromocytoma at 61 years of age.
  • Although other molecular mechanisms, such as particular variants in untranslated regions or partial gene deletions, cannot be ruled out, we think finding families with nonsyndromic pheochromocytoma without any RET, VHL, SDHB, SDHC, SDHD, or EGLN3 mutation may argue in favor of the presence of other pheochromocytoma susceptibility genes.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics

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  • (PMID = 17102081.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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9. Fariña LA, Antón I, Fernández GC: [Large adrenal pseudocyst mimicking cystic pheochromocytoma: excision minimising laparoscopic access]. Actas Urol Esp; 2009 Sep;33(8):913-6
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  • [Title] [Large adrenal pseudocyst mimicking cystic pheochromocytoma: excision minimising laparoscopic access].
  • [Transliterated title] Seudoquiste adrenal grande que simula feocromocitoma quístico: exéresis minimizando el acceso laparoscópico.
  • INTRODUCTION: Adrenal cysts and pseudocysts are rare and usually benign and asymptomatic; they are discovered either accidentally or during examination for non-specific abdominal discomfort.
  • The differential diagnosis for adrenal cysts must consider cystic adrenal carcinoma and cystic pheochromocytoma, which are both extremely rare, but more severe and difficult to treat.
  • PATIENTS AND RESULTS: A 55 year-old woman reported right-side flank pain; imaging studies discovered a 10 cm cystic adrenal tumour, with radiological indications of cystic pheochromocytoma.
  • Pathological study showed benign pseudocyst.
  • CONCLUSIONS: As less invasive laparoscopic procedures become widespread, an increasing number of case reports and series of cases regarding adrenal cyst operations have recently been reported.
  • A review of the literature shows that the cysts are generally benign, but it is not always possible to identify cystic adrenal carcinoma or cystic pheochromocytoma from the radiology image.
  • [MeSH-major] Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / surgery. Adrenal Gland Neoplasms / diagnosis. Cysts / diagnosis. Cysts / surgery. Laparoscopy. Pheochromocytoma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Middle Aged


10. Bembo SA, Elimian A, Waltzer W, Carlson HE: Pheochromocytoma in a pregnant woman with a history of intracerebral aneurysms. Am J Med Sci; 2005 Jun;329(6):317-9
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  • [Title] Pheochromocytoma in a pregnant woman with a history of intracerebral aneurysms.
  • Pheochromocytoma in pregnancy is rare; if unrecognized, potentially fatal hypertensive crises may occur.
  • Laboratory data revealed elevated 24-hour urinary catecholamine and metanephrine levels, and abdominal sonography showed a 10-cm right adrenal mass.
  • Postpartum abdominal computed tomography scanning confirmed a 10-cm right adrenal mass.
  • A benign pheochromocytoma was removed without incident.
  • This case illustrates the importance of early diagnosis and management of pheochromocytoma in pregnancy and is also an example of the rare association of pheochromocytoma with intracerebral aneurysms.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis. Pregnancy Complications, Neoplastic / diagnosis

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  • (PMID = 15958874.001).
  • [ISSN] 0002-9629
  • [Journal-full-title] The American journal of the medical sciences
  • [ISO-abbreviation] Am. J. Med. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antihypertensive Agents
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11. Bissada NK, Safwat AS, Seyam RM, Al Sobhi S, Hanash KA, Jackson RJ, Sakati N, Bissada MA: Pheochromocytoma in children and adolescents: a clinical spectrum. J Pediatr Surg; 2008 Mar;43(3):540-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma in children and adolescents: a clinical spectrum.
  • PURPOSE: The purpose of the study was to identify the spectrum of disease characteristics of pheochromocytoma in children.
  • METHODS: Records of 21 consecutive children diagnosed with pheochromocytoma were reviewed.
  • Patients included 17 with adrenal and 4 with extra-adrenal tumors including 1 in the urinary bladder.
  • Seventeen had sporadic and 4 had familial pheochromocytoma.
  • Associated manifestations were the predominant features in 1 of the 4 patients with familial pheochromocytoma.
  • The patient with bladder pheochromocytoma presented with gross hematuria.
  • Two patients (1 sporadic and 1 familial) had malignant pheochromocytoma.
  • One patient with benign pheochromocytoma had multiple recurrences in chromaffin-containing sites.
  • One patient with malignant pheochromocytoma died of the disease, and 1 with recurrent pheochromocytoma had neurologic consequences.
  • CONCLUSIONS: Pheochromocytoma in children has unique characteristics.
  • To our knowledge, this series is one of the largest reports of adrenal pheochromocytoma in children.
  • It also reflects the spectrum of pheochromocytoma in this age group.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Pheochromocytoma / diagnosis. Pheochromocytoma / surgery


12. Li Q, Fan Q, Li D, Zhang H: Nonfunctioning benign cardiac pheochromocytoma. J Cancer Res Clin Oncol; 2009 Jan;135(1):103-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Nonfunctioning benign cardiac pheochromocytoma.
  • PURPOSE: Nonfunctioning benign cardiac pheochromocytoma is one of the rarest tumors and only a few cases have been described before.
  • We present a rare case of nonfunctioning benign cardiac pheochromocytoma and a review of the literature with special emphasis on diagnosis and treatment.
  • METHODS: Different from the other cardiac pheochromocytomas, its symptoms and signs are so nonspecific that it is easy to make a misdiagnosis or missed diagnosis.
  • One patient with nonfunctioning benign cardiac pheochromocytoma was treated surgically and relevant cases data were collected.
  • Clinical features, diagnosis and treatment of pheochromocytoma were discussed.
  • The prognosis for patients of benign cardiac pheochromocytomas was excellent, but for those of malignant ones was very poor.
  • CONCLUSION: Reviewing the few reported cases, most nonfunctioning benign cardiac pheochromocytomas can be cured completely by operation with good prognosis.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Heart Neoplasms / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Echocardiography. Humans. Male. Radiography, Thoracic

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  • (PMID = 18563442.001).
  • [ISSN] 0171-5216
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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13. Han XN, Chen B, Ye XD, Wang J, Liu GH: [Clinical manifestation and multiphasic spiral CT scanning features of abdominal pheochromocytoma: report of 70 cases]. Zhonghua Zhong Liu Za Zhi; 2009 Feb;31(2):139-42
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  • [Title] [Clinical manifestation and multiphasic spiral CT scanning features of abdominal pheochromocytoma: report of 70 cases].
  • OBJECTIVE: The purpose of this study was to assess the imaging characteristics of abdominal pheochromocytoma in multiphasic spiral CT scanning, and to determine whether these image characteristics can aid in differentiating pheochromocytoma from other types of tumors or not.
  • RESULTS: Among the 70 patients, there were 41 patients with endocrine symptoms related to pheochromocytoma, 15 had a latent pheochromocytoma and the remaining 14 cases presented with a non-functioning pheochromocytoma.
  • Sixty cases had a tumor located in the adrenal gland, while 8 in retroperitoneal space, and 2 cases had both intraadrenal and ectopic lesions simultaneously.
  • Sixty patients had a benign pheochromocytoma, the other 10 had a malignant one or relapse after operation.
  • However, when a small tumor has moderate blood supply, it should be differentiated with an adrenal adenoma; when a big one has moderate blood supply, it should be differentiated with other malignant tumors.
  • Furthermore, a part of malignant pheochromocytomas is really difficult to be differentiated from some benign lesions by spiral CT images alone.
  • [MeSH-major] Adrenal Gland Neoplasms / radiography. Pheochromocytoma / radiography. Retroperitoneal Neoplasms / radiography. Tomography, Spiral Computed / methods
  • [MeSH-minor] Adenoma / blood supply. Adenoma / radiography. Adenoma / surgery. Adolescent. Adult. Aged. Aged, 80 and over. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Radiographic Image Enhancement. Retrospective Studies. Young Adult

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  • (PMID = 19538893.001).
  • [ISSN] 0253-3766
  • [Journal-full-title] Zhonghua zhong liu za zhi [Chinese journal of oncology]
  • [ISO-abbreviation] Zhonghua Zhong Liu Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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14. Zarnegar R, Kebebew E, Duh QY, Clark OH: Malignant pheochromocytoma. Surg Oncol Clin N Am; 2006 Jul;15(3):555-71
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  • [Title] Malignant pheochromocytoma.
  • Malignant pheochromocytoma is a rare disease with a high mortality.
  • However, differentiating between benign and malignant pheochromocytoma is impossible in the absence of locoregional invasion or distant metastasis.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Pheochromocytoma / surgery

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  • (PMID = 16882497.001).
  • [ISSN] 1055-3207
  • [Journal-full-title] Surgical oncology clinics of North America
  • [ISO-abbreviation] Surg. Oncol. Clin. N. Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Number-of-references] 79
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15. Gao B, Meng F, Bian W, Chen J, Zhao H, Ma G, Shi B, Zhang J, Liu Y, Xu Z: Development and validation of pheochromocytoma of the adrenal gland scaled score for predicting malignant pheochromocytomas. Urology; 2006 Aug;68(2):282-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Development and validation of pheochromocytoma of the adrenal gland scaled score for predicting malignant pheochromocytomas.
  • OBJECTIVES: To evaluate the diagnostic performances of the pheochromocytoma of the adrenal gland scaled score (PASS) proposed in a previous report and that of a logistic model developed in this investigation.
  • METHODS: In all 130 patients with malignant or assumed benign pheochromocytomas, 15 predictive variables were observed.
  • CONCLUSIONS: ROC analysis indicated that the PASS could be used for the diagnosis of malignant pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology

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  • (PMID = 16904437.001).
  • [ISSN] 1527-9995
  • [Journal-full-title] Urology
  • [ISO-abbreviation] Urology
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Validation Studies
  • [Publication-country] United States
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16. Scholz T, Schulz C, Klose S, Lehnert H: Diagnostic management of benign and malignant pheochromocytoma. Exp Clin Endocrinol Diabetes; 2007 Mar;115(3):155-9
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  • [Title] Diagnostic management of benign and malignant pheochromocytoma.
  • Measurement of free plasma metanephrines, genetic testing and specific imaging procedures--such as MIBG and octreotide scintigraphy or fluorodopamine PET--represent a considerable progress, and the management of benign pheochromocytomas has become very effective.
  • Here, telomerase catalytic subunit (hTERT) activity and heat shock protein 90 expression could serve both as molecular markers allowing an earlier diagnosis of malignancy and as therapeutic targets.
  • Familial syndromes should be considered both in benign and malignant pheochromocytoma, and should be tested for prior to surgery in selected patient groups.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Genetic Techniques. Humans. Sensitivity and Specificity

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  • (PMID = 17427102.001).
  • [ISSN] 0947-7349
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 48
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17. Zhu Y, He HC, Yuan F, Zhang J, Rui WB, Zhao JP, Shen ZJ, Ning G: Heparanase-1 and Cyclooxygenase-2: prognostic indicators of malignancy in pheochromocytomas. Endocrine; 2010 Aug;38(1):93-9
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  • The objective of this article is to evaluate Heparanase-1 and Cyclooxygenase-2 as tissue-based markers of pheochromocytoma prognosis.
  • Ninety-two sporadic pheochromocytoma patients with a minimum of 8-year follow-up post-diagnosis were enrolled.
  • Slides of normal adrenal glands in nephrectomy specimens from 20 patients with benign renal tumors were as control.
  • Positive staining for Heparanase-1 was observed in 23.68% of the benign and 77.78% of the malignant cases, whereas none of the normal adrenal controls showed positive staining.
  • Similarly, Cyclooxygenase-2 staining was seen in 23.68% of the benign versus 83.33% of the malignant cases, and none of the normal controls appeared positive for Cyclooxygenase-2.
  • Malignant cases showed higher microvessel density compared to benign tumors and normal controls (36.41, 21.43, and 13.36%, respectively).
  • Heparanase-1 and Cyclooxygenase-2 combined is better than their own to be used as a marker to distinguish malignant from benign pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Cyclooxygenase 2 / metabolism. Eukaryotic Initiation Factor-3 / metabolism. Glucuronidase / metabolism. Pheochromocytoma / metabolism

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  • (PMID = 20960108.001).
  • [ISSN] 1559-0100
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / EIF3A protein, human; 0 / Eukaryotic Initiation Factor-3; EC 1.14.99.1 / Cyclooxygenase 2; EC 3.2.1.- / heparanase; EC 3.2.1.31 / Glucuronidase
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18. Mischke K, Graf J, Rulands D, Koch KC, Hanrath P, Janssens U: [Cardiogenic shock in a 61 year old female with recurrent panic attacks]. Internist (Berl); 2005 May;46(5):580-5
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  • Conducting an abdominal ultrasound we detected a mass at the right adrenal gland.
  • The suspected diagnosis of pheochromocytoma was confirmed by elevated catecholamine levels in the urine and a CT scan.
  • After recompensation and subsequent administration of phenoxybenzamine a benign pheochromocytoma was resected.
  • A massive catecholamine secretion due to a pheochromocytoma can lead to a cardiogenic shock and multiple organ failure.
  • In patients with recurrent panic attacks and hypertension a pheochromocytoma should be included in the differential diagnosis.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Panic Disorder / diagnosis. Panic Disorder / prevention & control. Pheochromocytoma / diagnosis. Secondary Prevention. Shock, Cardiogenic / diagnosis. Shock, Cardiogenic / prevention & control
  • [MeSH-minor] Cardiomyopathies / diagnosis. Cardiomyopathies / etiology. Diagnosis, Differential. Female. Humans. Middle Aged

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  • (PMID = 15806412.001).
  • [ISSN] 0020-9554
  • [Journal-full-title] Der Internist
  • [ISO-abbreviation] Internist (Berl)
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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19. Agarwal A, Mehrotra PK, Jain M, Gupta SK, Mishra A, Chand G, Agarwal G, Verma AK, Mishra SK, Singh U: Size of the tumor and pheochromocytoma of the adrenal gland scaled score (PASS): can they predict malignancy? World J Surg; 2010 Dec;34(12):3022-8
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  • [Title] Size of the tumor and pheochromocytoma of the adrenal gland scaled score (PASS): can they predict malignancy?
  • The goal of this study was to find a correlation between the tumor size and malignant potential of PCC and determine whether the "Pheochromocytoma of the adrenal gland scaled score" (PASS) proposed by Thompson can be applied to predict malignancy.
  • RESULTS: Of the benign cases, none developed recurrence or metastasis.
  • Of the sporadic benign cases, 21 (41%) patients with tumor size > 6 cm had a PASS of >4, and none of them developed metastasis.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Glands / pathology. Neoplasm Staging. Pheochromocytoma / pathology

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  • (PMID = 20703467.001).
  • [ISSN] 1432-2323
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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20. Yu R, Nissen NN, Dhall D, Wei M: Pheochromocytoma in patients suspected of harboring adrenal metastasis: management and clinical predictors. Endocr Pract; 2008 Nov;14(8):967-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma in patients suspected of harboring adrenal metastasis: management and clinical predictors.
  • OBJECTIVE: To study clinical management of patients with suspected adrenal metastasis and to assess whether there are clinical predictors of pheochromocytoma in this patient population.
  • METHODS: In this retrospective cross-sectional study, we reviewed medical records of patients who had adrenalectomy for adrenal lesions or had adrenal biopsy performed between January 1997 and July 2007 in a large academic hospital.
  • Patients who harbored adrenal masses that were suspected of being metastases were identified on clinical findings.
  • Pathologic diagnosis, demographic data, clinical history, imaging studies, and laboratory test results were reviewed and compared among patients whose adrenal mass was determined to be metastasis, adenoma, or pheochromocytoma.
  • RESULTS: One-hundred sixty-three patients had adrenalectomy or had adrenal biopsy during the study period.
  • Thirty patients (18%) had adrenal masses that were suspected of being metastases.
  • Of the adrenal masses, 18 (60%) were metastases, 8 (27%) were benign adenomas, and 4 (13%) were pheochromocytomas.
  • Eleven patients (37%) had biochemical testing for pheochromocytoma.
  • Adrenal biopsy was performed without biochemical testing for pheochromocytoma in 9 patients (30%), including 2 subsequently found to have this tumor.
  • Adrenalectomy was performed in 10 patients (33%) without biochemical testing for pheochromocytoma.
  • Clinical parameters were similar among patients with metastasis, adenoma, or pheochromocytoma.
  • There were no clinical predictors to suggest pheochromocytoma.
  • CONCLUSIONS: Pheochromocytoma occurs frequently in patients suspected of harboring adrenal metastasis, but this tumor is often not considered in clinical practice.
  • The size and imaging characteristics of the adrenal mass and history of known metastasis may help clinicians in decision-making.
  • Biochemical testing for pheochromocytoma should ideally be performed in all patients suspected of having adrenal metastasis.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology

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  • (PMID = 19095594.001).
  • [ISSN] 1934-2403
  • [Journal-full-title] Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
  • [ISO-abbreviation] Endocr Pract
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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21. Tavangar SM, Shojaee A, Moradi Tabriz H, Haghpanah V, Larijani B, Heshmat R, Lashkari A, Azimi S: Immunohistochemical expression of Ki67, c-erbB-2, and c-kit antigens in benign and malignant pheochromocytoma. Pathol Res Pract; 2010 May 15;206(5):305-9
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  • [Title] Immunohistochemical expression of Ki67, c-erbB-2, and c-kit antigens in benign and malignant pheochromocytoma.
  • In this study, we evaluated the immunohistochemical expression and the possible advantages of Ki67 antigen, c-erbB-2, and c-kit proto-oncogenes in the differentiation between benign and malignant pheochromocytomas.
  • Paraffin-embedded tissue blocks from 44 benign (35 adrenal and 9 extra-adrenal) and 11 malignant (9 adrenal and 2 extra-adrenal) cases of pheochromocytoma were selected for immunohistochemical staining using antibodies against Ki67, c-erbB-2, and c-kit antigens.
  • In addition, Ki67 positivity and c-erbB-2 expression can be used as immunohistochemical markers for predicting the malignant behavior of pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Ki-67 Antigen / metabolism. Pheochromocytoma / metabolism. Proto-Oncogene Proteins c-kit / metabolism. Receptor, ErbB-2 / metabolism

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  • [Copyright] (c) 2010 Elsevier GmbH. All rights reserved.
  • (PMID = 20189725.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptor, ErbB-2
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22. Mazurkiewicz M, Wasiutyński A: [Current opinion in pathogenesis of pheochromocytoma]. Pol Merkur Lekarski; 2007 Jun;22(132):509-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Current opinion in pathogenesis of pheochromocytoma].
  • Pheochromocytoma is a neuroendocrine tumor with significant clinical relevance.
  • Most of the tumors are benign, but sometimes malignant cases are seen and there prognosis is unfavorable.
  • So far, none of the factors which could initiate development of pheochromocytoma are known, besides genetic ones.
  • In this review, most common familial syndromes connected with pheochromocytoma are characterized.
  • [MeSH-major] Adrenal Gland Neoplasms / etiology. Genetic Predisposition to Disease. Pheochromocytoma / etiology

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  • (PMID = 17874618.001).
  • [ISSN] 1426-9686
  • [Journal-full-title] Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego
  • [ISO-abbreviation] Pol. Merkur. Lekarski
  • [Language] pol
  • [Publication-type] Editorial; English Abstract
  • [Publication-country] Poland
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23. Adler JT, Meyer-Rochow GY, Chen H, Benn DE, Robinson BG, Sippel RS, Sidhu SB: Pheochromocytoma: current approaches and future directions. Oncologist; 2008 Jul;13(7):779-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma: current approaches and future directions.
  • Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites.
  • While medical management plays a significant role in the treatment of pheochromocytoma patients, surgical excision remains the only cure.
  • Improved medical management and surgical techniques and an increased understanding of hereditary disease have improved the outcome of pheochromocytoma patients with benign disease; however, the outcome of patients with malignant disease remains poor.
  • In this review, we discuss the presentation, diagnosis, management, and future directions in the management of this disease.
  • [MeSH-major] Adrenal Gland Neoplasms. Pheochromocytoma
  • [MeSH-minor] Adrenal Glands / surgery. Chemotherapy, Adjuvant. Genetic Predisposition to Disease. Humans. Neoplasm Metastasis / prevention & control. Radiotherapy, Adjuvant


24. Brouwers FM, Elkahloun AG, Munson PJ, Eisenhofer G, Barb J, Linehan WM, Lenders JW, De Krijger R, Mannelli M, Udelsman R, Ocal IT, Shulkin BL, Bornstein SR, Breza J, Ksinantova L, Pacak K: Gene expression profiling of benign and malignant pheochromocytoma. Ann N Y Acad Sci; 2006 Aug;1073:541-56
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  • [Title] Gene expression profiling of benign and malignant pheochromocytoma.
  • There are currently no reliable diagnostic and prognostic markers or effective treatments for malignant pheochromocytoma.
  • Other subgroups of tumors included those defined by tissue norepinephrine compared to epinephrine contents (i.e., noradrenergic versus adrenergic phenotypes), adrenal versus extra-adrenal locations, and presence of germline mutations of genes predisposing to the tumor.
  • Correcting for the confounding influence of noradrenergic versus adrenergic catecholamine phenotype by the analysis of variance revealed a larger and more accurate number of genes that discriminated benign from malignant pheochromocytomas than when the confounding influence of catecholamine phenotype was not considered.
  • Seventy percent of these genes were underexpressed in malignant compared to benign tumors.
  • Similarly, 89% of genes were underexpressed in malignant primary tumors compared to benign tumors, suggesting that malignant potential is largely characterized by a less-differentiated pattern of gene expression.
  • Further progress on the basis of this database can be made from follow-up confirmatory studies, application of bioinformatics approaches for data mining and pathway analyses, testing in pheochromocytoma cell culture and animal model systems, and retrospective and prospective studies of diagnostic markers.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Profiling. Pheochromocytoma / genetics

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  • (PMID = 17102123.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z99 CT999999; United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
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25. Kajor M, Ziaja J, Lange D, Król R, Ciupińska-Kajor M, Turska-d'Amico M, Maka B, Cierpka L: [Analysis of morphology of adrenal pheochromocytoma as regards their potential malignancy]. Endokrynol Pol; 2005 Nov-Dec;56(6):911-6
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  • [Title] [Analysis of morphology of adrenal pheochromocytoma as regards their potential malignancy].
  • BACKGROUND: Adrenal pheochromocytoma are diagnosed the most often in patients with arterial hypertension or with thyroid medullar cancer and suspicion of MEN II syndromes.
  • The aim of the study is to analyse the morphology of pheochromocytomas on the basis of Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) in order to estimate their potential malignancy.
  • The diagnosis of pheochromocytoma was establish before surgery in 87.5%.
  • RESULTS: In pathological examination benign pheochromo-cytoma was diagnosed in 39 presented patients.
  • In 1 cases malignant form of pheochromocytoma was diagnosed on the basis of lymph nodes metastases.
  • Among 35 patients operated on more than 12 months ago 2 patients died: 1 patient with malignant pheochromocytoma (PASS=8 points) and 1 patient with MEN IIA syndrome (due to disseminated thyroid medullar cancer).
  • CONCLUSION: Analysis of pheochromocytoma in PASS is only of rough character and does not allow for clear-cut histological diagnosis of benign and malignant tumours.
  • The only unquestionable criterion of pheochromocytoma's malignancy remain metastases.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology. Pheochromocytoma / secondary
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Diagnosis, Differential. Female. Humans. Lymphatic Metastasis. Male. Middle Aged. Multiple Endocrine Neoplasia Type 2a / pathology. Neoplasm Staging. Thyroid Neoplasms / pathology

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  • (PMID = 16821210.001).
  • [ISSN] 0423-104X
  • [Journal-full-title] Endokrynologia Polska
  • [ISO-abbreviation] Endokrynol Pol
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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26. Korpershoek E, Stobbe CK, van Nederveen FH, de Krijger RR, Dinjens WN: Intra-tumoral molecular heterogeneity in benign and malignant pheochromocytomas and extra-adrenal sympathetic paragangliomas. Endocr Relat Cancer; 2010 Sep;17(3):653-62
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  • [Title] Intra-tumoral molecular heterogeneity in benign and malignant pheochromocytomas and extra-adrenal sympathetic paragangliomas.
  • Pheochromocytomas (PCCs) and extra-adrenal sympathetic paragangliomas (sPGLs) are catecholamine-producing tumors occurring in the context of hereditary tumor syndromes, with known germline mutations, and as sporadic tumors.
  • Since knowledge on intra-tumoral heterogeneity is important for understanding the pathogenesis of these tumors, we investigated 12 benign and 8 malignant PCCs and sPGLs for loss of heterozygosity (LOH) on DNA extracted from different regions of each tumor and from metastases.
  • Benign tumors were found to have less intra-tumoral heterogeneity (overall 8%) than malignant tumors (overall 23%), with the highest frequencies for chromosome 1p36 in the benign tumors (17%) and 1p13 and 3q24 in malignant tumors (both 38%).
  • We demonstrate that malignant PCC and sPGL have more intra-tumoral molecular heterogeneity than benign tumors, which suggests that benign and malignant PCC and sPGL have a different pathogenesis.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Chromosomes, Human. Loss of Heterozygosity. Paraganglioma / genetics. Pheochromocytoma / genetics

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  • (PMID = 20488782.001).
  • [ISSN] 1479-6821
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA, Neoplasm
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27. González JI, Angoso M, García JA, Clemen JJ, Prado A, Angoso F: [Extra-adrenal pheochromocytoma: update of the most controversial features. Apropos of two cases]. Cir Esp; 2005 Mar;77(3):166-9
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  • [Title] [Extra-adrenal pheochromocytoma: update of the most controversial features. Apropos of two cases].
  • They are usually symptomatic and sporadic and are generally located in the adrenal medulla.
  • We present two cases of extra-adrenal nonfunctional pheochromocytomas located in the retroperitoneum.
  • The tumors were apparently benign and sporadic and were not associated with other neoplasms or hereditary diseases.
  • Several immunohistochemical techniques were performed to reach the definitive diagnosis.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / radiography. Pheochromocytoma / pathology. Pheochromocytoma / radiography

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  • (PMID = 16420910.001).
  • [ISSN] 0009-739X
  • [Journal-full-title] Cirugía española
  • [ISO-abbreviation] Cir Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 16
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28. Juan-Sallés C, Ramos-Vara JA, Garner MM: Pheochromocytoma in six New World primates. Vet Pathol; 2009 Jul;46(4):662-6
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  • [Title] Pheochromocytoma in six New World primates.
  • Six New World primates, including 2 golden lion tamarins (Leontopithecus rosalia), 2 cotton-top tamarins (Saguinus o. oedipus), 1 black howler monkey (Alouatta caraya), and 1 black-handed spider monkey (Ateles g. geoffroyi), were diagnosed with unilateral (4/6) or bilateral (1/6) adrenal or extra-adrenal (1/6) pheochromocytoma by light microscopy and immunohistochemical staining for chromogranin A.
  • Overt invasive behavior or metastases were not observed in any primate, and thus these neoplasms were considered benign.
  • [MeSH-major] Adrenal Gland Neoplasms / veterinary. Animals, Zoo. Monkey Diseases / pathology. Pheochromocytoma / veterinary. Platyrrhini

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  • (PMID = 19276065.001).
  • [ISSN] 1544-2217
  • [Journal-full-title] Veterinary pathology
  • [ISO-abbreviation] Vet. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chromogranin A
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29. Cotesta D, Petramala L, Serra V, Pergolini M, Crescenzi E, Zinnamosca L, De Toma G, Ciardi A, Carbone I, Massa R, Filetti S, Letizia C: Clinical experience with pheochromocytoma in a single centre over 16 years. High Blood Press Cardiovasc Prev; 2009 Dec;16(4):183-93

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical experience with pheochromocytoma in a single centre over 16 years.
  • BACKGROUND: Pheochromocytoma and paraganglioma are rare tumours of neuroectodermal origin.
  • Pheochromocytoma occurs in 0.1-2% of people with hypertension, while the incidence rises to 4-5% in patients with incidental adrenal mass.
  • AIM: To analyse the presentation, diagnosis and localization, pathology, molecular genetic aspects, surgical management and long-term outcome of a large series of patients with pheochromocytoma referred to a single centre.
  • METHODS: From 1992 to 2008, we observed 91 patients with pheochromocytoma, 70 (77%) with the sporadic form and 21 (23%) with the hereditary form.
  • In the group with the hereditary form, the mean age at diagnosis was significantly lower than the mean age of the group with the sporadic form (38 vs 48 years; p < 0.001).
  • RESULTS: Eighty (88%) patients with pheochromocytoma were symptomatic and the classical triad of palpitations, headache and diaphoresis was present in 30% of patients.
  • In 12% of patients, the pheochromocytoma was discovered during radiological images (adrenal incidentaloma).
  • The unilateral adrenal localization was observed in 78% of patients, bilateral in 15% and extra-adrenal in 7% of patients.
  • All pheochromocytoma patients underwent surgical procedure and the laparotomic approach was used in 49 (53%) patients, while the laparoscopic approach was performed in 42 (47%) patients.
  • Pheochromocytoma was benign in 86 patients (93%) [mean size was 4.3 cm] and the malignant form was found in five patients (7%) [mean size was 10 cm].
  • CONCLUSIONS: These data from a large cohort of patients are consistent with those reported in the literature and show that pheochromocytoma is an in important challenge for clinicians.

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30. Havekes B, Romijn JA, Eisenhofer G, Adams K, Pacak K: Update on pediatric pheochromocytoma. Pediatr Nephrol; 2009 May;24(5):943-50
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  • [Title] Update on pediatric pheochromocytoma.
  • Pheochromocytomas are rare tumors in children arising from chromaffin cells of adrenal medullary or extra-adrenal paraganglionic tissue.
  • The formerly used guidelines for pheochromocytoma have been changed by recent discoveries, implementation of new approaches, and understanding of biochemistry, genetics, imaging, pathophysiology, and nomenclature of these tumors.
  • In children, pheochromocytomas are more frequently familial, extra-adrenal, bilateral, and multifocal than in adults.
  • Because of a highly variable clinical presentation, pheochromocytoma is often referred to as the great mimic.
  • Measurements of plasma or urinary fractionated metanephrines are recommended as first-line biochemical tests for diagnosis, with optimum diagnostic sensitivity to be preferred over specificity.
  • This article reviews recent advances in biochemistry, genetics, and imaging and outlines recommendations for improved evaluation and treatment of children with benign or malignant pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology

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  • (PMID = 18566838.001).
  • [ISSN] 1432-198X
  • [Journal-full-title] Pediatric nephrology (Berlin, Germany)
  • [ISO-abbreviation] Pediatr. Nephrol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 5001-33-2 / Metanephrine
  • [Number-of-references] 66
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31. Namour F, Ayav A, Lu X, Klein M, Muresan M, Bresler L, Tramoy D, Guéant JL, Brunaud L: Lack of association between microsatellite instability and benign adrenal tumors. World J Surg; 2006 Jul;30(7):1240-6
MedlinePlus Health Information. consumer health - Pheochromocytoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Lack of association between microsatellite instability and benign adrenal tumors.
  • BACKGROUND: The adrenal gland may give rise to pheochromocytomas, which are catecholamine-producing tumors originating from the adrenal medulla, or to adrenocortical tumors, which derive from the adrenocortical cortex and may be secreting or not.
  • Microsatellite loci were analyzed in 32 benign tumors, including 11 pheochromocytomas and 21 adrenocortical tumors, in patients with and without familial syndrome.
  • One patient with a unilateral pheochromocytoma showed a loss of heterozygosity for D17S250.
  • A second patient with a MEN-2A syndrome and a two-sided pheochromocytoma exhibited a loss of heterozygosity for D2S123 in the right tumor only and a retention of heterozygosity for all markers in the left tumor.
  • CONCLUSIONS: These results suggest that microsatellite instability, evaluated by the five reference markers of the National Cancer Institute, is not a feature of benign adrenal tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Microsatellite Repeats / genetics. Pheochromocytoma / genetics

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  • (PMID = 16715450.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Proto-Oncogene Proteins
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32. Cherifi-Gatta B, Gaye D, Laurent F, Tabarin A: [Adrenal incidentalomas]. Rev Prat; 2008 May 15;58(9):979-89
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Adrenal incidentalomas].
  • The adrenal incidentaloma is an adrenal mass, generally more than 1 cm in diameter, that is discovered serendipitously during a radiologic examination performed for indications other than an evaluation of adrenal disease.
  • The prevalence of adrenal incidentalomas is at least 1% in patients who underwent abdominal CT examination.
  • Surgery is mandatory in patients with secretory tumours (pheochromocytoma, secreting cortical adenomas) and with adrenal cancer.
  • However, the vast majority of adrenal incidentalomas are benign cortical adenomas and surgery is mandatory only in a minority of patients.
  • Systematic aetiological investigation of adrenal incidentalomas should therefore be performed in order to guide the decision on surgical or non surgical management.
  • The optimal duration and modalities of follow-up for patients with adrenal incidentalomas are uncertain.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis

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  • (PMID = 18672664.001).
  • [ISSN] 0035-2640
  • [Journal-full-title] La Revue du praticien
  • [ISO-abbreviation] Rev Prat
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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33. Sandgren J, Andersson R, Rada-Iglesias A, Enroth S, Akerstrom G, Dumanski JP, Komorowski J, Westin G, Wadelius C: Integrative epigenomic and genomic analysis of malignant pheochromocytoma. Exp Mol Med; 2010 Jul 31;42(7):484-502
Hazardous Substances Data Bank. L-Lysine .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Integrative epigenomic and genomic analysis of malignant pheochromocytoma.
  • Pheochromocytoma is a rare endocrine tumor of the adrenal gland that mostly occurs sporadic with unknown epigenetic/genetic cause.
  • The majority of cases are benign.
  • Here we aimed to combine the genome-wide profiling of H3K4me3 and H3K27me3, obtained by the ChIP-chip methodology, and DNA copy number data with global gene expression examination in a malignant pheochromocytoma sample.
  • The integrated analysis of the tumor expression levels, in relation to normal adrenal medulla, indicated that either histone modifications or chromosomal alterations, or both, have great impact on the expression of a substantial fraction of the genes in the investigated sample.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / pathology. Epigenesis, Genetic. Genome, Human / genetics. Genomics. Pheochromocytoma / genetics. Pheochromocytoma / pathology

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  • (PMID = 20534969.001).
  • [ISSN] 2092-6413
  • [Journal-full-title] Experimental & molecular medicine
  • [ISO-abbreviation] Exp. Mol. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Histones; 0 / Tumor Suppressor Proteins; K3Z4F929H6 / Lysine
  • [Other-IDs] NLM/ PMC2912476
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34. Kalinin AP, Bogatyrev OP, Poliakova GA, Beloshitskiĭ ME, Britvin TA: [Adrenal gland surgery]. Vestn Ross Akad Med Nauk; 2005;(11):43-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Adrenal gland surgery].
  • The authors of the article analyze more than 30-year experience of surgical treatment of adrenal gland diseases, offering a detailed description of clinical and morphological features of the main nosological forms such as Itsenko-Cushing disease and syndrome, primary hyperaldosteronism, pheochromocytoma, incidentaloma, and malignant adrenal tumors.
  • The paper contains basic diagnostic criteria, indications to operative treatment, and extents of surgery for each of the diseases considered, as well as comparative analysis of peculiarities of conventional ("open"), and endoscopic adrenal gland surgeries.
  • The article demonstrates significance of various immunohistochemical markers in differentiation between malignant and benign adrenal tumors.

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  • (PMID = 16408654.001).
  • [ISSN] 0869-6047
  • [Journal-full-title] Vestnik Rossiiskoi akademii meditsinskikh nauk
  • [ISO-abbreviation] Vestn. Akad. Med. Nauk SSSR
  • [Language] RUS
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Russia (Federation)
  • [Number-of-references] 16
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35. Ahlman H: Malignant pheochromocytoma: state of the field with future projections. Ann N Y Acad Sci; 2006 Aug;1073:449-64
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant pheochromocytoma: state of the field with future projections.
  • The prevalence of malignant pheochromocytoma is about 10%, and is somewhat higher for paraganglioma.
  • A problem for clinical follow-up is that patients with "benign" histopathologic findings may develop metastatic disease.
  • At the first international symposium on pheochromocytoma in Bethesda (2005) experts from different disciplines and patients shared their experiences, and the present knowledge of this rare disease was updated.
  • The discussion related to future strategies for better clinical/histopathologic diagnosis and understanding of different geno- and phenotypes.
  • [MeSH-major] Adrenal Gland Neoplasms / epidemiology. Pheochromocytoma / epidemiology


36. Yu R, Furmark L, Wong C: Cardiac abnormalities associated with pheochromocytoma and other adrenal tumors. Endocr Pract; 2009 Jan-Feb;15(1):10-6
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  • [Title] Cardiac abnormalities associated with pheochromocytoma and other adrenal tumors.
  • OBJECTIVE: To study the specific cardiac abnormalities associated with pheochromocytoma and to suggest a strategy for evaluating cardiac function in patients with pheochromocytoma.
  • METHODS: In this case-control study, we reviewed pathology records of patients seen at Cedars-Sinai Medical Center between 1997 and 2007; patients with adrenal or extra-adrenal pheochromocytoma and those with nonfunctioning benign or malignant adrenal tumors were identified.
  • Patients with functioning adrenal adenomas that secreted cortisol or aldosterone were excluded.
  • Clinical history, imaging, pathology, biochemical test results, electrocardiographic findings, and echocardiographic findings were compared between patients with pheochromocytoma and patients with nonfunctioning adrenal tumors.
  • RESULTS: The charts of 22 patients with pheochromocytoma and 35 patients with nonfunctioning adrenal tumors were included.
  • The average age of patients with pheochromocytoma was similar to that of control patients (51.9 +/- 3.9 years vs 60.2 +/- 2.5 years, respectively), as was the number of patients with known cardiovascular diseases (2 [9%] in the pheochromocytoma group vs 5 [14%] in the control group).
  • Two patients with pheochromocytoma (9%) exhibited myocardial damage.
  • Abnormal electrocardiographic findings were present in 16 patients with pheochromocytoma (73%) and in 17 control patients (49%) (P = .1).
  • QTc was prolonged in patients with pheochromocytoma compared with control patients (448.3 +/- 9.7 ms vs 424.7 +/- 4.5 ms, respectively; P = .02) and was correlated with levels of norepinephrine and normetanephrine, but not with levels of epinephrine and metanephrine or tumor size.
  • ST-T abnormalities were present in 11 patients with pheochromocytoma (50%) and in 8 control patients (23%) (P = .04).
  • Echocardiographic findings were normal in most patients with pheochromocytoma; abnormal left ventricular wall motion was documented in 3 patients with long QTc.
  • CONCLUSIONS: The specific electrocardiographic findings in patients with pheochromocytoma are prolonged QTc and ST-T abnormalities.
  • Performing an electrocardiogram in patients with pheochromocytoma would be prudent.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Adrenal Gland Neoplasms / physiopathology. Heart / physiopathology. Pheochromocytoma / complications. Pheochromocytoma / physiopathology

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  • (PMID = 19211391.001).
  • [ISSN] 1934-2403
  • [Journal-full-title] Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
  • [ISO-abbreviation] Endocr Pract
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0J45DE6B88 / Normetanephrine; X4W3ENH1CV / Norepinephrine
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37. Tonks ID, Mould AW, Schroder WA, Cotterill A, Hayward NK, Walker GJ, Kay GF: Dual loss of rb1 and Trp53 in the adrenal medulla leads to spontaneous pheochromocytoma. Neoplasia; 2010 Mar;12(3):235-43
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  • [Title] Dual loss of rb1 and Trp53 in the adrenal medulla leads to spontaneous pheochromocytoma.
  • Using a Cre/loxP system, we have determined the phenotypic consequences attributable to in vivo deletion of both Rb1 and Trp53 in the mouse adrenal medulla.
  • The coablation of these two tumor suppressor genes during embryogenesis did not disrupt adrenal gland development but resulted in the neoplastic transformation of the neural crest-derived adrenal medulla, yielding pheochromocytomas (PCCs) that developed with complete penetrance and were inevitably bilateral.
  • Despite their typically benign status, these PCCs had profound ramifications on mouse vitality, with effected mice having a median survival of only 121 days.
  • The structural remodeling of the heart in mice harboring Rb1(-/-):Trp53(-/-) PCCs suggests that the mortality of these mice may be attributable to the inappropriate release of catecholamines from the mutated adrenal chromaffin cells.
  • On the basis of the collective data from Rb1 and Trp53 knockout mouse models, it seems that the conversion of Rb1 loss-driven adrenal medulla hyperplasia to PCC can be greatly enhanced by the compound loss of Trp53, whereas the loss of Trp53 alone is generally ineffectual on adrenal chromaffin cell homeostasis.
  • Consequently, the Trp53 tumor suppressor gene is an efficient genetic modifier of Rb1 loss in the development of PCC, and their compound loss in the adrenal medulla has a profound impact on both cellular homeostasis and animal vitality.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Genes, p53 / physiology. Pheochromocytoma / pathology. Retinoblastoma Protein / physiology

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  • (PMID = 20234817.001).
  • [ISSN] 1476-5586
  • [Journal-full-title] Neoplasia (New York, N.Y.)
  • [ISO-abbreviation] Neoplasia
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Retinoblastoma Protein; EC 2.7.7.- / Cre recombinase; EC 2.7.7.- / Integrases
  • [Other-IDs] NLM/ PMC2838441
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38. Rescorla FJ: Malignant adrenal tumors. Semin Pediatr Surg; 2006 Feb;15(1):48-56
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  • [Title] Malignant adrenal tumors.
  • Adrenal tumors, apart from neuroblastoma, are relatively rare in infancy and childhood.
  • Most adrenal lesions are benign, and both benign and malignant tumors may be hormonally active thus, making accurate preoperative diagnosis difficult.
  • The two main malignant tumors are adrenocortical carcinoma and pheochromocytoma.
  • In both tumors, it may be difficult to determine benign from malignant and the biologic behavior and degree of invasion may portend a more malignant course.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 16458846.001).
  • [ISSN] 1055-8586
  • [Journal-full-title] Seminars in pediatric surgery
  • [ISO-abbreviation] Semin. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 98
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39. Ridho FE, Adam FM, Adam JM: Adrenal incidentaloma. Acta Med Indones; 2009 Apr;41(2):87-93
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  • [Title] Adrenal incidentaloma.
  • Adrenal Incidentaloma is an adrenal tumor, which is unidentified before the imaging procedures conducted for an abnormality which is initially unforeseen as an adrenal disease.
  • Symptoms and/or clinical signs of adrenal tumor do not have to be present prior to a diagnosis.
  • Adrenal Incidentaloma can be divided into non-hypersecreting adrenal adenoma, hypersecreting tumor, primary adrenal carcinoma, other adrenal mass, and metastases.
  • The majority of adrenal tumor is non-hypersecreting adrenal adenoma, but it is always considered as hypersecreting tumor until proven otherwise.
  • Some conditions that can be found due to hormonal activity of adrenal incidentaloma is subclinical Cushing's syndrome, pheochromocytoma, aldosteronoma (Conn's disease), and several tumors which secrete androgen and sex hormone.
  • Diagnostic approach of adrenal incidentaloma is focused on two main problems, which are, whether the lesion is hormonally active even though lacking characteristic clinical signs, and whether the lesion is benign or malignant; thus it needs hormonal and radiologic evaluation, even a fine needle biopsy.
  • The management for adrenal incidentaloma includes surgical removal for hormonally active adrenal tumors, or inactive tumors with size less than 4 cm.
  • [MeSH-major] Adrenal Gland Neoplasms. Aldosterone / blood. Catecholamines / urine. Incidental Findings. Renin / blood
  • [MeSH-minor] Biopsy, Fine-Needle. Diagnosis, Differential. Humans. Indonesia / epidemiology. Morbidity / trends

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  • (PMID = 19390128.001).
  • [ISSN] 0125-9326
  • [Journal-full-title] Acta medica Indonesiana
  • [ISO-abbreviation] Acta Med Indones
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Indonesia
  • [Chemical-registry-number] 0 / Catecholamines; 4964P6T9RB / Aldosterone; EC 3.4.23.15 / Renin
  • [Number-of-references] 38
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40. Pacak K, Eisenhofer G, Ahlman H, Bornstein SR, Gimenez-Roqueplo AP, Grossman AB, Kimura N, Mannelli M, McNicol AM, Tischler AS, International Symposium on Pheochromocytoma: Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endocrinol Metab; 2007 Feb;3(2):92-102
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  • [Title] Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005.
  • The First International Symposium on Pheochromocytoma, held in October 2005, included discussions about developments concerning these rare catecholamine-producing tumors.
  • Recommendations were made during the symposium for biochemical diagnosis, localization, genetics, and treatment.
  • Measurement of plasma or urinary fractionated metanephrines, the most accurate screening approach, was recommended as the first-line test for diagnosis; reference intervals should favor sensitivity over specificity.
  • Inadequate methods to distinguish malignant from benign tumors and a lack of effective treatments for malignancy are important problems requiring further resolution.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Paraganglioma / diagnosis. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 17237836.001).
  • [ISSN] 1745-8366
  • [Journal-full-title] Nature clinical practice. Endocrinology & metabolism
  • [ISO-abbreviation] Nat Clin Pract Endocrinol Metab
  • [Language] eng
  • [Publication-type] Consensus Development Conference; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0J45DE6B88 / Normetanephrine; 5001-33-2 / Metanephrine; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret
  • [Number-of-references] 83
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41. Thouënnon E, Elkahloun AG, Guillemot J, Gimenez-Roqueplo AP, Bertherat J, Pierre A, Ghzili H, Grumolato L, Muresan M, Klein M, Lefebvre H, Ouafik L, Vaudry H, Plouin PF, Yon L, Anouar Y: Identification of potential gene markers and insights into the pathophysiology of pheochromocytoma malignancy. J Clin Endocrinol Metab; 2007 Dec;92(12):4865-72
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  • [Title] Identification of potential gene markers and insights into the pathophysiology of pheochromocytoma malignancy.
  • CONTEXT: Pheochromocytomas are catecholamine-producing tumors that are generally benign but that can also present as or develop into malignancy.
  • OBJECTIVES: We conducted a gene expression profiling of benign and malignant tumors to identify a gene signature that would allow us to discriminate benign from malignant pheochromocytomas and to gain a better understanding of tumorigenic pathways associated with malignancy.
  • DESIGN: A total of 36 patients with pheochromocytoma was studied retrospectively.
  • There were 18 (nine benign and nine malignant) tumors used for gene expression profiling on pangenomic oligonucleotide microarrays.
  • CONCLUSION: The gene expression profiling of benign and malignant pheochromocytomas clearly identified a set of genes that could be used as a prognostic multi-marker and revealed that the expression of several genes encoding neuroendocrine proteins was reduced in malignant compared with benign tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / pathology. Pheochromocytoma / genetics. Pheochromocytoma / pathology

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  • (PMID = 17878247.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Genetic Markers; 0 / RNA, Neoplasm
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42. Blake MA, Cronin CG, Boland GW: Adrenal imaging. AJR Am J Roentgenol; 2010 Jun;194(6):1450-60
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  • [Title] Adrenal imaging.
  • OBJECTIVE: Adrenal nodules are frequently encountered on current high-resolution imaging, and accurate characterization of such lesions is critical for appropriate patient care.
  • Our article highlights how imaging techniques such as CT densitometry, CT washout characteristics, chemical shift MRI, PET, and PET/CT help characterize most adrenal lesions.
  • We focus on these techniques as well as specifically, because of space constraints, the varied imaging appearances of adrenocortical carcinoma, pheochromocytoma, and lymphoma on these techniques.
  • CONCLUSION: The imaging characterization of adrenal lesions has continued to advance over the past decade as new technologies have evolved.
  • CT, MRI, PET, and PET/CT are now established clinical techniques capable of differentiating benign from malignant adrenal lesions.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis. Diagnostic Imaging. Lymphoma / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Contrast Media. Diagnosis, Differential. Humans. Magnetic Resonance Imaging / methods. Radiopharmaceuticals. Tomography, Emission-Computed / methods. Tomography, X-Ray Computed / methods

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  • [ErratumIn] AJR Am J Roentgenol. 2012 May;198(5):1232
  • (PMID = 20489083.001).
  • [ISSN] 1546-3141
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Contrast Media; 0 / Radiopharmaceuticals
  • [Number-of-references] 74
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43. Tömböl Z, Eder K, Kovács A, Szabó PM, Kulka J, Likó I, Zalatnai A, Rácz G, Tóth M, Patócs A, Falus A, Rácz K, Igaz P: MicroRNA expression profiling in benign (sporadic and hereditary) and recurring adrenal pheochromocytomas. Mod Pathol; 2010 Dec;23(12):1583-95
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  • [Title] MicroRNA expression profiling in benign (sporadic and hereditary) and recurring adrenal pheochromocytomas.
  • The objective of our study was to perform microRNA expression profiling in sporadic and hereditary benign, and recurring adrenomedullary tumors.
  • A total of 21 formalin-fixed paraffin-embedded samples (sporadic benign, multiple endocrine neoplasia 2, von Hippel-Lindau disease, sporadic recurring) were subjected to microRNA expression profiling using microarrays.
  • Furthermore, microRNA expression profiles of a malignant pheochromocytoma and a pair of primary and recurrent tumors were studied by TaqMan Human MicroRNA Cards.
  • Five of these were validated by real-time RT-PCR. miR-139-3p, miR-541 and miR-765 were significantly differentially expressed between sporadic benign and von Hippel-Lindau-related pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Profiling. MicroRNAs / genetics. Pheochromocytoma / genetics

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  • (PMID = 20818339.001).
  • [ISSN] 1530-0285
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MicroRNAs
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44. De Toma G, Letizia C, Cavallaro G, Polistena A, Cotesta D, Petramala L, Porciello R, Giustini S, Calvieri S: Adrenal pheochromocytoma and jejunal neurofibroma in type 1 neurofibromatosis: report of a case. G Chir; 2007 May;28(5):199-202
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  • [Title] Adrenal pheochromocytoma and jejunal neurofibroma in type 1 neurofibromatosis: report of a case.
  • Diagnosis, despite extreme clinical variability, is defined by established diagnostic criteria.
  • CASE REPORT: A case of a patient affected by NF1, with hypertension due to adrenal pheochromocytoma and with jejunal neurofibroma, is reported.
  • DISCUSSION AND CONCLUSIONS: Variability in clinical presentation of NF1 with possible manifestation of severe systemic benign and malignant diseases requires strict follow-up and specific screening of extra-cutaneous lesions.
  • [MeSH-major] Adrenal Gland Neoplasms. Jejunal Neoplasms. Neoplasms, Multiple Primary. Neurofibromatosis 1. Pheochromocytoma


45. Wang H, Zhou FJ, Qin ZK, Han H, Liu ZW: [Laparoscopic surgery for benign adrenal tumor: a report of 10 cases]. Ai Zheng; 2005 Jan;24(1):76-8
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  • [Title] [Laparoscopic surgery for benign adrenal tumor: a report of 10 cases].
  • BACKGROUND & OBJECTIVE: Adrenalectomy is a standard treatment for benign adrenal tumors.
  • Conventional open operation usually results in bigger incisions, more blood loss, and long hospital stay to patients; while laparoscopic approaches could make up above shortcomings, which is used to resect benign adrenal tumors gradually.
  • METHODS: Clinical data of 10 patients with benign adrenal tumors undergone laparoscopic adrenalectomy were retrospectively analyzed to summarize the experiences and techniques in laparoscopic surgery.
  • CONCLUSION: Laparoscopic surgery for treatment of benign adrenal tumors has the advantages of minimal invasion, safety, and efficiency, and may be a primary therapeutic option for benign adrenal tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy. Pheochromocytoma / surgery

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  • (PMID = 15642205.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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46. Feng C, Li HZ, Yan WG, Luo YF, Cao JL: [The expression and significance of chromogranin A and synaptophysin in adrenal gland tumors]. Zhonghua Zhong Liu Za Zhi; 2005 Aug;27(8):486-8
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  • [Title] [The expression and significance of chromogranin A and synaptophysin in adrenal gland tumors].
  • OBJECTIVE: To investigate the expression of chromogranin A (CgA) and synaptophysin (Syn) for differential diagnosis of different kinds of adrenal gland tumors.
  • METHODS: The samples of 69 adrenal gland tumors and 4 normal adrenal glands were immunohistochemically analyzed for the expression of chromogranin A and synaptophysin.
  • RESULTS: In the normal adrenal gland, CgA and Syn was exclusively detected in the medulla.
  • Syn was detected in adrenocortical adenomas 27/28 (96.4%), adrenocortical carcinoma 7/8 (87.5%), pheochromocytoma 24/25 (96.0%) and adrenal metastatic carcinoma 6/8 (75.0%), respectively.
  • CONCLUSION: There is statistically significant difference of CgA expression between adrenalcortical and adrenal medullary tumors, and also between benign and malignant pheochromocytomas.
  • CgA and Syn are immunohistochemically reliable markers in the differential diagnosis of various kinds of adrenal gland tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Chromogranin A / biosynthesis. Pheochromocytoma / metabolism. Synaptophysin / biosynthesis
  • [MeSH-minor] Adrenocortical Adenoma / diagnosis. Adrenocortical Adenoma / metabolism. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / metabolism. Diagnosis, Differential. Female. Humans. Male

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  • (PMID = 16188147.001).
  • [ISSN] 0253-3766
  • [Journal-full-title] Zhonghua zhong liu za zhi [Chinese journal of oncology]
  • [ISO-abbreviation] Zhonghua Zhong Liu Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Chromogranin A; 0 / Synaptophysin
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47. Valade S, Chazerain P, Khanine V, Lazard T, Baudin E, Ziza JM: [Late bone metastases of a pheochromocytoma]. Rev Med Interne; 2010 Nov;31(11):772-5
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  • [Title] [Late bone metastases of a pheochromocytoma].
  • Pheochromocytoma is a usually benign tumor of chromaffin tissue (90% of cases).
  • We report a 77-year-old patient with a past medical history of surgically removed pheochromocytoma that was considered to be "benign", who presented with secondary bone metastases 5 years later.
  • The possibility of late metastatic extension in patients with pheochromocytoma should be kept in mind.

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  • [Copyright] Copyright © 2010 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.
  • (PMID = 20884095.001).
  • [ISSN] 1768-3122
  • [Journal-full-title] La Revue de medecine interne
  • [ISO-abbreviation] Rev Med Interne
  • [Language] FRE
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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48. DeWitt J, Alsatie M, LeBlanc J, McHenry L, Sherman S: Endoscopic ultrasound-guided fine-needle aspiration of left adrenal gland masses. Endoscopy; 2007 Jan;39(1):65-71
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  • [Title] Endoscopic ultrasound-guided fine-needle aspiration of left adrenal gland masses.
  • BACKGROUND AND STUDY AIM: Although the left adrenal gland is readily visible by endoscopic ultrasound (EUS), there are few published data on the utility of EUS-guided fine-needle aspiration (EUS-FNA) of this site.
  • The aim of this study was to report our experience of EUS-FNA of left adrenal gland masses.
  • PATIENTS AND METHODS: In this retrospective case series, we reviewed our EUS and cytology databases to identify consecutive patients who underwent EUS-FNA of the left adrenal gland between January 1997 and January 2004.
  • RESULTS: Our searches resulted in the identification of a series of 38 consecutive patients who underwent EUS for the evaluation of a lung mass (n = 14), a pancreatic mass (n = 14), obstructive jaundice (n = 1), dysphagia (n = 2), an ampullary adenoma (n = 1), celiac block (n = 1), or a left adrenal gland mass (n = 5).
  • The mean maximal left adrenal mass diameter was 24 mm (range 7-66 mm).
  • Diagnoses after EUS-FNA (the mean number of passes was 3.6) were: metastatic lung cancer (n = 2), esophageal adenocarcinoma (n = 1), melanoma (n = 1), renal cell carcinoma (n = 1), and pancreatic neuroendocrine tumor (n = 1); primary pheochromocytoma (n = 1); benign adrenal tissue (n = 21); and granulomatous inflammation (n = 1).
  • The absence of a discrete adrenal mass on EUS occurred more frequently in patients with nondiagnostic biopsies than in those with diagnostic biopsies (56 % vs. 7 %; odds ratio 23.4, 95 %CI 3.5 - 157.0; P = 0.004).
  • CONCLUSIONS: EUS-FNA of the left adrenal gland is safe and can be useful for the evaluation and staging of suspected malignancy.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Biopsy, Fine-Needle / methods. Endoscopy. Ultrasonography

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  • (PMID = 17252463.001).
  • [ISSN] 0013-726X
  • [Journal-full-title] Endoscopy
  • [ISO-abbreviation] Endoscopy
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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49. Altinova AE, Toruner F, Cimen AR, Karakoc A, Atasever T, Yetkin I, Ayvaz G, Cakir N, Arslan M: The association of neurofibromatosis, bilateral pheochromocytoma and primary hyperparathyroidism. Exp Clin Endocrinol Diabetes; 2007 Jul;115(7):468-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The association of neurofibromatosis, bilateral pheochromocytoma and primary hyperparathyroidism.
  • Neurofibromatosis (NF) is a hereditary disease and carries increased risk of both benign and malignant tumor development.
  • Pheochromocytoma or hyperparathyroidism have been reported to be associated with NF type 1 (NF1).
  • However, the coexistance of pheochromocytoma and parathyroid adenoma in a patient with NF1 is very rare.
  • We report a case of a 37-year-old male with NF1, bilateral pheochromocytoma and parathyroid adenoma.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Hyperparathyroidism, Primary / complications. Neurofibromatoses / complications. Pheochromocytoma / complications


50. Widimský J Jr: Recent advances in the diagnosis and treatment of pheochromocytoma. Kidney Blood Press Res; 2006;29(5):321-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Recent advances in the diagnosis and treatment of pheochromocytoma.
  • Pheochromocytoma (PHEO) is considered to be a rare cause of hypertension.
  • The diagnosis of PHEO is therefore extremely important.
  • Plasma-free metanephrines or urinary fractionated metanephrines seem to have higher diagnostic values compared to plasma or urinary catecholamines for the biochemical diagnosis of PHEO.
  • Imaging with (123)I-metaiodobenzylguanidine or (18)F-fluorodopamine PET, if available, are in addition to CT/MRI useful for the detection of multifocal/extra-adrenal forms.
  • Appropriate pharmacologic treatment with subsequent laparoscopic extirpation of PHEO is usually successful in benign forms.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis

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  • (PMID = 17119341.001).
  • [ISSN] 1420-4096
  • [Journal-full-title] Kidney & blood pressure research
  • [ISO-abbreviation] Kidney Blood Press. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 26
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51. Dossett LA, Rudzinski ER, Blevins LS, Chambers EP Jr: Malignant pheochromocytoma of the organ of Zuckerkandl requiring aortic and vena caval reconstruction. Endocr Pract; 2007 Sep;13(5):493-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant pheochromocytoma of the organ of Zuckerkandl requiring aortic and vena caval reconstruction.
  • OBJECTIVE: To describe a case of a malignant pheochromocytoma located in the organ of Zuckerkandl that required aortic and vena caval resection and reconstruction.
  • RESULTS: A 46-year-old man was referred for evaluation of a 1.4-cm left adrenal mass incidentally discovered on an abdominopelvic computed tomography (CT) scan.
  • Imaging characteristics of the left adrenal mass were consistent with a benign adenoma, but CT also demonstrated a hypervascular para-aortic mass.
  • Final pathologic and operative findings confirmed a malignant pheochromocytoma of the organ of Zuckerkandl with invasion into the wall of the inferior vena cava and tumor thrombus extending into the lumen.
  • CONCLUSION: Malignant pheochromocytoma of the organ of Zuckerkandl involving the aorta and inferior vena cava is exceedingly rare, and although surgical resection and reconstruction can be radical and aggressive, this treatment offers the only chance for cure.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Aorta, Abdominal / surgery. Para-Aortic Bodies / pathology. Pheochromocytoma / pathology. Pheochromocytoma / surgery. Vena Cava, Inferior / surgery

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  • (PMID = 17872352.001).
  • [ISSN] 1934-2403
  • [Journal-full-title] Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
  • [ISO-abbreviation] Endocr Pract
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Iodine Radioisotopes
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52. Elder EE, Elder G, Larsson C: Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor. J Surg Oncol; 2005 Mar 1;89(3):193-201
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor.
  • Recent developments in clinical and molecular research on these tumor forms have significantly clarified their genetic backgrounds and challenged the view of "pheochromocytoma as the 10% rule tumor."
  • Firstly, a larger proportion of these tumors are today discovered in normotensive patients during imaging carried out for other reasons than suspicion of pheochromocytoma.
  • Secondly, although the differential diagnosis between malignant and benign tumors remains a challenge, the risk of malignancy well exceeds the classical 10% in patients with extra-adrenal disease, and/or carriers of germ-line SDHB mutations.
  • Finally, up to a third of patients carry a germ-line mutation in a gene predisposing to pheochromocytoma and/or paraganglioma.
  • Genetic testing in apparently sporadic cases is therefore regarded as beneficial, especially in patients diagnosed before 50 years of age, and in patients with bilateral, multifocal, malignant and/or extra-adrenal disease.
  • [MeSH-major] Abdominal Neoplasms / genetics. Adrenal Gland Neoplasms / genetics. Genetic Testing. Germ-Line Mutation. Paraganglioma, Extra-Adrenal / genetics. Pheochromocytoma / genetics
  • [MeSH-minor] Diagnosis, Differential. Genetic Predisposition to Disease / genetics. Head and Neck Neoplasms / genetics. Humans. Membrane Proteins / genetics. Neurofibromatosis 1 / genetics. Succinate Dehydrogenase. Tumor Suppressor Proteins / genetics. Ubiquitin-Protein Ligases / genetics. Von Hippel-Lindau Tumor Suppressor Protein. von Hippel-Lindau Disease / genetics

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  • [Copyright] (c) 2005 Wiley-Liss, Inc.
  • (PMID = 15719371.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Membrane Proteins; 0 / SDHD protein, human; 0 / Tumor Suppressor Proteins; EC 1.3.99.1 / Succinate Dehydrogenase; EC 6.3.2.19 / Ubiquitin-Protein Ligases; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
  • [Number-of-references] 59
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53. Pradeep PV, Mishra AK, Aggarwal V, Bhargav PR, Gupta SK, Agarwal A: Adrenal cysts: an institutional experience. World J Surg; 2006 Oct;30(10):1817-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenal cysts: an institutional experience.
  • INTRODUCTION: Adrenal cysts are rare clinical entities.
  • We report our institutional experience with adrenal cysts and also assess various management options.
  • MATERIAL AND METHODS: Over the past 15 years the Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, has had seven cases of adrenal cysts, of which two were functional: one patient had Cushing's syndrome and the other patient had pheochromocytoma.
  • It is generally agreed that a hormonal work-up is necessary in all cases of adrenal cysts to rule out a sub-clinical disease.
  • Adrenal neoplasms, including adrenocortical carcinomas, can be associated with cysts that are benign in appearance.
  • However, surgical excision provides a definite histopathological diagnosis and also removes the fear of future complications such as hemorrhage into the cyst and local pressure effects due to the tumor.
  • CONCLUSIONS: Given that the adrenals are a vascular gland and taking into consideration the possibilities of bleeding and complications in the cyst, our treatment of choice is the elective excision of adrenal cysts.
  • [MeSH-major] Academies and Institutes / statistics & numerical data. Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / surgery. Adrenalectomy / methods. Cysts / diagnosis. Cysts / surgery
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Follow-Up Studies. Humans. India. Laparoscopy. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed

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  • (PMID = 16983481.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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54. Hasse-Lazar K, Krajewska J, Paliczka-Cieślik E, Jurecka-Lubieniecka B, Michalik B, Handkiewicz-Junak D, Roskosz J, Jarzab B: [131I-MIBG therapy in the treatment of pheochromocytoma in children--own experiences]. Endokrynol Pol; 2008 May-Jun;59(3):235-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [131I-MIBG therapy in the treatment of pheochromocytoma in children--own experiences].
  • Three cases of pheochromocytoma in children/adolescents or young adults treated by 131I-MIBG are presented in this study.
  • In one patient 131I-MIBG was administrated after ineffective surgical treatment and chemotherapy of a benign retroperitoneal tumor, whereas in two other patients 131I-MIBG therapy was carried out because of malignant pheochromocytoma dissemination.
  • The presented data confirm effectiveness and acceptable tolerance of 131I-MIBG treatment in pheochromocytoma, what is very important in pediatric patients.
  • [MeSH-major] 3-Iodobenzylguanidine / therapeutic use. Adrenal Gland Neoplasms / radiotherapy. Antineoplastic Agents / therapeutic use. Iodine Radioisotopes / therapeutic use. Pheochromocytoma / radiotherapy

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  • (PMID = 18615399.001).
  • [ISSN] 0423-104X
  • [Journal-full-title] Endokrynologia Polska
  • [ISO-abbreviation] Endokrynol Pol
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Iodine Radioisotopes; 35MRW7B4AD / 3-Iodobenzylguanidine
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55. Micali S, Peluso G, De Stefani S, Celia A, Sighinolfi MC, Grande M, Bianchi G: Laparoscopic adrenal surgery: new frontiers. J Endourol; 2005 Apr;19(3):272-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Laparoscopic adrenal surgery: new frontiers.
  • After about 10 years of experience, laparoscopic adrenalectomy has become the gold standard for the treatment of adrenal lesions.
  • Here, we describe the presenting features, imaging methods, and current surgical approaches to diseases of the adrenal gland.
  • There is general agreement on the suitability of the laparoscopic approach for benign adrenal lesions, but controversy exists about using laparoscopy for suspected adrenal malignancy, metastasis, and partial adrenalectomy.
  • In particular, we focus our attention on the new surgical approaches to the gland.

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  • (PMID = 15865511.001).
  • [ISSN] 0892-7790
  • [Journal-full-title] Journal of endourology
  • [ISO-abbreviation] J. Endourol.
  • [Language] ENG
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 67
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56. Widimský J Jr, Zelinka T, Petrák O, Strauch B, Safarík L, Kasalický M, Vranková A, Holaj R: [Diagnostic and therapeutic procedures in pheochromocytoma: current trends]. Vnitr Lek; 2007 Apr;53(4):428-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Diagnostic and therapeutic procedures in pheochromocytoma: current trends].
  • 24-hour monitoring of blood pressure (BP) can already contribute to the diagnosis of pheochromacytoma based on the frequent occurrence of BP variability and the absence of a night-time fall in BP.
  • The diagnosis of extraadrenal or multiple forms can use not only CT/MR but also imaging using the radiopharmaceutical 123I-Metaiodobenzylguanidine (MIBG) or 18F-fluorodopamine PET (only available in the USA).
  • Pharmacological treatment using alpha or beta receptor blockers with subsequent laparoscopic excision of the tumor is usually successful in benign forms of pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Humans. Hypertension / etiology. Multiple Endocrine Neoplasia Type 2a / diagnosis. Neurofibromatosis 1 / diagnosis. Paraganglioma / diagnosis. von Hippel-Lindau Disease / diagnosis. von Hippel-Lindau Disease / etiology

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  • (PMID = 17578179.001).
  • [ISSN] 0042-773X
  • [Journal-full-title] Vnitr̆ní lékar̆ství
  • [ISO-abbreviation] Vnitr Lek
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Czech Republic
  • [Number-of-references] 25
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57. Welsh SJ, Khan S: Radiological localizing techniques in adrenal tumors. Minerva Endocrinol; 2009 Jun;34(2):161-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Radiological localizing techniques in adrenal tumors.
  • The characterisation of adrenal lesions is a common radiological dilemma.
  • Incidental adrenal lesions are commonly detected with computed tomography (CT), and lesion characterisation is critical.
  • The prevalence of incidental adrenal lesions has been reported to be 2.3% at autopsy and 0.5-2% with abdominal CT.
  • Although the majority of adrenal lesions are benign, in patients with an extra-adrenal primary cancer the probability of an adrenal mass being a metastasis is 52%.
  • Unfortunately, there may be significant overlap between the imaging appearances of benign lesions such as lipid-poor adenomas and malignant lesions, particularly metastases and small adrenal carcinomas.
  • This review highlights recent advances in radiological imaging of adrenal lesions and we discuss the relative merits of CT and magnetic resonance imaging to aid the identification of benign and malignant adrenal lesions and their roles, in combination with biochemical and clinical data, in recognizing common pathologies such as adrenal adenoma, phaeochromocytoma, carcinoma and metastases.
  • We also discuss the radiological characteristics of rarer adrenal lesions including lymphoma, neuroblastic tumours (neuroblastoma, ganglioneuroblastoma, and ganglioneuroma), lipomatous tumours (myelolipoma, angiolipoma, teratoma, lipoma and liposarcoma), in addition to hemangioma, hemangiosarcoma and leiomyosarcoma.
  • [MeSH-major] Adrenal Gland Neoplasms / radiography. Incidental Findings. Tomography, X-Ray Computed
  • [MeSH-minor] Adrenal Cortex Neoplasms / radiography. Adrenocortical Adenoma / radiography. Adrenocortical Carcinoma / radiography. Diagnosis, Differential. Ganglioneuroblastoma / radiography. Ganglioneuroma / radiography. Humans. Lymphoma / radiography. Magnetic Resonance Imaging. Neoplasm Metastasis. Neoplasms, Adipose Tissue / radiography. Neoplasms, Vascular Tissue / radiography. Neuroblastoma / radiography. Pheochromocytoma / radiography. Predictive Value of Tests. Sensitivity and Specificity. Teratoma / radiography

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  • (PMID = 19471240.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 42
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58. Kasahara T, Nishiyama T, Takahashi K: Laparoscopic adrenalectomy for pheochromocytoma: evaluation of experience and strategy at a single institute. BJU Int; 2009 Jan;103(2):218-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Laparoscopic adrenalectomy for pheochromocytoma: evaluation of experience and strategy at a single institute.
  • OBJECTIVE: To assess the utility, safety and feasibility of laparoscopic adrenalectomy (LA) for pheochromocytoma.
  • PATIENTS AND METHODS: Between 1997 and 2007, 23 patients with pheochromocytoma underwent LA at our institution.
  • In the first cases of LA, we used an abdominal wall-lifting device with low pneumoperitoneal pressure and initial ligation of the adrenal vein.
  • In subsequent cases, we adopted similar methods to those we perform for other benign adrenal tumours, including a 'regular' pneumoperitoneal pressure, and disconnection of the adrenal vein as the last step of tumour dissection.
  • RESULTS: Rates of hypertension crisis (systolic blood pressure (sBP) of >200 mmHg) and sudden hypotension subsequent to ligation of the adrenal vein (sBP of <80 mmHg) were 17% and 48% in the LA group, and 44% and 72% in the OA group, respectively.
  • CONCLUSION: Laparoscopic removal of pheochromocytoma can be safely and feasibly accomplished by surgical approaches similar to those used for other pathological conditions of the adrenal gland.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy. Pheochromocytoma / surgery

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  • (PMID = 18671788.001).
  • [ISSN] 1464-410X
  • [Journal-full-title] BJU international
  • [ISO-abbreviation] BJU Int.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] England
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59. Brauckhoff M, Dralle H: [Recurrent operations on the adrenal glands]. Chirurg; 2005 Mar;76(3):227-37
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Recurrent operations on the adrenal glands].
  • Repeat adrenalectomy may be required due to ipsilateral recurrence of benign or malignant adrenal tumors after previous total or subtotal adrenalectomy.
  • Following subtotal adrenalectomy, the risk of ipsilateral recurrence correlates with disease, follow-up, localization, size of the adrenal remnant, and, in case of familial pheochromocytoma, probably with genotype.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Adrenocortical Carcinoma / surgery. Neoplasm Recurrence, Local / surgery. Pheochromocytoma / surgery
  • [MeSH-minor] 3-Iodobenzylguanidine / therapeutic use. Adult. Antineoplastic Agents, Hormonal / therapeutic use. Chemotherapy, Adjuvant. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Lymph Node Excision. Male. Middle Aged. Mitotane / therapeutic use. Octreotide / therapeutic use. Palliative Care. Paraneoplastic Endocrine Syndromes / diagnosis. Paraneoplastic Endocrine Syndromes / mortality. Paraneoplastic Endocrine Syndromes / surgery. Radiotherapy, Adjuvant. Reoperation

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  • (PMID = 15739057.001).
  • [ISSN] 0009-4722
  • [Journal-full-title] Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
  • [ISO-abbreviation] Chirurg
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 35MRW7B4AD / 3-Iodobenzylguanidine; 78E4J5IB5J / Mitotane; RWM8CCW8GP / Octreotide
  • [Number-of-references] 45
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60. Singh PK, Buch HN: Adrenal incidentaloma: evaluation and management. J Clin Pathol; 2008 Nov;61(11):1168-73
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenal incidentaloma: evaluation and management.
  • Adrenal incidentalomas are adrenal masses discovered incidental to imaging studies performed for reasons unrelated to adrenal pathology.
  • Although most adrenal incidentalomas are non-functioning benign adenomas, their increasing prevalence presents diagnostic and therapeutic challenges.
  • The assessment of adrenal incidentalomas is aimed at deciding whether or not the tumour should be surgically removed.
  • Biochemical screening for tumour hypersecretion is mandatory in all adrenal incidentalomas, since hormone secreting tumours may be clinically silent.
  • The diagnosis of phaeochromocytoma is of paramount importance because of its life-threatening complications.
  • Non-functioning adrenal incidentalomas need assessment for risk of malignancy, and this is based on the size of the tumour and its imaging characteristics.
  • The duration and frequency of reassessment remains unclear, as the natural history of adrenal incidentalomas has yet to be clearly defined, and there is a lack of controlled studies comparing surgical intervention with observation.
  • However, the possibility of acquiring autonomous hypersecretion or conversion to malignancy in an incidentaloma diagnosed to be a benign non-functioning lesion is very low, and most patients may be safely discharged after an initial follow-up period of 2 years.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis
  • [MeSH-minor] Adenoma / diagnosis. Diagnosis, Differential. Humans. Incidental Findings. Long-Term Care / methods. Pheochromocytoma / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 18955573.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 117
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61. Mannelli M, Colagrande S, Valeri A, Parenti G: Incidental and metastatic adrenal masses. Semin Oncol; 2010 Dec;37(6):649-61
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Incidental and metastatic adrenal masses.
  • In the last decades discoveries of adrenal masses incidentally during the course of diagnostic procedures for unrelated disorders (incidentalomas) have become progressively more frequent.
  • The clinician in this position must answer two main questions: Is the mass benign or malignant?
  • , and To what extent is the adrenal secretion altered?
  • To come to a clinical decision, several diagnostic tools need to be engaged, starting with an accurate and correct radiological evaluation and a hormonal assessment of the adrenal function.
  • Most frequently, adrenal incidentalomas (AIs) are represented by benign cortical adenomas, a subset of which causes a mild hypercortisolism, known as subclinical Cushing's syndrome (SCS).
  • Laparoscopic surgery is the recommended procedure to remove benign masses.
  • The surgical procedure for adrenal malignancies is still debated.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Incidental Findings
  • [MeSH-minor] Adenoma / diagnosis. Adrenal Cortex Hormones / secretion. Adrenal Gland Diseases / diagnosis. Adrenalectomy. Adrenocortical Carcinoma / diagnosis. Algorithms. Diagnosis, Differential. Humans. Myelolipoma / diagnosis. Pheochromocytoma / diagnosis. Tomography, X-Ray Computed / methods

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  • [Copyright] Copyright © 2010 Elsevier Inc. All rights reserved.
  • (PMID = 21167383.001).
  • [ISSN] 1532-8708
  • [Journal-full-title] Seminars in oncology
  • [ISO-abbreviation] Semin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones
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62. Kuruba R, Gallagher SF: Current management of adrenal tumors. Curr Opin Oncol; 2008 Jan;20(1):34-46
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Current management of adrenal tumors.
  • PURPOSE OF REVIEW: Adrenal tumors evoke considerable interest and diagnostic challenges.
  • RECENT FINDINGS: Significant advances have been made in diagnostic imaging modalities for identifying malignancy risk in adrenal incidentalomas.
  • SUMMARY: Laparoscopic adrenalectomy has emerged as standard of care in the treatment of functional benign adenomas and nonfunctional tumors larger than 4 cm when adrenocortical carcinoma is not suspected.
  • International consensus conferences have attempted to standardize diagnostic and treatment approaches in the management of adrenal tumors; further research is necessary.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / drug therapy
  • [MeSH-minor] Adrenal Glands / pathology. Adrenal Glands / radiography. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / drug therapy. Adrenocortical Carcinoma / genetics. Chemotherapy, Adjuvant. Humans. Pheochromocytoma / diagnosis. Pheochromocytoma / drug therapy. Prognosis. Tomography, X-Ray Computed

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  • (PMID = 18043254.001).
  • [ISSN] 1040-8746
  • [Journal-full-title] Current opinion in oncology
  • [ISO-abbreviation] Curr Opin Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 68
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63. Terzolo M, Bovio S, Pia A, Reimondo G, Angeli A: Management of adrenal incidentaloma. Best Pract Res Clin Endocrinol Metab; 2009 Apr;23(2):233-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of adrenal incidentaloma.
  • Clinically inapparent adrenal masses, or adrenal incidentalomas, are discovered inadvertently in the course of work-up or treatment of unrelated disorders.
  • Two critical questions should be answered before trying to outline the management of adrenal incidentaloma:.
  • (1) identify either primary (adrenocortical cancer) or secondary (adrenal metastasis) malignancy;.
  • Radiological evaluation is the key to the differential diagnosis of benign and malignant tumours.
  • Endocrine testing is necessary to exclude phaeochromocytoma in all patients with an adrenal incidentaloma because this tumour may remain undiagnosed after imaging studies.
  • The management of clinically inapparent adrenal adenomas may vary depending whether or not they are functioning.
  • [MeSH-major] Adrenal Gland Neoplasms / therapy
  • [MeSH-minor] Adenoma / diagnosis. Adenoma / drug therapy. Adenoma / therapy. Animals. Humans. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 19500766.001).
  • [ISSN] 1878-1594
  • [Journal-full-title] Best practice & research. Clinical endocrinology & metabolism
  • [ISO-abbreviation] Best Pract. Res. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 46
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64. Nürnberg D: [Ultrasound of adrenal gland tumours and indications for fine needle biopsy (uFNB)]. Ultraschall Med; 2005 Dec;26(6):458-69
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Ultrasound of adrenal gland tumours and indications for fine needle biopsy (uFNB)].
  • The normal adrenal glands can be detected by high-resolution-sonography in a high percentage of cases.
  • Sonography is also highly sensitive in the diagnosis of tumours of the adrenal glands.
  • Amongst the benign tumours, adenomas are the most frequent (up to 8%).
  • The adrenal glands are the fourth most common location of metastases.
  • Much less frequent are infiltrates of lymphomas in adrenal glands tumours (3.4%).
  • Incidentalomas are accidentally detected tumours of the adrenal glands without clinical symptoms.
  • The uFNB of the adrenal glands has a high sensitivity (90-95%).
  • Recommendations for this procedure in accidentally detected tumours of the adrenal glands: < 3 cm and hormonally inactive --> US-follow up, > 5 cm + suspected tumour --> surgery, 3-5 cm uFNB after laboratory diagnostics.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / ultrasonography. Biopsy, Fine-Needle
  • [MeSH-minor] Adrenal Glands / cytology. Adrenal Glands / pathology. Adrenal Glands / ultrastructure. Humans. Lymphoma / pathology. Lymphoma / ultrasonography. Pheochromocytoma / pathology. Pheochromocytoma / ultrasonography

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  • (PMID = 16453217.001).
  • [ISSN] 0172-4614
  • [Journal-full-title] Ultraschall in der Medizin (Stuttgart, Germany : 1980)
  • [ISO-abbreviation] Ultraschall Med
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 116
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65. Bahrami A, Truong LD, Shen SS, Krishnan B: Synchronous renal and adrenal masses: an analysis of 80 cases. Ann Diagn Pathol; 2009 Feb;13(1):9-15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Synchronous renal and adrenal masses: an analysis of 80 cases.
  • Synchronous renal and adrenal masses are uncommon.
  • Although adrenal masses in the context of renal cell carcinoma (RCC) are often suspected as metastasis, other adrenal lesions with different therapeutic and prognostic implications may coexist with RCC.
  • In a retrospective review of 550 radical nephrectomies with ipsilateral adrenalectomy, 80 cases of coexisting renal and adrenal masses were identified.
  • The renal masses included 76 RCCs, 3 oncocytomas, and 1 malignant pheochromocytoma of adrenal gland involving the kidney.
  • Although the gross pathologic impression of adrenal masses in the presence of RCC was metastasis, on histologic examination, 56% of them were found to be benign lesions (mostly adrenal adenoma/hyperplasia), whereas malignant involvement from RCC was seen in 43%.
  • The benign and malignant nature of the adrenal lesions in the context of RCC could not be discriminated based on the size of adrenal mass.
  • Because of the prognostic implication of direct or metastatic involvement of adrenal gland in the setting of RCC and the possibility of finding small metastatic foci, a meticulous gross and microscopic examination of adrenal glands is emphasized.
  • Rare unusual combinations of renal and adrenal lesions such as RCC and adrenal histoplasmosis, RCC and adrenal myelolipoma, renal oncocytoma, and adrenal pheochromocytoma are also described.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Glands / pathology. Carcinoma, Renal Cell / pathology. Kidney / pathology. Kidney Neoplasms / pathology
  • [MeSH-minor] Adenoma, Oxyphilic / pathology. Adenoma, Oxyphilic / surgery. Adrenalectomy. Adult. Aged. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Neoplasm Staging. Nephrectomy. Pheochromocytoma / pathology. Pheochromocytoma / surgery. Retrospective Studies

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  • (PMID = 19118776.001).
  • [ISSN] 1532-8198
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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66. Hasani-Ranjbar S, Amoli MM, Ebrahim-Habibi A, Haghpanah V, Hejazi M, Soltani A, Larijani B: Mutation screening of VHL gene in a family with malignant bilateral pheochromocytoma: from isolated familial pheochromocytoma to von Hippel-Lindau disease. Fam Cancer; 2009;8(4):465-71
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mutation screening of VHL gene in a family with malignant bilateral pheochromocytoma: from isolated familial pheochromocytoma to von Hippel-Lindau disease.
  • von Hippel-Lindau (vHL) disease is an inherited, autosomal dominant syndrome manifested by a variety of benign and malignant tumors.
  • It was detected in a family with bilateral malignant pheochromocytoma who has been followed for at least 9 years as RET negative isolated familial pheochromocytoma, finally diagnosed as von Hipple-Lindau disease according to retinal angioma and VHL gene mutation.
  • VHL type 2 presenting with both pheochromocytoma and retinal angioma in this family found to be associated with the new missense mutation (c499 C>T) of VHL gene.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics. von Hippel-Lindau Disease / genetics

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  • (PMID = 19649731.001).
  • [ISSN] 1573-7292
  • [Journal-full-title] Familial cancer
  • [ISO-abbreviation] Fam. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] EC 2.3.2.27 / Von Hippel-Lindau Tumor Suppressor Protein; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 6.3.2.- / VHL protein, human
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67. Tica I, Tica VI, Mihailov C: Rare association of adrenal tumors. Rom J Intern Med; 2007;45(1):107-11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rare association of adrenal tumors.
  • Adrenal incidentalomas represent a true problem both in the clinical diagnosis and in their treatment.
  • A great variety of pathologies may be found under the umbrella of this concept: benign adenomas - functioning or not, myelolipomas, hamartomas, or granulomatous infiltrations of the adrenal.
  • The possibility of malignancy should be considered in each case, especially in patients with a known extra-adrenal primary.
  • We present an interesting case because of the surprising association of two adrenal tumors, with a long time lapse between them, with ascites and pleurisy and because of the difficulty of treatment in a patient refusing surgery.
  • Miss MR, 61 years old, was treated surgically for pheochromocytoma 28 years ago (left adrenalectomy).
  • A right adrenal incidentaloma of 21/15 mm - in association with ascites and pleurisy - was found at CT scan.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Adrenal Gland Neoplasms / diagnosis. Ascites / complications. Pheochromocytoma / complications. Pleurisy / complications

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  • (PMID = 17966451.001).
  • [ISSN] 1220-4749
  • [Journal-full-title] Romanian journal of internal medicine = Revue roumaine de médecine interne
  • [ISO-abbreviation] Rom J Intern Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Romania
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68. Wagnerova H, Dudasova D, Lazurova I: Hormonal and metabolic evaluation of adrenal incidentalomas. Neoplasma; 2009;56(6):521-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hormonal and metabolic evaluation of adrenal incidentalomas.
  • The biochemical and hormonal data in patients with adrenal incidentalomas were evaluated to compare the differences between adrenal adenomas and other benign lesions and to find the relationship between metabolic parameters and adrenal hormones.
  • Ninety two patients (29men, age 20-90 years) with incidentally discovered unilateral or bilateral adrenal masses detected on CT were included in this study for the reasons others than adrenal pathology.
  • In the group of patients with adrenal masses the prevalence of arterial hypertension was three fold higher, the prevalence of DM was approximately five fold higher and the prevalence of the overweight and obesity two fold higher than is reported in the general population.
  • The most frequent adrenal masses were nonfunctional masses, the occurence of functional lesions was as follows: steroid enzymopathies (an exaggerated response of 17-OHP indicating a possible 21-hydroxylase deficiency), subclinical Cushing syndrome, primary aldosteronism and pheochromocytoma (5%, 2%, 2% and 1% respectively).
  • In this study we confirmed a higher prevalence of symptoms characteristic for different metabolic syndromes in these patients with adrenal incidentalomas, which indicate systematic screening for the metabolic syndrome including evaluation of the insuline resistance in this patients.
  • [MeSH-major] Adenoma / metabolism. Adrenal Gland Neoplasms / metabolism. Hormones / analysis. Metabolic Syndrome X / metabolism

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  • [CommentIn] J Urol. 2010 Nov;184(5):1925 [22519994.001]
  • (PMID = 19728761.001).
  • [ISSN] 0028-2685
  • [Journal-full-title] Neoplasma
  • [ISO-abbreviation] Neoplasma
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Slovakia
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Hormones; 0 / Triglycerides; 5001-33-2 / Metanephrine; 97C5T2UQ7J / Cholesterol; EC 1.14.99.10 / Steroid 21-Hydroxylase; EC 3.4.23.15 / Renin
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69. El-Daly H, Rao P, Palazzo F, Gudi M: A rare entity of an unusual site: adenomatoid tumour of the adrenal gland: a case report and review of the literature. Patholog Res Int; 2010;2010:702472
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A rare entity of an unusual site: adenomatoid tumour of the adrenal gland: a case report and review of the literature.
  • This is a case report of a 51 year old male who was found to have an incidental left sided non-functioning adrenal mass on routine medical examination and which was confirmed by CT and MRI scans.
  • There was focal extension to capsule and peri-adrenal fat.
  • A diagnosis of an adenomatoid tumour as made.
  • Adenomatoid tumours are rare benign tumours of mesothelial derivation.
  • The adrenal gland is devoid of a mesothelial lining and the most accepted hypothesis for an adenomatoid tumour originating in the adrenal gland is derivation from mesothelial rests.
  • As the adrenal gland is an extremely rare site of occurrence for an adenomatoid tumour, it is frequently mistaken for adrenocortical tumours or a pheochromocytoma clinically and radiologically.

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  • (PMID = 21151721.001).
  • [ISSN] 2042-003X
  • [Journal-full-title] Pathology research international
  • [ISO-abbreviation] Patholog Res Int
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2990199
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70. Beuschlein F: Adrenal incidentalomas: presentation and clinical work-up. Horm Res; 2007;68 Suppl 5:191-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenal incidentalomas: presentation and clinical work-up.
  • BACKGROUND: Through the widespread use of imaging techniques with great sensitivity, adrenal tumours are often diagnosed as an incidental finding.
  • The majority of these adrenal lesions are benign and without evidence of endocrine activity or malignancy.
  • However, in addition to the classic forms of overt adrenal hypersecretion, it has become evident in recent years that even modest adrenal hormonal autonomy, as exhibited in clinically silent phaeochromocytoma, normokalaemic primary aldosteronism and subclinical Cushing syndrome, is associated with significant morbidity.
  • CONCLUSIONS: Detection and differential diagnosis of subtle changes in adrenal hormone secretion can pose a diagnostic challenge to the clinician, and accurate diagnosis is dependent on use of tests with reliable sensitivity and specificity.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Adrenal Gland Neoplasms / diagnosis. Incidental Findings
  • [MeSH-minor] Adrenocortical Carcinoma / complications. Adrenocortical Carcinoma / diagnosis. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Pheochromocytoma / complications. Pheochromocytoma / diagnosis. Tomography, X-Ray Computed

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  • [Copyright] Copyright (c) 2007 S. Karger AG, Basel.
  • (PMID = 18174744.001).
  • [ISSN] 1423-0046
  • [Journal-full-title] Hormone research
  • [ISO-abbreviation] Horm. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 42
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71. van Nederveen FH, Korpershoek E, deLeeuw RJ, Verhofstad AA, Lenders JW, Dinjens WN, Lam WL, de Krijger RR: Array-comparative genomic hybridization in sporadic benign pheochromocytomas. Endocr Relat Cancer; 2009 Jun;16(2):505-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Array-comparative genomic hybridization in sporadic benign pheochromocytomas.
  • Pheochromocytomas (PCC) are catecholamine-producing tumors arising from the adrenal medulla that occur either sporadically or in the context of hereditary cancer syndromes, such as multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau disease (VHL), neurofibromatosis type 1, and the PCC-paraganglioma syndrome.
  • The development of a submegabase tiling resolution array enabled us to perform a genome-wide high-resolution analysis of 36 sporadic benign PCC.
  • We conclude that there appear to be two subgroups of benign sporadic PCC, one of which has a pattern of chromosomal abnormalities that is comparable with PCC from patients with MEN2 and the other that is comparable with the PCC that arise in patients with VHL disease.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Chromosome Aberrations. Comparative Genomic Hybridization. Oligonucleotide Array Sequence Analysis. Pheochromocytoma / genetics

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  • (PMID = 19153209.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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72. Kobayashi H, Kaneko G, Nishimoto K, Uchida A: [A case of pheochromocytoma associated with neurofibromatosis type 1]. Hinyokika Kiyo; 2009 Dec;55(12):749-52
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  • [Title] [A case of pheochromocytoma associated with neurofibromatosis type 1].
  • Pheochromocytoma occurs in 0.1 to 5.7% of patients with type 1 neurofibromatosis (NF1).
  • Radiological findings of pheochromocytoma are often similar to those of neurofibroma; therefore, any pheochromocytoma should be excised in hypertensive patients with NF1.
  • A 60-year-old male patient with NF1 was referred to this hospital for an incidentally discovered right adrenal mass, 7 x 6 mm in diameter.
  • The patient had multiple benign tumors and suffered from hypertension for 4 years.
  • A histopathological diagnosis of pheochromocytoma was made.
  • Screening of the adrenal tumor is strongly recommended for NF1 patients with hypertension, since any unfavorable events due to catecholamine such as cardiomyopathy and fatal arrhythmia can be avoided by adequate surgical intervention.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Neurofibromatosis 1 / complications. Pheochromocytoma / complications


73. Imanaka M, Iida K, Takahashi K, Tsuji K, Nishizawa H, Fukuoka H, Takeno R, Takahashi Y, Okimura Y, Kaji H, Chihara K: The N131S mutation in the von Hippel-Lindau gene in a Japanese family with pheochromocytoma and hemangioblastomas. Endocr J; 2006 Dec;53(6):819-27
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  • [Title] The N131S mutation in the von Hippel-Lindau gene in a Japanese family with pheochromocytoma and hemangioblastomas.
  • von Hippel-Lindau (VHL) disease (VHLD) is a hereditary autosomal dominant syndrome that causes various benign and malignant tumors.
  • Here, we report a mutation in the VHL gene in a Japanese family with VHLD type 2A, characterized by pheochromocytoma (PHE), and hemangioblastomas (HAB) in both the retina and thoracic spinal cord but without renal cell carcinoma (RCC).
  • We also identified somatic loss of heterozygosity (LOH) at chromosome 3p25-26 in the adrenal tumor of the patient.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Cerebellar Neoplasms / genetics. Hemangioblastoma / genetics. Mutation. Pheochromocytoma / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • (PMID = 17001110.001).
  • [ISSN] 0918-8959
  • [Journal-full-title] Endocrine journal
  • [ISO-abbreviation] Endocr. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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74. Timmers HJ, Gimenez-Roqueplo AP, Mannelli M, Pacak K: Clinical aspects of SDHx-related pheochromocytoma and paraganglioma. Endocr Relat Cancer; 2009 Jun;16(2):391-400
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  • [Title] Clinical aspects of SDHx-related pheochromocytoma and paraganglioma.
  • Paragangliomas (PGLs) derive from either sympathetic chromaffin tissue in adrenal and extra-adrenal abdominal or thoracic locations, or from parasympathetic tissue of the head and neck.
  • SDHB mutations mainly predispose to extra-adrenal, and to a lesser extent, adrenal PGLs, with a high malignant potential, but also head and neck paragangliomas (HNPGL).
  • SDHD mutations are typically associated with multifocal HNPGL and usually benign adrenal and extra-adrenal PGLs.
  • The identification of SDHx mutations in patients with PGL is warranted for a tailor-made approach to the biochemical diagnosis, imaging, treatment, follow-up, and family screening.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics. Succinate Dehydrogenase / genetics

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  • (PMID = 19190077.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 HD008735-08
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase
  • [Number-of-references] 54
  • [Other-IDs] NLM/ NIHMS750209; NLM/ PMC4711350
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75. Meyer-Rochow GY, Jackson NE, Conaglen JV, Whittle DE, Kunnimalaiyaan M, Chen H, Westin G, Sandgren J, Stålberg P, Khanafshar E, Shibru D, Duh QY, Clark OH, Kebebew E, Gill AJ, Clifton-Bligh R, Robinson BG, Benn DE, Sidhu SB: MicroRNA profiling of benign and malignant pheochromocytomas identifies novel diagnostic and therapeutic targets. Endocr Relat Cancer; 2010 Sep;17(3):835-46
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MicroRNA profiling of benign and malignant pheochromocytomas identifies novel diagnostic and therapeutic targets.
  • There is increasing evidence to suggest that miRNAs could be useful in cancer diagnosis, prognosis, and therapy.
  • We performed miRNA microarray expression profiling on a cohort of 12 benign and 12 malignant pheochromocytomas and identified a number of differentially expressed miRNAs.
  • These results were validated in a separate cohort of ten benign and ten malignant samples using real-time quantitative RT-PCR; benign samples had a minimum follow-up of at least 2 years.
  • It was found that IGF2 as well as its intronic miR-483-5p was over-expressed, while miR-15a and miR-16 were under-expressed in malignant tumours compared with benign tumours.
  • These miRNAs were found to be diagnostic and prognostic markers for malignant pheochromocytoma.
  • The functional role of miR-15a and miR-16 was investigated in vitro in the rat PC12 pheochromocytoma cell line, and these miRNAs were found to regulate cell proliferation via their effect on cyclin D1 and apoptosis.
  • These data indicate that miRNAs play a pivotal role in the biology of malignant pheochromocytoma, and represent an important class of diagnostic and prognostic biomarkers and therapeutic targets warranting further investigation.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Biomarkers, Tumor / genetics. Gene Expression Profiling. Gene Expression Regulation, Neoplastic. MicroRNAs / physiology. Pheochromocytoma / genetics
  • [MeSH-minor] Adrenal Glands / metabolism. Adrenal Glands / pathology. Animals. Apoptosis. Blotting, Western. Cell Cycle. Cohort Studies. Follow-Up Studies. Humans. Oligonucleotide Array Sequence Analysis. Prognosis. RNA, Messenger / genetics. Rats. Reverse Transcriptase Polymerase Chain Reaction. Survival Rate. Tumor Cells, Cultured


76. Martiniova L, Lai EW, Elkahloun AG, Abu-Asab M, Wickremasinghe A, Solis DC, Perera SM, Huynh TT, Lubensky IA, Tischler AS, Kvetnansky R, Alesci S, Morris JC, Pacak K: Characterization of an animal model of aggressive metastatic pheochromocytoma linked to a specific gene signature. Clin Exp Metastasis; 2009;26(3):239-50
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  • [Title] Characterization of an animal model of aggressive metastatic pheochromocytoma linked to a specific gene signature.
  • There is presently no cure for metastatic pheochromocytoma and no reliable way to distinguish malignant from benign tumors before the development of metastases.
  • In order to successfully manage pheochromocytoma, it is necessary to better understand the biological determinants of tumor behavior.
  • For this purpose, we have recently established a mouse model of metastatic pheochromocytoma using tail vein injection of mouse pheochromocytoma (MPC) cells.
  • Serial harvest, culture and re-selection of metastatic liver lesions produced even more aggressive pheochromocytoma cells that retained their biochemical phenotype.

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  • [Cites] Int J Cancer. 2005 Mar 10;114(1):139-43 [15523699.001]
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  • (PMID = 19169894.001).
  • [ISSN] 1573-7276
  • [Journal-full-title] Clinical & experimental metastasis
  • [ISO-abbreviation] Clin. Exp. Metastasis
  • [Language] ENG
  • [Grant] United States / Intramural NIH HHS / / ZIC HG200365-03; United States / Intramural NIH HHS / / ZIC HG200365-01; United States / NINDS NIH HHS / NS / R01 NS037685; United States / NINDS NIH HHS / NS / NS 37685; United States / Intramural NIH HHS / / ZIC HG200365-02
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Other-IDs] NLM/ NIHMS420223; NLM/ PMC3505859
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77. Wängberg B, Muth A, Khorram-Manesh A, Jansson S, Nilsson O, Forssell-Aronsson E, Tisell L, Ahlman H: Malignant pheochromocytoma in a population-based study: survival and clinical results. Ann N Y Acad Sci; 2006 Aug;1073:512-6
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  • [Title] Malignant pheochromocytoma in a population-based study: survival and clinical results.
  • One hundred fifty-four consecutive patients with pheochromocytoma (PC, n=137) or paraganglioma (PG, n=17) were treated at our unit.
  • Within 4 years two patients died of tumor progression. (2) Locally advanced disease (n=4), all resected for cure. (3) Malignancy disclosed during follow-up after adrenalectomy with "benign" histopathology (n=4).
  • Active surgical treatment and phenoxybenzamine resulted in low tumor-related mortality in groups 2 and 3; five patients died 8-30 years after diagnosis, four of PC/PG (three from group 2 and one from group 3) and one of other causes.
  • To diagnose PC/PG early, screening of adrenal incidentalomas has been suggested.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology

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  • (PMID = 17102119.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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78. Guillemot J, Compagnon P, Cartier D, Thouennon E, Bastard C, Lihrmann I, Pichon P, Thuillez C, Plouin PF, Bertherat J, Anouar Y, Kuhn JM, Yon L, Lefebvre H: Metoclopramide stimulates catecholamine- and granin-derived peptide secretion from pheochromocytoma cells through activation of serotonin type 4 (5-HT4) receptors. Endocr Relat Cancer; 2009 Mar;16(1):281-90
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metoclopramide stimulates catecholamine- and granin-derived peptide secretion from pheochromocytoma cells through activation of serotonin type 4 (5-HT4) receptors.
  • The aim of the study was to investigate the mechanism of action of metoclopramide and expression of serotonin type 4 (5-HT(4)) receptors in pheochromocytoma tissues.
  • Tissue explants, obtained from 18 pheochromocytomas including the tumor removed from a 46-year-old female patient who experienced life-threatening hypertension crisis after metoclopramide administration and 17 additional pheochromocytomas (9 benign and 8 malignant) were studied.
  • Cultured pheochromocytoma cells derived from the patient who previously received metoclopramide were incubated with metoclopramide and various 5-HT(4) receptor ligands.
  • Catecholamine- and granin-derived peptide concentrations were measured in pheochromocytoma cell incubation medium by HPLC and radioimmunological assays.
  • All 5-HT(4) receptor agonists must therefore be contraindicated in patients with proven or suspected pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Dopamine Antagonists / pharmacology. Metoclopramide / pharmacology. Pheochromocytoma / drug therapy. Receptors, Serotonin, 5-HT4 / genetics
  • [MeSH-minor] Adrenal Medulla / cytology. Adrenal Medulla / drug effects. Catecholamines / secretion. Chromogranins / secretion. Cisapride / pharmacology. Domperidone / pharmacology. Female. Humans. Middle Aged. RNA, Messenger / metabolism. Retrospective Studies. Reverse Transcriptase Polymerase Chain Reaction. Serotonin Receptor Agonists / pharmacology. Tumor Cells, Cultured

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  • (PMID = 18948374.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Chromogranins; 0 / Dopamine Antagonists; 0 / RNA, Messenger; 0 / Serotonin Receptor Agonists; 158165-40-3 / Receptors, Serotonin, 5-HT4; 5587267Z69 / Domperidone; L4YEB44I46 / Metoclopramide; UVL329170W / Cisapride
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79. Guillemot J, Anouar Y, Montero-Hadjadje M, Grouzmann E, Grumolato L, Roshmaninho-Salgado J, Turquier V, Duparc C, Lefebvre H, Plouin PF, Klein M, Muresan M, Chow BK, Vaudry H, Yon L: Circulating EM66 is a highly sensitive marker for the diagnosis and follow-up of pheochromocytoma. Int J Cancer; 2006 Apr 15;118(8):2003-12
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  • [Title] Circulating EM66 is a highly sensitive marker for the diagnosis and follow-up of pheochromocytoma.
  • We have previously demonstrated that measurement of tissue concentration of the novel secretogranin II-derived peptide EM66 may help to discriminate between benign and malignant pheochromocytomas.
  • The aim of the present study was to characterize EM66 in plasma and urine of healthy volunteers and pheochromocytoma patients, in order to further evaluate the usefulness of this peptide as a circulating marker for the management of the tumors.
  • In patients with pheochromocytoma, plasma EM66 levels were 10-fold higher than those of healthy volunteers (26.9 (7.3-44) ng/ml), and returned to normal values after removal of the tumor.
  • These data indicate that EM66 is secreted in the general circulation and that elevated plasma EM66 levels are correlated with the occurrence of pheochromocytoma.
  • Thus, EM66 is a sensitive plasma marker that should be considered as a complementary tool in the management of pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / pathology. Biomarkers, Tumor / blood. Chromogranins / blood. Chromogranins / urine. Peptide Fragments / blood. Pheochromocytoma / diagnosis. Pheochromocytoma / pathology. Secretogranin II / blood
  • [MeSH-minor] Adult. Aged. Case-Control Studies. Chromatography, High Pressure Liquid. Diagnosis, Differential. Female. Humans. Immunoassay. Male. Metanephrine. Middle Aged. Sensitivity and Specificity

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  • [Copyright] Copyright (c) 2005 Wiley-Liss, Inc.
  • (PMID = 16287097.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chromogranins; 0 / EM66 peptide; 0 / Peptide Fragments; 0 / Secretogranin II; 5001-33-2 / Metanephrine
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80. Stelow EB, Debol SM, Stanley MW, Mallery S, Lai R, Bardales RH: Sampling of the adrenal glands by endoscopic ultrasound-guided fine-needle aspiration. Diagn Cytopathol; 2005 Jul;33(1):26-30
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  • [Title] Sampling of the adrenal glands by endoscopic ultrasound-guided fine-needle aspiration.
  • Endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) has proven to be a valuable modality for the primary diagnosis and staging of gastrointestinal, and perigastrointestinal malignancy.
  • Aside from assessing thoracic and abdominal lymph nodes and the liver for metastases, EUS can assess and sample the adrenal glands, which are frequently involved by metastatic disease, but can also harbor benign primary neoplasms.
  • The cytology files at our institution were reviewed for all cases of EUS-guided FNA of the adrenal glands.
  • Results were compared with overall EUS-guided FNA performance and the performance of non-EUS-guided FNA of the adrenal.
  • Between 1/1/00 and 5/15/04 there were 24 cases of EUS-guided FNA of the adrenal gland from 22 different patients (13 men; 9 women) at our institution.
  • This represented 1.4% of overall EUS-guided FNA and 77% of adrenal gland FNA.
  • Almost all FNAs were of the left adrenal gland (23 of 24).
  • Final diagnoses were as follows: cortical tissue consistent with cortical adenoma (19), metastatic adenocarcinoma (3), pheochromocytoma (1), and adrenal cortical carcinoma (1).
  • EUS-guided FNA of the adrenal gland is primarily used in the staging of other malignancies when lesions of the left adrenal are recognized sonographically.
  • Diagnostic tissue is easily obtained, including material for cell block IHC, which allows definitive diagnosis in cases that present difficult differential diagnoses.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Glands / pathology. Biopsy, Fine-Needle / methods. Endosonography / methods
  • [MeSH-minor] Adenocarcinoma / diagnosis. Adenoma / diagnosis. Adrenocortical Carcinoma / diagnosis. Adult. Aged. Aged, 80 and over. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Pheochromocytoma / diagnosis. Sensitivity and Specificity

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  • [Copyright] 2005 Wiley-Liss, Inc
  • (PMID = 15945088.001).
  • [ISSN] 8755-1039
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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81. Mazzaglia PJ, Monchik JM: Limited value of adrenal biopsy in the evaluation of adrenal neoplasm: a decade of experience. Arch Surg; 2009 May;144(5):465-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Limited value of adrenal biopsy in the evaluation of adrenal neoplasm: a decade of experience.
  • OBJECTIVE: To determine the value of percutaneous adrenal biopsy in the evaluation of adrenal neoplasm.
  • PATIENTS: All adult patients undergoing image-guided adrenal biopsy from 1997 to 2007.
  • Eighty-eight biopsies (53.4%) were performed in patients with a prior diagnosis of cancer.
  • Forty-five (26.4%) were performed when imaging study results suggested previously undiagnosed cancer with a simultaneous adrenal metastasis.
  • Thirty (20.2%) were performed for isolated adrenal incidentalomas.
  • Prebiopsy evaluation for pheochromocytoma was performed in less than 5% of patients with established or suspected nonadrenal malignancies and 32% of patients with incidentalomas.
  • In patients with isolated adrenal incidentaloma, a radiology report recommended biopsy 33% of the time for characteristics inconsistent with benign adenoma.
  • Benign incidentalomas measured mean (SD) 4.2 (2.1) cm (range, 1.4-10.7 cm), and malignancies measured mean (SD) 9.3 (3.3) cm (range, 5.3-14 cm) (P < .05).
  • All incidentalomas 5 cm or less (n = 18) were benign.
  • There were 4 false-negative biopsy results: 3 adrenocortical carcinomas and 1 pheochromocytoma.
  • CONCLUSIONS: Biopsy is unhelpful in patients with isolated adrenal incidentaloma.
  • Despite atypical radiographic findings, all nonfunctioning nodules 5 cm or less were benign.
  • The value of biopsy remains the diagnosis of metastatic carcinoma in patients with a nonadrenal primary malignancy, proven by the more than 70% positive rate in this group.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Biopsy / methods. Pheochromocytoma / pathology

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  • (PMID = 19451490.001).
  • [ISSN] 1538-3644
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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82. Guillemot J, Barbier L, Thouennon E, Vallet-Erdtmann V, Montero-Hadjadje M, Lefebvre H, Klein M, Muresan M, Plouin PF, Seidah N, Vaudry H, Anouar Y, Yon L: Expression and processing of the neuroendocrine protein secretogranin II in benign and malignant pheochromocytomas. Ann N Y Acad Sci; 2006 Aug;1073:527-32
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  • [Title] Expression and processing of the neuroendocrine protein secretogranin II in benign and malignant pheochromocytomas.
  • The aim of the present study was to compare the expression levels of secretogranin II (SgII), prohormone convertases (PC)1 and PC2, and the proteolytic processing of SgII in benign versus malignant pheochromocytomas.
  • Quantitative (Q)-PCR experiments indicated that SgII, PC1, and PC2 mRNAs were overexpressed in pheochromocytoma compared to non-tumoral chromaffin cells (P<0.001) and in benign compared to malignant tumors (P<0.01).
  • Western blot analysis using a human SgII antiserum revealed the occurrence of a 97-kDa band corresponding to the expected size of SgII, with significantly higher quantities in benign than in malignant tumors (P<0.05).
  • Using antisera directed against sequential regions of SgII (N-terminal, secretoneurin [SN], EM66, internal, and C-terminal sequences), we observed distinct processing profiles between benign and malignant pheochromocytomas.
  • RIA measurement showed that EM66 median values between benign and malignant chromaffin cell tumors were significantly different (128.5 vs. 6.3 ng/mg protein, respectively; P<0.001).
  • Taken together, these results indicate that, in pheochromocytoma, malignancy is associated with reduced PC1, PC2, and SgII mRNA expression and decreased levels of processing products of SgII, in line with the low concentrations of EM66 that occur in malignant tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Gene Expression Profiling. Pheochromocytoma / diagnosis

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  • (PMID = 17102121.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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83. Schalin-Jäntti C: [Adrenal incidentaloma--a common dilemma]. Duodecim; 2010;126(9):1037-45
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Adrenal incidentaloma--a common dilemma].
  • An adrenal incidentaloma is an adrenal tumor larger than 1 cm, incidentally detected in imaging studies carried out for other reasons than adrenal disease.
  • The most frequent explanation is a benign, non-functional adenoma, other frequent diagnoses include cortisol- or aldosterone-secreting adenomas, pheochromocytoma, cortical carcinoma and metastatic lesions.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis
  • [MeSH-minor] Adenoma / diagnosis. Adenoma / surgery. Aldosterone / secretion. Humans. Hydrocortisone / secretion. Incidental Findings. Phenotype. Pheochromocytoma / diagnosis. Pheochromocytoma / surgery. Tomography, X-Ray Computed

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  • (PMID = 20593627.001).
  • [ISSN] 0012-7183
  • [Journal-full-title] Duodecim; lääketieteellinen aikakauskirja
  • [ISO-abbreviation] Duodecim
  • [Language] fin
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Finland
  • [Chemical-registry-number] 4964P6T9RB / Aldosterone; WI4X0X7BPJ / Hydrocortisone
  • [Number-of-references] 21
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84. Kupka S, Haack B, Zdichavsky M, Mlinar T, Kienzle C, Bock T, Kandolf R, Kroeber SM, Königsrainer A: Large proportion of low frequency microsatellite-instability and loss of heterozygosity in pheochromocytoma and endocrine tumors detected with an extended marker panel. J Cancer Res Clin Oncol; 2008 Apr;134(4):463-71
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  • [Title] Large proportion of low frequency microsatellite-instability and loss of heterozygosity in pheochromocytoma and endocrine tumors detected with an extended marker panel.
  • PURPOSE: Pheochromocytoma (PCC) is a usually benign tumor originated in the majority of patients from the adrenal medulla.
  • [MeSH-major] Endocrine Gland Neoplasms / genetics. Loss of Heterozygosity. Microsatellite Instability. Microsatellite Repeats. Pheochromocytoma / genetics

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  • (PMID = 17828419.001).
  • [ISSN] 0171-5216
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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85. Tabarin A, Bardet S, Bertherat J, Dupas B, Chabre O, Hamoir E, Laurent F, Tenenbaum F, Cazalda M, Lefebvre H, Valli N, Rohmer V, French Society of Endocrinology Consensus: Exploration and management of adrenal incidentalomas. French Society of Endocrinology Consensus. Ann Endocrinol (Paris); 2008 Dec;69(6):487-500
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Exploration and management of adrenal incidentalomas. French Society of Endocrinology Consensus.
  • The French Society of Endocrinology convened a multidisciplinary panel of endocrinologists, radiologists, nuclear physicians and surgeons to address the appropriate evaluation and treatment of adrenal incidentalomas.
  • The crucial points of the French consensus are: the usefulness of CT-scanning evaluation of adrenal incidentalomas, the systematic screening for pheochromocytoma, the usefulness of the 1mg overnight dexamethasone test to screen for latent hypercortisolism, the difficulty to interpret mild biological abnormalities of the HPA axis, the consensus to remove surgically most of tumours greater than 4cm, the necessity to follow clinically glucorticoid tissular targets in the follow-up of non operated benign adrenocortical incidentalomas.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Adrenal Gland Neoplasms / therapy
  • [MeSH-minor] Adrenal Cortex Neoplasms / pathology. Adrenal Cortex Neoplasms / therapy. Adrenal Glands / pathology. Animals. Biopsy. Humans. Positron-Emission Tomography

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  • (PMID = 19022420.001).
  • [ISSN] 0003-4266
  • [Journal-full-title] Annales d'endocrinologie
  • [ISO-abbreviation] Ann. Endocrinol. (Paris)
  • [Language] eng
  • [Publication-type] Guideline; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 117
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86. Pham TH, Moir C, Thompson GB, Zarroug AE, Hamner CE, Farley D, van Heerden J, Lteif AN, Young WF Jr: Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center. Pediatrics; 2006 Sep;118(3):1109-17
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center.
  • METHODS: A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients < 18 years of age with histologically confirmed pheochromocytoma or paraganglioma.
  • Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma.
  • Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease.
  • Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of > 6 cm.
  • All patients with benign disease appeared cured after resection.
  • CONCLUSIONS: The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of > 6 cm.
  • Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates.
  • Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Paraganglioma / surgery. Pheochromocytoma / surgery


87. Gonsior A, Pfeiffer H, Führer D, Liatsikos E, Schwalenberg T, Stolzenburg JU: [Adrenal tumors. Principles of diagnostics and operative treatment]. Urologe A; 2010 May;49(5):659-68; quiz 669-70
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  • [Title] [Adrenal tumors. Principles of diagnostics and operative treatment].
  • Adrenal masses are very heterogeneous and comprise benign or malignant tumors, unilateral or bilateral masses and variable endocrine activity.
  • Because of these attributes adrenal gland masses are a clinical challenge.
  • This article gives a summary of diagnostic steps and indications for adrenal surgery including perioperative management.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery
  • [MeSH-minor] Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / surgery. Adrenal Hyperplasia, Congenital / diagnosis. Adrenal Hyperplasia, Congenital / surgery. Adrenalectomy / instrumentation. Adrenalectomy / methods. Adrenogenital Syndrome / diagnosis. Adrenogenital Syndrome / surgery. Chemotherapy, Adjuvant. Combined Modality Therapy. Cushing Syndrome / diagnosis. Cushing Syndrome / surgery. Diagnosis, Differential. Diagnostic Imaging. Humans. Hyperaldosteronism / diagnosis. Hyperaldosteronism / surgery. Laparoscopes. Neoplasms, Multiple Primary / diagnosis. Neoplasms, Multiple Primary / drug therapy. Neoplasms, Multiple Primary / radiotherapy. Neoplasms, Multiple Primary / surgery. Pheochromocytoma / diagnosis. Pheochromocytoma / surgery. Radiotherapy, Adjuvant. Sensitivity and Specificity

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  • (PMID = 20449781.001).
  • [ISSN] 1433-0563
  • [Journal-full-title] Der Urologe. Ausg. A
  • [ISO-abbreviation] Urologe A
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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88. Lombardi CP, Raffaelli M, De Crea C, Traini E, D'Amore AM, Bellantone R: [Pheochromocytoma: role of preoperative diagnosis in the assessment of malignancy risk and in the choice of surgical approach]. Suppl Tumori; 2005 May-Jun;4(3):S211
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Pheochromocytoma: role of preoperative diagnosis in the assessment of malignancy risk and in the choice of surgical approach].
  • Differential diagnosis with benign lesions can be difficult even on a pathological basis.
  • Despite safe laparoscopic adrenalectomy (LA) has been reported for lesions up to 10 cm, it is considered hazardous for pheochromocytoma larger than 6 cm, because of the risk of malignancy and iatrogenic pheochromocytomatosis.
  • The medical records of all the patients who underwent adrenalectomy for pheochromocytoma were reviewed.
  • All the preoperatively available data (demographic, clinical, biochemical and radiological) were recorded as well as final pathological diagnosis.
  • Comparative analysis of patients with benign and malignant pheochromocytomas was performed.
  • Sixty-three adrenalectomies for pheochromocytoma were performed in 60 patients.
  • Fifty-seven benign and 6 malignant pheochromocytomas were identified.
  • No significant difference was found between patients with malignant and benign lesions concerning age, gender, family history, symptoms, laboratory and radiological findings.
  • In particular, no significant difference was found for lesion size between the benign (63.3 +/- 30.6 mm, range, 20-150) and the malignant group (48.6 +/- 16.5 mm; range, 30-70).
  • No preoperatively available data can reliably differentiate between benign and malignant pheochromocytomas.
  • Thus, pheochromocytoma size does not seem a reliable predictor of malignancy.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Pheochromocytoma / diagnosis. Pheochromocytoma / surgery

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  • (PMID = 16437995.001).
  • [ISSN] 2283-5423
  • [Journal-full-title] I supplementi di Tumori : official journal of Società italiana di cancerologia ... [et al.]
  • [ISO-abbreviation] Suppl Tumori
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Italy
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89. Reisch N, Walz MK, Erlic Z, Neumann HP: [Pheochromocytoma - still a challenge]. Internist (Berl); 2009 Jan;50(1):27-35
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Pheochromocytoma - still a challenge].
  • Pheochromocytomas are rare, mostly benign catecholamine-producing tumors arising from the chromaffin cells of the adrenal medulla or in the paraganglia.
  • Computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen as well as functional imaging with (123)Iod-MIBG scintigraphy and (18)F-dopa positron emission tomography are used for tumor localization.
  • The therapy of choice is the endoscopic adrenal sparing surgery following preoperative alpha-blockade.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Hypertension / diagnosis. Hypertension / prevention & control. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 19099277.001).
  • [ISSN] 1432-1289
  • [Journal-full-title] Der Internist
  • [ISO-abbreviation] Internist (Berl)
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 49
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90. Feng C, Li HZ, Yan WG, Gao JG, Xu WF, Luo YF, Cao JL: [The significance of Ki-67 antigen expression in the distinction between benign and malignant pheochromocytomas]. Zhonghua Wai Ke Za Zhi; 2007 Dec 15;45(24):1697-700
MedlinePlus Health Information. consumer health - Pheochromocytoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [The significance of Ki-67 antigen expression in the distinction between benign and malignant pheochromocytomas].
  • OBJECTIVES: To investigate the expression of Ki-67 antigen in benign and malignant pheochromocytomas, and to evaluate whether the expression of Ki-67 antigen could serve as a diagnostic marker for predicting the biological behaviour of these tumors.
  • METHODS: Ki-67 antigen were detected by immunohistochemical technique and image analysis in 57 cases of clinically documented benign and malignant pheochromocytomas were analyzed.
  • RESULTS: Ki-67 index was low in benign pheochromocytomas (average 0.98%), and high in malignant pheochromocytomas (average 3.78%).
  • There was statistically significant difference in expressions of Ki-67 antigen between benign and malignant pheochromocytomas.
  • The Ki-67 index of 2 cases in benign pheochromocytomas (5.1%, 2/39) and 10 cases in malignant pheochromocytomas (55.6%, 10/18) was higher than 3%.
  • The accuracy, sensitivity, specificity, positive predictive value and negative predictive value of Ki-67 index higher than 3% to diagnosis malignant pheochromocytomas was 82.5%, 55.6%, 94.9%, 83.3% and 82.2%.
  • Immunohistochemical assessment of Ki-67 antigen can be useful in the differential diagnosis of malignant from benign pheochromocytomas.
  • Ki-67 index (> 3%) is a useful marker for distinguishing benign from malignant tumors or for predicting the malignant potential of pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Biomarkers, Tumor / analysis. Ki-67 Antigen / analysis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Immunohistochemistry. Male. Middle Aged. Sensitivity and Specificity

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  • (PMID = 18476530.001).
  • [ISSN] 0529-5815
  • [Journal-full-title] Zhonghua wai ke za zhi [Chinese journal of surgery]
  • [ISO-abbreviation] Zhonghua Wai Ke Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen
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91. Ilias I, Pacak K: Diagnosis and management of tumors of the adrenal medulla. Horm Metab Res; 2005 Dec;37(12):717-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnosis and management of tumors of the adrenal medulla.
  • The adrenal medulla consists of chromaffin cells, the site of catecholamine biosynthesis.
  • Pheochromocytomas are chromaffin-cell tumors; 80-85 % arise from the adrenal medulla and 15-20 % arise from extra-adrenal chromaffin tissues (paragangliomas).
  • Pheochromocytomas account for 6.5 % of incidentally discovered adrenal tumors; they are found in 50 % of patients with multiple endocrine neoplasia 2A (MEN 2A) and 5-25 % of patients with von Hippel-Lindau (VHL) syndrome.
  • The diagnosis of pheochromocytoma should first be established biochemically by measuring plasma free metanephrines (the measurement of urinary fractionated metanephrines is the second choice).
  • After successful surgery, survival of patients with benign, sporadic pheochromocytomas is believed to be equal to that of the general population.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Adrenal Medulla / physiopathology. Neuroendocrine Tumors / diagnosis. Neuroendocrine Tumors / therapy
  • [MeSH-minor] Humans. Multiple Endocrine Neoplasia Type 2a / diagnosis. Multiple Endocrine Neoplasia Type 2a / therapy. Neuroblastoma / diagnosis. Neuroblastoma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy. Prognosis

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  • (PMID = 16372223.001).
  • [ISSN] 0018-5043
  • [Journal-full-title] Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et métabolisme
  • [ISO-abbreviation] Horm. Metab. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 39
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92. Gao B, Kong F, Xu Z: Development of differential diagnosis for benign and malignant pheochromocytomas. Int J Urol; 2008 Sep;15(9):771-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Development of differential diagnosis for benign and malignant pheochromocytomas.
  • A review on the development of differentiating diagnosis between malignant and benign pheochromocytomas in imaging studies, biological fluid examinations, pathological examinations, molecular markers and genome studies, was updated in the hopes of guiding the next studies of pheochromcytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology
  • [MeSH-minor] Diagnosis, Differential. Genome, Human. Humans

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  • (PMID = 18651863.001).
  • [ISSN] 1442-2042
  • [Journal-full-title] International journal of urology : official journal of the Japanese Urological Association
  • [ISO-abbreviation] Int. J. Urol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Australia
  • [Number-of-references] 58
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93. Legmann P: [Adrenal incidentaloma: management approaches: CT - MRI]. J Radiol; 2009 Mar;90(3 Pt 2):426-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Adrenal incidentaloma: management approaches: CT - MRI].
  • The adrenal gland may be affected by several pathologies, and the detection of an adrenal nodule may occur during the work-up of a biological abnormality, oncologic work-up, or be incidental.
  • Cross-sectional imaging has had a great impact on the diagnostic work-up of adrenal nodules.
  • CT, without and with intravenous contrast, is the first line imaging study for evaluation of adrenal nodules.
  • A spontaneous density below 10 HU confirms the benign nature of a nodule.
  • When lesions cannot be characterized, postcontrast CT or MR imaging, including in and out of phase imaging, may allow diagnosis.
  • [MeSH-major] Adenoma / diagnosis. Adenoma / radiography. Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / radiography. Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / radiography. Cushing Syndrome / diagnosis. Cushing Syndrome / radiography. Incidental Findings. Magnetic Resonance Imaging / methods. Sarcoma, Ewing / diagnosis. Sarcoma, Ewing / radiography. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / radiography. Adrenal Glands / pathology. Adult. Algorithms. Biopsy. Cysts / diagnosis. Cysts / radiography. Diagnosis, Differential. Female. Hemangioma / diagnosis. Hemangioma / radiography. Hematoma / diagnosis. Hematoma / radiography. Humans. Lymphoma / diagnosis. Lymphoma / radiography. Pheochromocytoma / diagnosis. Pheochromocytoma / radiography. Sarcoma / diagnosis. Sarcoma / radiography


94. Reisch N, Peczkowska M, Januszewicz A, Neumann HP: Pheochromocytoma: presentation, diagnosis and treatment. J Hypertens; 2006 Dec;24(12):2331-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma: presentation, diagnosis and treatment.
  • Pheochromocytomas are rare, mostly benign catecholamine-producing tumors of chromaffin cells of the adrenal medulla or of a paraganglion.
  • The diagnosis of pheochromocytomas depends mainly upon the demonstration of catecholamine excess by 24-h urinary catecholamines and metanephrines or plasma metanephrines.
  • They are localized by a computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen; complementary 123I-metaiodobenzylguanidine scintigraphy and 18F-dihydroxyphenylalanine-positron emission tomography are available.
  • Laparoscopic and adrenal sparing surgical intervention following preoperative alpha-blockade is the treatment of choice and usually curative.
  • This review provides an update on identification and management of pheochromocytomas, emphasizing current developments in diagnosis, including genetic testing, pathophysiology and treatment of pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • [CommentIn] J Hypertens. 2006 Dec;24(12):2341-3 [17082710.001]
  • (PMID = 17082709.001).
  • [ISSN] 0263-6352
  • [Journal-full-title] Journal of hypertension
  • [ISO-abbreviation] J. Hypertens.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 73
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95. McHugh K: Renal and adrenal tumours in children. Cancer Imaging; 2007;7:41-51
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Renal and adrenal tumours in children.
  • The differential diagnosis of renal and supra-renal masses firstly depends on the age of the child.
  • Benign renal masses predominate in early infancy but beyond the first year of life Wilms' tumour is the most common renal malignancy, until adolescence when renal cell carcinoma has similar or increased frequency as children get older.
  • Adrenal adenomas and carcinomas also occur in childhood; these tumours are indistinguishable on imaging but criteria for the diagnosis of adrenal carcinoma include size larger than 5 cm, a tendency to invade the inferior vena cava and to metastasise.
  • The most topical dilemmas in the radiological assessment of renal and adrenal tumours are presented.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Diagnostic Imaging / methods. Kidney Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Age of Onset. Carcinoma, Renal Cell / diagnosis. Carcinoma, Renal Cell / epidemiology. Child. Child, Preschool. Diagnosis, Differential. Ganglioneuroblastoma / diagnosis. Ganglioneuroblastoma / epidemiology. Ganglioneuroblastoma / pathology. Humans. Infant. Infant, Newborn. Lung Neoplasms / radiography. Lung Neoplasms / secondary. Magnetic Resonance Imaging. Mass Screening. Neoplasm Staging. Neuroblastoma / diagnosis. Neuroblastoma / epidemiology. Neuroblastoma / pathology. Neuroblastoma / secondary. Neuroblastoma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / epidemiology. Positron-Emission Tomography. Tomography, X-Ray Computed. Wilms Tumor / diagnosis. Wilms Tumor / epidemiology. Wilms Tumor / pathology. Wilms Tumor / therapy

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  • (PMID = 17339140.001).
  • [ISSN] 1470-7330
  • [Journal-full-title] Cancer imaging : the official publication of the International Cancer Imaging Society
  • [ISO-abbreviation] Cancer Imaging
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 19
  • [Other-IDs] NLM/ PMC1828369
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96. Petri BJ, Speel EJ, Korpershoek E, Claessen SM, van Nederveen FH, Giesen V, Dannenberg H, van der Harst E, Dinjens WN, de Krijger RR: Frequent loss of 17p, but no p53 mutations or protein overexpression in benign and malignant pheochromocytomas. Mod Pathol; 2008 Apr;21(4):407-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Frequent loss of 17p, but no p53 mutations or protein overexpression in benign and malignant pheochromocytomas.
  • Genetic changes in the tumorigenesis of sporadic pheochromocytomas are poorly understood, and there are no good markers to discriminate benign from malignant pheochromocytomas. p53 is a tumor suppressor gene and aberrations in this gene are frequently found in many tumor types.
  • The role of p53 in pheochromocytoma tumorigenesis is unclear, with some studies suggesting that p53 mutations can be used to discriminate benign from malignant pheochromocytomas while other studies do not find such an association.
  • Comparative genomic hybridization analysis of 31 benign and 20 malignant tumors showed loss of the p53 locus at chromosome 17p13.1 in 23/51 (45%) cases, and most of these results were confirmed by fluorescence in situ hybridization.
  • Furthermore, p53 immunohistochemistry on 35 cases revealed strong nuclear p53 expression in only two pheochromocytoma metastases, all other tumors being negative.
  • We conclude that, although there is frequent loss of the p53 locus on 17p, the p53 gene does not appear to play a major role in pheochromocytoma tumorigenesis.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Biomarkers, Tumor / genetics. Chromosomes, Human, Pair 17 / genetics. Pheochromocytoma / genetics. Tumor Suppressor Protein p53 / genetics. Tumor Suppressor Protein p53 / metabolism

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  • (PMID = 18223555.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Tumor Suppressor Protein p53
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97. Iihara M, Obara T: [Diagnosis and surgical treatment of adrenal tumors]. Nihon Geka Gakkai Zasshi; 2005 Aug;106(8):479-83
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Diagnosis and surgical treatment of adrenal tumors].
  • Adrenal surgery is necessary for the management of functioning adrenal tumors, such as aldosterone-producing adenoma, cortisol-producing adenoma, and pheochromocytoma.
  • The role of adrenal imaging in primary hyperaldosteronism is to separate the surgically resectable unilateral aldosteronoma from bilateral hyperplasia.
  • Once the clinical diagnosis of primary hyperaldosteronism is confirmed, adrenal computed tomography (CT) with 3-mm sections should be the first imaging study.
  • If the results of CT and NP-59 scintigraphy are equivocal, adrenal venous sampling is necessary.
  • Cortisol-producing adrenocortical adenomas are seen as adrenal masses 2.5 cm or larger in diameter in CT scanning.
  • When an adrenal mass measures more than 5 cm in diameter, a functioning adrenal carcinoma should be considered.
  • In the past decade, laparoscopic adrenalectomy has replaced open adrenalectomy as a standard operative procedure for benign adrenal tumors.
  • Adrenal-sparing laparoscopic surgery has recently become a feasible option in patients with hereditary bilateral pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery
  • [MeSH-minor] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / surgery. Adrenal Medulla. Humans

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  • (PMID = 16119111.001).
  • [ISSN] 0301-4894
  • [Journal-full-title] Nihon Geka Gakkai zasshi
  • [ISO-abbreviation] Nihon Geka Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 17
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98. Michalakis K, Ilias I: Medical management of adrenal disease: a narrative review. Endocr Regul; 2009 Jul;43(3):127-35
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Medical management of adrenal disease: a narrative review.
  • Adrenal diseases comprise for a variety of medical endocrine issues, ranging from partial or complete gland insufficiency, to several kinds of adrenal hyperfunction, either of congenital or neoplastic etiology.
  • Patients with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency are treated with glucocorticoids to control androgen excess.
  • Most benign neoplastic adrenal diseases that cause hyperfunction of the gland are surgically treated, however this may not be always feasible or effective.
  • [MeSH-major] Adrenal Gland Diseases / drug therapy
  • [MeSH-minor] Adrenal Gland Neoplasms / drug therapy. Adrenal Gland Neoplasms / surgery. Adrenal Glands / surgery. Adrenal Hyperplasia, Congenital / drug therapy. Adrenal Insufficiency / drug therapy. Adrenocortical Carcinoma / drug therapy. Clinical Trials as Topic. Cushing Syndrome / drug therapy. Humans. Hyperaldosteronism / drug therapy. Pheochromocytoma / drug therapy. Pheochromocytoma / surgery

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  • (PMID = 19817507.001).
  • [ISSN] 1210-0668
  • [Journal-full-title] Endocrine regulations
  • [ISO-abbreviation] Endocr Regul
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Slovakia
  • [Number-of-references] 51
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99. Chervin RA, Danilowicz K, Pitoia F, Gómez RM, Bruno OD: [A study of 34 cases of adrenal incidentaloma]. Medicina (B Aires); 2007;67(4):341-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A study of 34 cases of adrenal incidentaloma].
  • Adrenal incidentaloma, a tumor discovered unexpectedly during imaging performed for non-adrenal related causes, has become a frequent clinical concern.
  • Although in most cases they are benign and hormonally nonfunctioning, it is important to appropriately identify those few cases of malignant or hyperfunctioning lesions of surgical resolution.
  • In the present retrospective study we analyzed 34 patients with adrenal incidentaloma.
  • Of these, 32% of the patients displayed hyperfunctioning pathologies that included subclinical Cushing's syndrome in four patients, probable primary hyperaldosteronism in two, late onset congenital adrenal hyperplasia in one, adrerocortical carcinoma in one and pheochromocytoma in three.
  • CT and/or MRI permitted the identification of adenomas and were crucial to decide on surgery in two patients with nonfunctioning pheochromocytomas and in a patient carrying a primitive adrenocortical carcinoma, a diagnosis also suggested by a mixed pattern of hypersecretion of androgens and cortisol.
  • Nonfunctioning tumors were mainly adenomas (87%) with individual cases of histoplasmosis, pseudocyst, idiopathic adrenal hyperplasia and mielolipoma.
  • [MeSH-major] Adenoma / diagnosis. Adrenal Gland Neoplasms / diagnosis. Cushing Syndrome / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Aged. Androgens / secretion. Child. Diagnosis, Differential. Female. Humans. Incidental Findings. Male. Middle Aged. Retrospective Studies. Treatment Outcome

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  • (PMID = 17891929.001).
  • [ISSN] 0025-7680
  • [Journal-full-title] Medicina
  • [ISO-abbreviation] Medicina (B Aires)
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Argentina
  • [Chemical-registry-number] 0 / Androgens
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100. van Nederveen FH, Perren A, Dannenberg H, Petri BJ, Dinjens WN, Komminoth P, de Krijger RR: PTEN gene loss, but not mutation, in benign and malignant phaeochromocytomas. J Pathol; 2006 Jun;209(2):274-80
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  • [Title] PTEN gene loss, but not mutation, in benign and malignant phaeochromocytomas.
  • Furthermore, benign and malignant PCCs are found in several PTEN knockout mouse models.
  • Tumour and germline DNA was analysed from 31 patients with apparently sporadic PCC, including 14 clinically benign and 17 malignant tumours, for loss of the PTEN gene locus, mutations in the PTEN gene, and for PTEN protein expression by immunohistochemistry.
  • Loss of heterozygosity (LOH) analysis showed loss of PTEN in four malignant tumours (40%) and in one benign tumour (14%).
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Genes, Tumor Suppressor / physiology. Neoplasm Proteins / genetics. PTEN Phosphohydrolase / genetics. Pheochromocytoma / genetics

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  • [Copyright] Copyright (c) 2006 Pathological Society of Great Britain and Ireland.
  • (PMID = 16538614.001).
  • [ISSN] 0022-3417
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Neoplasm Proteins; EC 3.1.3.48 / PTEN protein, human; EC 3.1.3.67 / PTEN Phosphohydrolase
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