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1. Nawar R, Aron D: Adrenal incidentalomas -- a continuing management dilemma. Endocr Relat Cancer; 2005 Sep;12(3):585-98
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  • Most such tumors are benign and hormonally inactive.
  • [MeSH-minor] Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / etiology. Humans. National Institutes of Health (U.S.). Pheochromocytoma / diagnosis. United States

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  • (PMID = 16172193.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 99
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2. 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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  • 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.

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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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3. Ambrosio A, Scaramuzzi M, Torlontano M, Di Sebastiano P: [Laparoscopic adrenalectomy: analysis of 65 cases]. Chir Ital; 2007 Nov-Dec;59(6):853-6
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  • On the basis of our analysis of the results we feel that we can safely claim, in agreement with the literature, that laparoscopic cholecystectomy is the treatment of choice for benign disease and that lesions measuring > 6 cm can be dealt with by surgical teams with good laparoscopic experience.
  • [MeSH-major] Adenoma / surgery. Adrenal Gland Neoplasms / surgery. Adrenal Hyperplasia, Congenital / surgery. Adrenalectomy. Hemangioma / surgery. Laparoscopy. Myelolipoma / surgery. Neurilemmoma / surgery. Pheochromocytoma / surgery

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  • (PMID = 18360991.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] ita
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Italy
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4. 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 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.
  • 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


5. Bollo J, López-Boado MA, Fernández-Cruz L: [Pheochromocytoma and benign mediastinal cyst]. Cir Esp; 2007 Jan;81(1):52
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  • [Title] [Pheochromocytoma and benign mediastinal cyst].
  • [Transliterated title] Feocromocitoma y quiste benigno mediastínico.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Mediastinal Cyst / diagnosis. Pheochromocytoma / diagnosis


6. Joynt KE, Moslehi JJ, Baughman KL: Paragangliomas: etiology, presentation, and management. Cardiol Rev; 2009 Jul-Aug;17(4):159-64
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  • These tumors commonly present with episodic hypertension, tachycardia, headache, and diaphoresis, and can be either benign or malignant.
  • The majority of these tumors are benign, but for patients with malignant disease, chemotherapy, and radiation therapy may provide modest benefit.
  • [MeSH-major] Adrenal Gland Neoplasms / therapy. Paraganglioma, Extra-Adrenal / therapy. Pheochromocytoma / therapy

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  • (PMID = 19525677.001).
  • [ISSN] 1538-4683
  • [Journal-full-title] Cardiology in review
  • [ISO-abbreviation] Cardiol Rev
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 45
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7. Lodin M, Privitera A, Giannone G: Laparoscopic adrenalectomy (LA): keys to success: correct surgical indications, adequate preoperative preparation, surgical team experience. Surg Laparosc Endosc Percutan Tech; 2007 Oct;17(5):392-5
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  • Controversy still remains about the treatment for primary and secondary malignant lesions and for pheochromocytoma.
  • Selection criteria for laparoscopic treatment included benign characteristics of the lesions regardless of size and absence of virilization syndrome.
  • Laparoscopic surgery in patients with pheochromocytoma was associated with a better intraoperative hemodynamic stability.
  • CONCLUSIONS: LA is the treatment of choice for benign adrenal tumors regardless of size.

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  • (PMID = 18049399.001).
  • [ISSN] 1530-4515
  • [Journal-full-title] Surgical laparoscopy, endoscopy & percutaneous techniques
  • [ISO-abbreviation] Surg Laparosc Endosc Percutan Tech
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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8. Tiberio GA, Piardi T, Cerea K, Ronconi M, Bertoloni G, Frassi E, Portolani N, Giulini SM: [Laparoscopic adrenalectomy: an initial experience]. Chir Ital; 2005 May-Jun;57(3):273-81
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  • Our aim was to evaluate the short-term results of a series of 65 consecutive laparoscopic adrenalectomies performed on 63 patients with benign or metastatic tumours measuring < or = 7 cm.
  • The surgical indication was primary hyperaldosteronism in 32 cases, pheochromocytoma in 7, Cushing syndrome and disease in 5 and 2 cases, respectively, incidentaloma in 11 and metastasis in 8 cases.
  • [MeSH-minor] Adult. Aged. Cushing Syndrome / surgery. Female. Humans. Hyperaldosteronism / surgery. Male. Middle Aged. Pheochromocytoma / surgery. Retrospective Studies. Treatment Outcome

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  • (PMID = 16231814.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Italy
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9. Tenenbaum F: [Adrenal incidentaloma and nuclear medicine examination]. J Radiol; 2009 Mar;90(3 Pt 2):444-8
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  • To characterize lesions of the adrenal cortex, iodocholesterol scintigraphy is performed to confirm the origin of the adenoma and the benign or malignant nature of the lesion since benign adenomas show tracer uptake and malignant lesions show no tracer uptake.
  • 18F FDG PET only characterizes the lesion as benign or malignant.
  • [MeSH-major] Adenoma / radionuclide imaging. Adrenal Cortex Neoplasms / radionuclide imaging. Adrenal Gland Neoplasms / radionuclide imaging. Incidental Findings. Pheochromocytoma / radionuclide imaging. Positron-Emission Tomography / methods

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  • (PMID = 19421135.001).
  • [ISSN] 0221-0363
  • [Journal-full-title] Journal de radiologie
  • [ISO-abbreviation] J Radiol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Fluorine Radioisotopes; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 2C598205QX / fluorodopa F 18; 30461-91-7 / 19-Iodocholesterol; 35MRW7B4AD / 3-Iodobenzylguanidine; 63-84-3 / Dihydroxyphenylalanine
  • [Number-of-references] 16
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10. Nocca D, Aggarwal R, Mathieu A, Blanc PM, Denève E, Salsano V, Figueira G, Sanders G, Domergue J, Millat B, Fabre PR: Laparoscopic surgery and corticoadrenalomas. Surg Endosc; 2007 Aug;21(8):1373-6
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  • BACKGROUND: Nowadays, laparoscopic adrenalectomy is the "gold standard" procedure for the treatment of benign lesions.
  • Twelve patients (9%) had a malignant tumor: nine corticoadrenalomas, one pleomorphic sarcoma, one metastatic deposit from a previously excised colonic cancer, and one malignant pheochromocytoma.

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11. 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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  • Adrenal masses are very heterogeneous and comprise benign or malignant tumors, unilateral or bilateral masses and variable endocrine activity.
  • [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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12. Anouar Y, Yon L, Guillemot J, Thouennon E, Barbier L, Gimenez-Roqueplo AP, Bertherat J, Lefebvre H, Klein M, Muresan M, Grouzmann E, Plouin PF, Vaudry H, Elkahloun AG: Development of novel tools for the diagnosis and prognosis of pheochromocytoma using peptide marker immunoassay and gene expression profiling approaches. Ann N Y Acad Sci; 2006 Aug;1073:533-40
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  • [Title] Development of novel tools for the diagnosis and prognosis of pheochromocytoma using peptide marker immunoassay and gene expression profiling approaches.
  • These neuroendocrine tumors are usually benign, but may also present as or develop into a malignancy.
  • In addition, much higher EM66 levels were measured in benign than in malignant tumoral tissues, suggesting that this peptide could represent a valuable tool for the prognosis of PHEO.
  • We have also initiated a comparative microarray study of benign and malignant PHEOs, which allowed the identification of a set of about 100 genes that were differentially expressed and best discriminated the two types of tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Profiling. Pheochromocytoma / genetics


13. Willenberg HS, Schott M, Saeger W, Tries A, Scherbaum WA, Bornstein SR: Expression of connexins in chromaffin cells of normal human adrenals and in benign and malignant pheochromocytomas. Ann N Y Acad Sci; 2006 Aug;1073:578-83
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  • [Title] Expression of connexins in chromaffin cells of normal human adrenals and in benign and malignant pheochromocytomas.
  • We studied the expression of Cx26, Cx32, Cx43, and Cx50 in chromaffin cells of 10 normal human adrenal glands, 10 benign, and 13 malignant pheochromocytomas.
  • Immunohistological testing for Cx expression does not however allow differentiation of benign from malignant pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Adrenal Glands / metabolism. Chromaffin Cells / metabolism. Connexins / metabolism. Pheochromocytoma / metabolism

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  • (PMID = 17102126.001).
  • [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] 0 / Connexins
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14. Gazymov MM, Filipov DS: [Adrenal tumors: topical diagnosis and surgical outcomes]. Urologiia; 2005 Sep-Oct;(5):9-12
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  • A stable hypotensive result after adrenalectomy was observed in 97.1, 66.8% patients with pheochromocytoma and mineralocorticism, respectively.
  • The rest patients improved, i.e. their malignant hypertension converted to a benign one.

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  • (PMID = 16281831.001).
  • [ISSN] 1728-2985
  • [Journal-full-title] Urologii︠a︡ (Moscow, Russia : 1999)
  • [ISO-abbreviation] Urologiia
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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15. 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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  • 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.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Chromogranin A / biosynthesis. Pheochromocytoma / metabolism. Synaptophysin / biosynthesis

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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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16. Bradley S, Dumas N, Ludman M, Wood L: Hereditary renal cell carcinoma associated with von Hippel-Lindau disease: a description of a Nova Scotia cohort. Can Urol Assoc J; 2009 Feb;3(1):32-6
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  • BACKGROUND: von Hippel-Lindau (VHL) disease is an autosomal dominant condition characterized by the development of benign and malignant tumours, including cases of renal cell carcinoma (RCC).
  • Manifestations included cases of RCC (31.6%), central nervous system (CNS) hemangioblastoma (73.7%), retinal hemangioma (47.4%), renal cyst (47.4%) and pheochromocytoma (10.5%).

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  • (PMID = 19293973.001).
  • [ISSN] 1911-6470
  • [Journal-full-title] Canadian Urological Association journal = Journal de l'Association des urologues du Canada
  • [ISO-abbreviation] Can Urol Assoc J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC2645893
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17. 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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  • [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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18. Mazurkiewicz M, Wasiutyński A: [Current opinion in pathogenesis of pheochromocytoma]. Pol Merkur Lekarski; 2007 Jun;22(132):509-13
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  • [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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19. 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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  • [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

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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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20. 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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  • (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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21. Luo Z, Li J, Qin Y, Ma Y, Liang X, Xian J, Lu D, Wei M, Yang JY, Yang MQ, He Z: Differential expression of human telomerase catalytic subunit mRNA by in situ hybridization in pheochromocytomas. Endocr Pathol; 2006;17(4):387-98
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  • We investigated the expression of human telomerase catalytic component (hTERT) mRNA, hTERT protein, Ki-67 antigen, and p27kip1 in pheochromocytomas (27 benign, 7 suspected malignant, and 7 malignant), and evaluated the possibility of expressions of these proteins, and hTERT mRNA serve as diagnostic markers for predicting the biological behavior of these tumors.
  • By in situ hybridization, hTERT mRNA was expressed in 5/7 malignant tumors (defined as the presence of metastasis and/or extensive local invasion) as compared with 3/27 benign tumors.
  • All benign tumors exhibited no immunopositivity or <1% of cells stained for Ki-67 antigen.
  • While no statistical difference in p27kip1 expressions was observed among benign, malignant, and suspected malignant tumors, there was a statistical difference between the normal adrenal medulla samples and tumors (p < 0.001).
  • Thus, hTERT mRNA detection by in situ hybridization, hTERT expression, and Ki-67 antigen expression are all useful tools for differentiating malignant from benign pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics. RNA, Messenger / metabolism. Telomerase / genetics

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  • (PMID = 17525487.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; 0 / Neoplasm Proteins; 0 / RNA, Messenger; 147604-94-2 / Cyclin-Dependent Kinase Inhibitor p27; EC 2.7.7.49 / Telomerase
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22. Sanz C, Vezzosi D, Pigny P, Bennet A, Caron P: Multiple endocrine neoplasia type 2a and germ line C634G RET mutation diagnosed in an 80-year-old patient. Ann Endocrinol (Paris); 2009 Apr;70(2):141-4
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  • After right adrenalectomy which confirmed the diagnosis of pheochromocytoma, the patient underwent total thyroidectomy with dissection of the central lymph node compartment and right parathyroidectomy.
  • On histopathologic examination, both thyroid lobes presented 13 foci of MTC without lymph node metastasis and the parathyroid gland presented a benign adenoma without hyperplasia.

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  • (PMID = 19201392.001).
  • [ISSN] 0003-4266
  • [Journal-full-title] Annales d'endocrinologie
  • [ISO-abbreviation] Ann. Endocrinol. (Paris)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Radiopharmaceuticals; 9002-71-5 / Thyrotropin; 9007-12-9 / Calcitonin; 971Z4W1S09 / Technetium Tc 99m Sestamibi; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret
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23. Yuan W, Wang W, Cui B, Su T, Ge Y, Jiang L, Zhou W, Ning G: Overexpression of ERBB-2 was more frequently detected in malignant than benign pheochromocytomas by multiplex ligation-dependent probe amplification and immunohistochemistry. Endocr Relat Cancer; 2008 Mar;15(1):343-50
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  • [Title] Overexpression of ERBB-2 was more frequently detected in malignant than benign pheochromocytomas by multiplex ligation-dependent probe amplification and immunohistochemistry.
  • To analyze the genetic alterations of pheochromocytomas and evaluate the difference among malignant, extra-adrenal, and benign pheochromocytomas.
  • All 43 patients (24 women and 19 men; mean age 44.6+/-13.6 years; range 18-75 years; 9 with malignant, 7 extra-adrenal, and 27 benign) showed multiple copy number losses or gains.
  • The average copy number change was 13.10 in malignant, 13.93 in benign, and 13.47 in paraganglioma patients.
  • However, we discovered that in the malignant pheochromocytomas, 6 of the 9 patients (67%) showed erythroblastic leukemia viral oncogene homolog 2 (ERBB-2) oncogene gain, whereas only 12 of the 34 (35%) identified change in the benign and extra-adrenal pheochromocytomas.
  • Further, IHC confirmed that ERBB-2-positive staining was more frequent and stronger in malignant pheochromocytomas than in benign and extra-adrenal pheochromocytomas.
  • Our study illustrates the chromosomal changes of the whole genome of Chinese pheochromocytoma patients.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Amplification. Genes, erbB-2 / genetics. Paraganglioma / genetics. Pheochromocytoma / classification. Pheochromocytoma / genetics

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  • (PMID = 18310300.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2254511
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24. 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

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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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25. Klein RD, Jin L, Rumilla K, Young WF Jr, Lloyd RV: Germline SDHB mutations are common in patients with apparently sporadic sympathetic paragangliomas. Diagn Mol Pathol; 2008 Jun;17(2):94-100
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  • An excess of SDHB mutations in paragangliomas versus pheochromocytomas was found, with no difference in the frequency of mutations in malignant versus benign paragangliomas.
  • [MeSH-minor] Adolescent. Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / metabolism. Adrenal Gland Neoplasms / pathology. Adult. Child. DNA Mutational Analysis. DNA, Neoplasm / analysis. Female. Humans. Male. Middle Aged. Pheochromocytoma / genetics. Pheochromocytoma / metabolism. Pheochromocytoma / secondary

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  • (PMID = 18382370.001).
  • [ISSN] 1533-4066
  • [Journal-full-title] Diagnostic molecular pathology : the American journal of surgical pathology, part B
  • [ISO-abbreviation] Diagn. Mol. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Iron-Sulfur Proteins; 0 / SDHD protein, human; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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26. 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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  • [Title] Pheochromocytoma: current approaches and future directions.
  • 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.
  • [MeSH-major] Adrenal Gland Neoplasms. Pheochromocytoma


27. Aguilera Bazán A, Pérez Utrilla M, Alvarez Escolá C, Reinoso J, Fernández Martínez A, Cisneros Ledo J, de la Peña Barthel J: [Laparoscopic adrenalectomy. Five-year experience]. Actas Urol Esp; 2010 Feb;34(2):181-5
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  • MATERIALS AND METHODS: A total of 37 laparoscopic adrenalectomies were performed over 53 months for benign and malignant conditions.
  • A malignancy was found in 4 patients (10%), while all other patients (90%) had a benign condition, including 8 pheochromocytomas.
  • CONCLUSIONS: Laparoscopy is considered to be the gold standard for benign adrenal conditions.
  • [MeSH-minor] Adenoma / surgery. Adrenal Gland Diseases / surgery. Adrenal Gland Neoplasms / secondary. Adrenal Gland Neoplasms / surgery. Adult. Aged. Carcinoma / secondary. Carcinoma / surgery. Cushing Syndrome / surgery. Cysts / surgery. Female. Humans. Hyperaldosteronism / surgery. Male. Middle Aged. Myelolipoma / surgery. Pheochromocytoma / surgery. Pregnancy. Pregnancy Complications / surgery. Retrospective Studies

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  • (PMID = 20403283.001).
  • [ISSN] 1699-7980
  • [Journal-full-title] Actas urologicas españolas
  • [ISO-abbreviation] Actas Urol Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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28. 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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  • [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


29. 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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  • [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.
  • 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.
  • 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.
  • 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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30. Brauckhoff M, Dralle H: [Recurrent operations on the adrenal glands]. Chirurg; 2005 Mar;76(3):227-37
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  • 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

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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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31. Poulose BK, Holzman MD, Lao OB, Grogan EL, Goldstein RE: Laparoscopic adrenalectomy: 100 resections with clinical long-term follow-up. Surg Endosc; 2005 Mar;19(3):379-85
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  • RESULTS: The diseases included Conn's syndrome (27 patients), Cushing's syndrome (30 patients), pheochromocytoma (11 patients), and Other tumors (26 patients).
  • Antihypertensive therapy was eliminated or reduced for Conn's syndrome (75%), Cushing's syndrome (27%), pheochromocytoma (88%) and patients with Other tumors (54%).
  • Clinical improvement was observed by 12 months for pheochromocytoma patients as compared with 35 to 45 months for the other groups (p < 0.05).
  • Multivariate analysis showed that pheochromocytoma patients were more likely to experience improvement or cure than the Other tumor group (hazard ratio, 4.87; 95% confidence interval, 1.61-14.7).
  • CONCLUSIONS: Laparoscopic adrenalectomy continues to be safe and efficacious for benign adrenal diseases.

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  • (PMID = 15624053.001).
  • [ISSN] 1432-2218
  • [Journal-full-title] Surgical endoscopy
  • [ISO-abbreviation] Surg Endosc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antihypertensive Agents
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32. 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.
  • 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


33. 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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  • Adenomatoid tumours are rare benign tumours of mesothelial derivation.
  • 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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34. 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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  • [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.
  • 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

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  • [ISSN] 0171-5216
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
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35. Ciotti P, Garuti A, Gulli R, Ballestrero A, Bellone E, Mandich P: Germline mutations in the von Hippel-Lindau gene in Italian patients. Eur J Med Genet; 2009 Sep-Oct;52(5):311-4
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  • von Hippel-Lindau syndrome (VHL) is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign tumours, most frequently retinal, cerebellar, and spinal hemangioblastoma, renal cell carcinoma, pheochromocytoma, and pancreatic tumours.

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  • (PMID = 19464396.001).
  • [ISSN] 1878-0849
  • [Journal-full-title] European journal of medical genetics
  • [ISO-abbreviation] Eur J Med Genet
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 9007-49-2 / DNA; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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36. 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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  • Cortical adenoma is the most frequent tumour detected incidentally, but adrenocortical cancer, phaeochromocytoma and metastasis are not rare.
  • (2) identify phaeochromocytoma.
  • 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.
  • [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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37. Walz MK, Petersenn S, Koch JA, Mann K, Neumann HP, Schmid KW: Endoscopic treatment of large primary adrenal tumours. Br J Surg; 2005 Jun;92(6):719-23
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  • BACKGROUND: Endoscopic adrenalectomy has become the treatment of choice for small benign adrenal tumours but should not be used for malignant lesions.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Adrenocortical Carcinoma / surgery. Endoscopy / methods. Pheochromocytoma / surgery

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  • [Copyright] Copyright (c) 2005 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.
  • (PMID = 15856491.001).
  • [ISSN] 0007-1323
  • [Journal-full-title] The British journal of surgery
  • [ISO-abbreviation] Br J Surg
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] England
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38. Tsitouridis I, Michaelides M, Stratilati S, Sidiropoulos D, Bintoudi A, Rodokalakis G: CT guided percutaneous adrenal biopsy for lesions with equivocal findings in chemical shift MR imaging. Hippokratia; 2008 Jan;12(1):37-42
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  • RESULTS: From the 57 lesions that were sent for histopathological evaluation, 31 proved to be metastases (54.4%), 20 adenomas (35.1%), 3 cortical carcinomas (5.3%), 1 benign pheochromocytoma (1.8%) and 2 samples were non diagnostic(3.5%).
  • Since most adrenal lesions are benign, dedicated adrenal imaging is necessary for choosing which of them have to be further evaluated by biopsy.

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  • (PMID = 18923755.001).
  • [ISSN] 1790-8019
  • [Journal-full-title] Hippokratia
  • [ISO-abbreviation] Hippokratia
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Other-IDs] NLM/ PMC2532963
  • [Keywords] NOTNLM ; CT / MRI / adrenal biopsy / chemical shift / enhancement washout
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39. Björklund P, Cupisti K, Fryknäs M, Isaksson A, Willenberg HS, Akerström G, Hellman P, Westin G: Stathmin as a marker for malignancy in pheochromocytomas. Exp Clin Endocrinol Diabetes; 2010 Jan;118(1):27-30
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  • Differential diagnosis between malignant and benign tumors is dependent on the development of metastasis or extensive local invasion.
  • RT-PCR analysis further confirmed mRNA overexpression, 6 to 8-fold for benign and malignant tumors, and 16-fold for metastases.
  • Stathmin protein overexpression was observed by immunohistochemistry, and distinct differential protein expression between benign and malignant/metastasis specimens was confirmed by Western blot analysis.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Biomarkers, Tumor / metabolism. Pheochromocytoma / diagnosis. Stathmin / metabolism

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  • [Copyright] J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart, New York.
  • (PMID = 19449284.001).
  • [ISSN] 1439-3646
  • [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] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / RNA, Messenger; 0 / STMN1 protein, human; 0 / Stathmin
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40. Waldmann J, Slater EP, Langer P, Buchholz M, Ramaswamy A, Walz MK, Schmid KW, Feldmann G, Bartsch DK, Fendrich V: Expression of the transcription factor snail and its target gene twist are associated with malignancy in pheochromocytomas. Ann Surg Oncol; 2009 Jul;16(7):1997-2005
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  • METHODS: Snail, Twist, and E-cadherin expression were assessed by immunohistochemistry and real-time quantitative reverse transcriptase-polymerase chain reaction in 12 malignant and 35 benign pheochromocytomas (PCC).
  • We observed Snail expression in 7 (58%) of 12 malignant PCC, whereas only 6 (17%) of 35 apparently benign PCC revealed Snail expression (P = 0.01).
  • Furthermore, 11 (92%) of 12 malignant PCC, but only 20 (57%) of 35 benign PCC, revealed Twist expression (P = 0.03).
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Biomarkers, Tumor / genetics. Nuclear Proteins / genetics. Pheochromocytoma / genetics. Transcription Factors / genetics. Twist Transcription Factor / genetics

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  • (PMID = 19412634.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cadherins; 0 / Nuclear Proteins; 0 / TWIST1 protein, human; 0 / Transcription Factors; 0 / Twist Transcription Factor; 0 / snail family transcription factors
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41. Pozo J, Muñoz MT, Martos G, Argente J: Sporadic phaeochromocytoma in childhood: clinical and molecular variability. J Pediatr Endocrinol Metab; 2005 Jun;18(6):527-32
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  • [Title] Sporadic phaeochromocytoma in childhood: clinical and molecular variability.
  • Sporadic phaeochromocytoma is an infrequent tumour during paediatric age and may or may not be associated with specific autosomal dominant inherited cancer syndromes such as multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau syndrome (VHL) type 2 or neurofibromatosis (NF) type 1.
  • We report two cases of benign, adrenal, and unilateral phaeochromocytoma that clearly demonstrate the clinical and molecular heterogeneity of this disease during the paediatric period.
  • The first patient presented a characteristic symptomatic form of sporadic phaeochromocytoma.
  • The frequency of de novo mutations in susceptible genes (especially the VHL gene) in paediatric patients with sporadic phaeochromocytoma and the elevated mortality of these cancer syndromes suggest that screening for mutations should be performed even in cases of non-familial sporadic phaeochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics

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  • (PMID = 16042317.001).
  • [ISSN] 0334-018X
  • [Journal-full-title] Journal of pediatric endocrinology & metabolism : JPEM
  • [ISO-abbreviation] J. Pediatr. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine; X4W3ENH1CV / Norepinephrine
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42. Ruiz-Tovar J, Pérez de Oteyza J, Alonso Hernández N, Díez Tabernilla M, Rojo Blanco R, Collado Guirao MV, García Villanueva A: [Laparoscopic adrenalectomy]. Cir Esp; 2007 Sep;82(3):161-5
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  • RESULTS: Surgery was indicated for Cushing's syndrome in 46% of the patients, aldosteronoma in 25%, incidentaloma in 21% and pheochromocytoma in 8%.
  • CONCLUSIONS: Laparoscopic adrenalectomy should be considered the procedure of choice for the surgical management of benign adrenal disease.
  • [MeSH-minor] Female. Humans. Hyperaldosteronism / surgery. Male. Middle Aged. Pheochromocytoma / surgery. Pituitary ACTH Hypersecretion / surgery

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  • (PMID = 17916287.001).
  • [ISSN] 0009-739X
  • [Journal-full-title] Cirugía española
  • [ISO-abbreviation] Cir Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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43. Beuschlein F: Adrenal incidentalomas: presentation and clinical work-up. Horm Res; 2007;68 Suppl 5:191-4
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  • 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.
  • [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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44. Arcos CT, Luque VR, Luque JA, García PM, Jiménez AB, Muñoz MM: Malignant giant pheochromocytoma: a case report and review of the literature. Can Urol Assoc J; 2009 Dec;3(6):E89-91
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  • [Title] Malignant giant pheochromocytoma: a case report and review of the literature.
  • Malignant pheochromocytoma is a rare disease and surgical resection is the only curative treatment.
  • We report a case of a patient with a giant retroperitoneal tumour, the second largest to be published, which was diagnosed as a malignant pheochromocytoma; it was treated with surgery.
  • The literature is reviewed to evaluate tumour features and criteria to distinguish between benign and malignant pheochromocytomas.

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  • (PMID = 20019963.001).
  • [ISSN] 1920-1214
  • [Journal-full-title] Canadian Urological Association journal = Journal de l'Association des urologues du Canada
  • [ISO-abbreviation] Can Urol Assoc J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC2792417
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45. 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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  • 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).


46. Ctvrtlík F, Herman M, Student V, Tichá V, Minarík J: Differential diagnosis of incidentally detected adrenal masses revealed on routine abdominal CT. Eur J Radiol; 2009 Feb;69(2):243-52
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  • According to the definitive histological diagnosis after adrenalectomy, the lesions were divided into the following six groups: primary adrenocortical carcinoma (n=4), metastasis (n=7), adrenocortical adenoma (n=37), pheochromocytoma (n=9), myelolipoma (n=2), and others (n=3).
  • CONCLUSION: Standard CT of the abdomen (not specifically aimed at adrenal glands) is a suitable method for distinguishing adrenal lesions which need to be operated on from those which are probably benign but need to be monitored.

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  • (PMID = 18226485.001).
  • [ISSN] 1872-7727
  • [Journal-full-title] European journal of radiology
  • [ISO-abbreviation] Eur J Radiol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Ireland
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47. Pasquali D, Rossi V, Conzo G, Pannone G, Bufo P, De Bellis A, Renzullo A, Bellastella G, Colao A, Vallone G, Bellastella A, Sinisi AA: Effects of somatostatin analog SOM230 on cell proliferation, apoptosis, and catecholamine levels in cultured pheochromocytoma cells. J Mol Endocrinol; 2008 Jun;40(6):263-71
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  • [Title] Effects of somatostatin analog SOM230 on cell proliferation, apoptosis, and catecholamine levels in cultured pheochromocytoma cells.
  • Surgery is the primary therapy for pheochromocytoma (PHEO), a catecholamine-producing tumor.
  • Benign and malignant PHEO could develop recurrences, and the intraoperative risk of recurrent PHEO is an important unresolved issue.
  • Pheo-c from six benign surgical samples were set up and characterized by immunocytochemistry.
  • This improves our knowledge on the mechanism of SOM230 action in PHEO and supports a possible therapeutic use in benign PHEO recurrence.
  • [MeSH-major] Apoptosis / drug effects. Catecholamines / metabolism. Pheochromocytoma / pathology. Somatostatin / analogs & derivatives

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  • (PMID = 18502819.001).
  • [ISSN] 1479-6813
  • [Journal-full-title] Journal of molecular endocrinology
  • [ISO-abbreviation] J. Mol. Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Receptors, Somatostatin; 51110-01-1 / Somatostatin; 98H1T17066 / pasireotide; EC 2.4.2.30 / Poly(ADP-ribose) Polymerases; EC 3.4.22.- / Caspase 3; RWM8CCW8GP / Octreotide
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48. de Herder WW, Kwekkeboom DJ, Valkema R, Feelders RA, van Aken MO, Lamberts SW, van der Lely AJ, Krenning EP: Neuroendocrine tumors and somatostatin: imaging techniques. J Endocrinol Invest; 2005;28(11 Suppl International):132-6
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  • 111In-pentetreotide scintigraphy generally has a lower detection rate for benign pheochromocytomas than 123I-MIBG scintigraphy, but it can have a complementary role for the staging of malignant pheochromocytomas.
  • [MeSH-minor] Carcinoid Tumor / radionuclide imaging. Gastrointestinal Neoplasms / radionuclide imaging. Humans. Indium Radioisotopes. Paraganglioma / radionuclide imaging. Pheochromocytoma / radionuclide imaging. Pituitary Neoplasms / radionuclide imaging. Receptors, Somatostatin / analysis

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  • (PMID = 16625862.001).
  • [ISSN] 0391-4097
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Indium Radioisotopes; 0 / Receptors, Somatostatin; 0 / somatostatin receptor 2; 0 / somatostatin receptor 5; 51110-01-1 / Somatostatin; G083B71P98 / pentetreotide
  • [Number-of-references] 49
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49. Safwat AS, Bissada NK, Seyam RM, Al Sobhi S, Hanash KA: The clinical spectrum of phaeochromocytoma: analysis of 115 patients. BJU Int; 2008 Jun;101(12):1561-4
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  • [Title] The clinical spectrum of phaeochromocytoma: analysis of 115 patients.
  • OBJECTIVE: To analyse the presentation, manifestations and outcome in consecutive patients with phaeochromocytoma, as this disease has a wide range of pathological and clinical expressions.
  • PATIENTS AND METHODS: The records of 115 patients with phaeochromocytoma were analysed retrospectively, recording the patients' age, sex, presenting symptoms and clinical signs, chemical, radiological and pathological findings and associated conditions.
  • Ten patients had malignant and 105 had benign phaeochromocytoma.
  • Eighty-six patients had sporadic and 29 had familial phaeochromocytoma, comprising eight with von Hippel-Lindau (VHL) disease, 17 with multiple endocrine neoplasia type II (MEN II) and four with von Recklinghausen disease.
  • Two patients with sporadic phaeochromocytoma had Grave's disease.
  • Ten patients (8.7%) had malignant phaeochromocytoma, of whom two had MEN II.
  • CONCLUSION: Phaeochromocytoma is an interesting clinical entity with a wide spectrum of pathological and clinical manifestations.
  • The diagnosis of phaeochromocytoma is confirmed by chemical methods, and located using imaging techniques, with computed tomography, magnetic resonance imaging and (131)I-meta-iodobenzyl guanidine radioisotope scanning being the most common.
  • This series reflects the pathological and clinical spectrum of phaeochromocytoma.
  • The presence of other manifestations of familial phaeochromocytoma influenced the presentation and prognosis of these patients.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Hypertension / etiology. Pheochromocytoma / pathology


50. Sakamaki Y, Yasukawa M, Kido T: Pheochromocytoma of the posterior mediastinum undiagnosed until the onset of intraoperative hypertension. Gen Thorac Cardiovasc Surg; 2008 Oct;56(10):509-11
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  • [Title] Pheochromocytoma of the posterior mediastinum undiagnosed until the onset of intraoperative hypertension.
  • An asymptomatic 42-year-old man was diagnosed with a posterior mediastinal mass, most likely a nonfunctioning, benign, neurogenic tumor for which thoracoscopic surgery was initially indicated.
  • However, the systemic blood pressure rapidly increased to a critical level immediately after starting the surgical manipulation of the tumor, which was suggestive of a hyperfunctioning pheochromocytoma.
  • The tumor was removed after controlling the blood pressure and was histologically diagnosed as a pheochromocytoma.
  • The possibility of extraadrenal pheochromocytoma should be considered in the preoperative diagnosis of an intrathoracic paraaortic tumor, even in an asymptomatic patient.
  • [MeSH-major] Hypertension / etiology. Mediastinal Neoplasms / diagnosis. Pheochromocytoma / diagnosis. Thoracoscopy / adverse effects

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  • (PMID = 18854928.001).
  • [ISSN] 1863-6705
  • [Journal-full-title] General thoracic and cardiovascular surgery
  • [ISO-abbreviation] Gen Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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51. Yao L, Schiavi F, Cascon A, Qin Y, Inglada-Pérez L, King EE, Toledo RA, Ercolino T, Rapizzi E, Ricketts CJ, Mori L, Giacchè M, Mendola A, Taschin E, Boaretto F, Loli P, Iacobone M, Rossi GP, Biondi B, Lima-Junior JV, Kater CE, Bex M, Vikkula M, Grossman AB, Gruber SB, Barontini M, Persu A, Castellano M, Toledo SP, Maher ER, Mannelli M, Opocher G, Robledo M, Dahia PL: Spectrum and prevalence of FP/TMEM127 gene mutations in pheochromocytomas and paragangliomas. JAMA; 2010 Dec 15;304(23):2611-9
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  • The most common presentation was that of a single benign adrenal tumor in patients older than 40 years.
  • CONCLUSIONS: Germline mutations of FP/TMEM127 were associated with pheochromocytoma but not paraganglioma and occurred in an age group frequently excluded from genetic screening algorithms.

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  • (PMID = 21156949.001).
  • [ISSN] 1538-3598
  • [Journal-full-title] JAMA
  • [ISO-abbreviation] JAMA
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / 5 P30 CA465920; United States / NIA NIH HHS / AG / P01AG19316; United States / NIA NIH HHS / AG / P30 AG013319; United States / NCI NIH HHS / CA / P30 CA54174; United Kingdom / Cancer Research UK / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Membrane Proteins; 0 / TMEM127 protein, human
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52. Rescorla FJ: Malignant adrenal tumors. Semin Pediatr Surg; 2006 Feb;15(1):48-56
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  • 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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53. 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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  • [Title] [Diagnostic and therapeutic procedures in pheochromocytoma: current trends].
  • 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

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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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54. Krombach GA, Spuentrup E, Buecker A, Mahnken AH, Katoh M, Temur Y, Higgins CB, Günther RW: [Heart tumors: magnetic resonance imaging and multislice spiral CT]. Rofo; 2005 Sep;177(9):1205-18
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  • Primary benign and malignant cardiac tumors have several characteristic features in MR imaging.
  • [MeSH-major] Fibroma / diagnosis. Heart Neoplasms / diagnosis. Hemangioma / diagnosis. Hemangiosarcoma / diagnosis. Lipoma / diagnosis. Magnetic Resonance Imaging / methods. Myxoma / diagnosis. Pheochromocytoma / diagnosis. Rhabdomyoma / diagnosis. Tomography, Spiral Computed / methods

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  • (PMID = 16123866.001).
  • [ISSN] 1438-9029
  • [Journal-full-title] RöFo : Fortschritte auf dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
  • [ISO-abbreviation] Rofo
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Contrast Media; K2I13DR72L / Gadolinium DTPA
  • [Number-of-references] 59
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55. Mackenzie IS, Gurnell M, Balan KK, Simpson H, Chatterjee K, Brown MJ: The use of 18-fluoro-dihydroxyphenylalanine and 18-fluorodeoxyglucose positron emission tomography scanning in the assessment of metaiodobenzylguanidine-negative phaeochromocytoma. Eur J Endocrinol; 2007 Oct;157(4):533-7
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  • [Title] The use of 18-fluoro-dihydroxyphenylalanine and 18-fluorodeoxyglucose positron emission tomography scanning in the assessment of metaiodobenzylguanidine-negative phaeochromocytoma.
  • 123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy scanning is commonly used in the imaging of phaeochromocytoma (and paraganglioma) to confirm the site of disease and whether any spread has occurred.
  • However, 123I-MIBG imaging is negative in 15% of cases of benign phaeochromocytoma and around 50% of cases of malignant phaeochromocytoma.
  • In recent years, positron emission tomography (PET) scanning using various different radiotracers has been shown to be a good alternative or supplementary investigation in phaeochromocytoma.
  • We present the cases of four patients with symptoms and signs suggestive of phaeochromocytoma, but who had negative 123I-MIBG scans, and illustrate the usefulness of 18-fluoro-dihydroxyphenylalanine PET scanning in their assessment.
  • These illustrative cases lend further support for the use of PET scanning in the assessment of phaeochromocytoma and suggest that it may have a particularly important role in the investigation of patients in whom 123I-MIBG scanning is negative.

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  • (PMID = 17893270.001).
  • [ISSN] 1479-683X
  • [Journal-full-title] European journal of endocrinology
  • [ISO-abbreviation] Eur. J. Endocrinol.
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Fluorine Radioisotopes; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 2C598205QX / fluorodopa F 18; 35MRW7B4AD / 3-Iodobenzylguanidine; 63-84-3 / Dihydroxyphenylalanine
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56. Suárez C, Rodrigo JP, Ferlito A, Cabanillas R, Shaha AR, Rinaldo A: Tumours of familial origin in the head and neck. Oral Oncol; 2006 Nov;42(10):965-78
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  • Individuals with inherited cancer syndromes are at significant risk of developing both benign and malignant tumours as a result of a germline mutation in a specific tumour suppressor gene.
  • Hallmarks of MEN-2A (the commonest phenotypic variant) include medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism.

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  • (PMID = 16857415.001).
  • [ISSN] 1368-8375
  • [Journal-full-title] Oral oncology
  • [ISO-abbreviation] Oral Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 121
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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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  • 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.
  • [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. 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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  • 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.
  • Adrenal neoplasms, including adrenocortical carcinomas, can be associated with cysts that are benign in appearance.

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  • (PMID = 16983481.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
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  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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59. 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).
  • [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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60. 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].
  • [Transliterated title] Feocromocitoma extraadrenal: actualización de los aspectos más controvertidos. A propósito de dos casos.
  • The tumors were apparently benign and sporadic and were not associated with other neoplasms or hereditary diseases.
  • [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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61. Safo AO, Pambuccian SE: Pancreatic manifestations of von Hippel-Lindau disease. Arch Pathol Lab Med; 2010 Jul;134(7):1080-3
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  • Von Hippel-Lindau disease is an uncommon, multisystem, multitumor disorder that can present in sporadic form but is more commonly inherited as an autosomal-dominant disease with high penetrance.
  • Affected patients are at increased risk for developing multiple synchronous or metachronous benign or malignant, cystic, and vascular neoplasms of various organs.
  • The characteristic neoplasms associated with von Hippel-Lindau are hemangioblastoma of the central nervous system and retina, clear cell renal cell carcinoma, and pheochromocytoma, but other lesions are well recognized.

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  • (PMID = 20586642.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 30
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62. Waldmann J, Bartsch DK, Kann PH, Fendrich V, Rothmund M, Langer P: Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening. Langenbecks Arch Surg; 2007 Jul;392(4):437-43
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  • In three patients, functioning tumors (one pheochromocytoma, one bilateral Cushing adenoma, and one adrenocortical carcinoma) and one nonfunctioning adenoma were diagnosed by histology and biochemical assessment.
  • CONCLUSION: MEN-1-associated adrenal tumors are mostly small, benign, and nonfunctioning and much more common than previously reported.

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  • (PMID = 17235589.001).
  • [ISSN] 1435-2443
  • [Journal-full-title] Langenbeck's archives of surgery
  • [ISO-abbreviation] Langenbecks Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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63. 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


64. 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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  • 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 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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65. de Krijger RR, van Nederveen FH, Korpershoek E, Dinjens WN: New developments in the detection of the clinical behavior of pheochromocytomas and paragangliomas. Endocr Pathol; 2006;17(2):137-41
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  • As is true for many (neuro)endocrine tumors, but unlike most other epithelial tumors, histopathological analysis does not allow a distinction to be made between PCC and PGL that will follow a benign course and those that have metastasized or will do so, a condition associated with poor prognosis.
  • In this article, recent attempts at marker detection, such as those mentioned above, as well as emerging knowledge on the molecular abnormalities in benign and malignant PCC and PGL will be presented.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Paraganglioma / diagnosis. Pheochromocytoma / diagnosis

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  • (PMID = 17159246.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 18
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66. Fuentes C, Menéndez E, Pineda J, Martínez De Esteban JP, Anda E, Goñi MJ, Bausch B, Neumann HP: The malignant potential of a succinate dehydrogenase subunit B germline mutation. J Endocrinol Invest; 2006 Apr;29(4):350-2
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  • Two years later a retroperitoneal benign paraganglioma was found and resected in his 32-yr-old daughter.
  • We conclude that the SDHB mutation predisposes to abdominal extra-adrenal and potential malignant pheochromocytoma with incomplete penetrance.

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  • (PMID = 16699302.001).
  • [ISSN] 0391-4097
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Iron-Sulfur Proteins; 0 / Protein Subunits; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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67. Meyer-Rochow GY, Soon PS, Delbridge LW, Sywak MS, Bambach CP, Clifton-Bligh RJ, Robinson BG, Sidhu SB: Outcomes of minimally invasive surgery for phaeochromocytoma. ANZ J Surg; 2009 May;79(5):367-70
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  • [Title] Outcomes of minimally invasive surgery for phaeochromocytoma.
  • Laparoscopic adrenalectomy is now accepted as the procedure of choice for the resection of benign adrenocortical tumours, but few studies have assessed whether the outcomes of laparoscopic adrenalectomy for adrenal phaeochromocytoma are similar to that of other adrenal tumour types.
  • A total of 191 patients had laparoscopic adrenalectomy, of which 36 were for phaeochromocytoma, over a 12-year period.
  • Length of hospital stay (4.8 vs 3.6 days, P= 0.03) and total operating times (183 vs 157 min, P= 0.01) were greater in the laparoscopic phaeochromocytoma resection group.
  • Laparoscopic adrenalectomy for phaeochromocytoma is a safe procedure with similar outcomes to laparoscopic adrenalectomy for other adrenal tumour types.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy / methods. Pheochromocytoma / surgery

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  • (PMID = 19566519.001).
  • [ISSN] 1445-2197
  • [Journal-full-title] ANZ journal of surgery
  • [ISO-abbreviation] ANZ J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
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68. Geli J, Nord B, Frisk T, Edström Elder E, Ekström TJ, Carling T, Bäckdahl M, Larsson C: Deletions and altered expression of the RIZ1 tumour suppressor gene in 1p36 in pheochromocytomas and abdominal paragangliomas. Int J Oncol; 2005 May;26(5):1385-91
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  • A panel of 18 pheochromocytomas (14 benign, and 4 malignant) and 11 abdominal paragangliomas (4 benign, and 7 malignant) were characterised for somatic deletions and mRNA expression status of RIZ1 using loss of heterozygosity (LOH) analysis and real-time quantitative PCR, respectively.
  • [MeSH-major] Abdominal Neoplasms / genetics. Adrenal Gland Neoplasms / genetics. Chromosomes, Human, Pair 1. DNA-Binding Proteins / biosynthesis. DNA-Binding Proteins / genetics. Gene Deletion. Gene Expression Profiling. Nuclear Proteins / biosynthesis. Nuclear Proteins / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics. Transcription Factors / biosynthesis. Transcription Factors / genetics

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  • (PMID = 15809732.001).
  • [ISSN] 1019-6439
  • [Journal-full-title] International journal of oncology
  • [ISO-abbreviation] Int. J. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / Nuclear Proteins; 0 / RNA, Messenger; 0 / Retinoblastoma Protein; 0 / Transcription Factors; EC 2.1.1.43 / Histone-Lysine N-Methyltransferase; EC 2.1.1.43 / PRDM2 protein, human
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69. 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.
  • SDHD mutations are typically associated with multifocal HNPGL and usually benign adrenal and extra-adrenal PGLs.
  • [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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70. Castillo OA, Vitagliano G, Cortes O, Kerkebe M, Pinto I, Arellano L: Bilateral laparoscopic adrenalectomy. J Endourol; 2007 Sep;21(9):1053-8
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  • Also, it is the first choice for bilateral benign tumors and metastatic neoplasia.
  • There were 6 cases of bilateral pheochromocytoma, 6 patients with Cushing's disease, 3 cases of metastasis, 3 congenital adrenal hyperplasias, 2 hyperaldosteronisms, and a single case each of adrenal adenoma and myelolipoma.

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  • (PMID = 17941786.001).
  • [ISSN] 0892-7790
  • [Journal-full-title] Journal of endourology
  • [ISO-abbreviation] J. Endourol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] United States
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71. Taïeb D, Sebag F, Barlier A, Tessonnier L, Palazzo FF, Morange I, Niccoli-Sire P, Fakhry N, De Micco C, Cammilleri S, Enjalbert A, Henry JF, Mundler O: 18F-FDG avidity of pheochromocytomas and paragangliomas: a new molecular imaging signature? J Nucl Med; 2009 May;50(5):711-7
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  • A combination of preoperative imaging work-up, surgical findings, and pathologic analyses was used to classify the patients into 2 groups: those with nonmetastatic disease (presumed benign, n = 18) and those with metastatic tumors (n = 10). (18)F-FDG PET was performed in all cases.
  • Somatic mutations of the succinate dehydrogenase subunits B and D and Von-Hippel Lindau genes were also evaluated in 6 benign sporadic tumor samples.
  • By contrast, (18)F-FDG PET underestimated the extent of the disease, compared with 6-(18)F-fluorodopa PET, in 5 patients with metastatic pheochromocytoma.
  • [MeSH-major] Fluorodeoxyglucose F18 / pharmacokinetics. Molecular Probe Techniques. Paraganglioma / metabolism. Paraganglioma / radionuclide imaging. Pheochromocytoma / metabolism. Pheochromocytoma / radionuclide imaging

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  • (PMID = 19372492.001).
  • [ISSN] 0161-5505
  • [Journal-full-title] Journal of nuclear medicine : official publication, Society of Nuclear Medicine
  • [ISO-abbreviation] J. Nucl. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18; EC 1.3.99.1 / Succinate Dehydrogenase
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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.
  • The patient had multiple benign tumors and suffered from hypertension for 4 years.
  • A histopathological diagnosis of pheochromocytoma was made.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Neurofibromatosis 1 / complications. Pheochromocytoma / complications


73. 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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74. 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
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  • [Title] Lack of association between microsatellite instability and benign adrenal tumors.
  • 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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75. 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

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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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76. 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.


77. Lumachi F, Borsato S, Tregnaghi A, Marino F, Fassina A, Zucchetta P, Marzola MC, Cecchin D, Bui F, Iacobone M, Favia G: High risk of malignancy in patients with incidentally discovered adrenal masses: accuracy of adrenal imaging and image-guided fine-needle aspiration cytology. Tumori; 2007 May-Jun;93(3):269-74
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  • AIMS AND BACKGROUND: The incidental finding of nonfunctioning adrenal masses (incidentalomas) is common, but no reliable criteria in differentiating between benign and malignant adrenal masses have been defined.
  • RESULTS: The revised final pathology showed 30 (71.4%) benign (26 adrenocortical adenomas, of which 3 were atypical, 2 ganglioneuromas, and 2 nonfunctioning benign pheochromocytomas) and 12 (28.6%, 95% CI = 15-42) adrenal malignancies (8 adrenocortical carcinomas and 4 unsuspected adrenal metastases).
  • [MeSH-minor] 19-Iodocholesterol / analogs & derivatives. Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / diagnostic imaging. Adrenal Gland Diseases / metabolism. Adrenal Gland Diseases / pathology. Adrenal Gland Diseases / surgery. Adrenalectomy. Adrenocortical Adenoma / diagnosis. Adrenocortical Adenoma / diagnostic imaging. Adrenocortical Adenoma / metabolism. Adrenocortical Adenoma / pathology. Adrenocortical Adenoma / surgery. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / diagnostic imaging. Adrenocortical Carcinoma / metabolism. Adrenocortical Carcinoma / pathology. Adrenocortical Carcinoma / surgery. Adult. Aged. Aldosterone / blood. Epinephrine / urine. Female. Ganglioneuroma / diagnosis. Ganglioneuroma / diagnostic imaging. Ganglioneuroma / metabolism. Ganglioneuroma / pathology. Ganglioneuroma / surgery. Humans. Hydrocortisone / blood. Iodine Radioisotopes. Laparoscopy. Male. Middle Aged. Norepinephrine / urine. Pheochromocytoma / diagnosis. Pheochromocytoma / diagnostic imaging. Pheochromocytoma / metabolism. Pheochromocytoma / pathology. Pheochromocytoma / surgery. Predictive Value of Tests. Prospective Studies. Radiography, Abdominal. Renin / blood. Sensitivity and Specificity. Tomography, X-Ray Computed

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  • (PMID = 17679462.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Iodine Radioisotopes; 30461-91-7 / 19-Iodocholesterol; 4964P6T9RB / Aldosterone; 6B3QJ94C7P / 6-iodomethylcholesterol; EC 3.4.23.15 / Renin; WI4X0X7BPJ / Hydrocortisone; X4W3ENH1CV / Norepinephrine; YKH834O4BH / Epinephrine
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78. Huang KH, Chung SD, Chen SC, Chueh SC, Pu YS, Lai MK, Lin WC: Clinical and pathological data of 10 malignant pheochromocytomas: long-term follow up in a single institute. Int J Urol; 2007 Mar;14(3):181-5
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  • We report the clinical and histological data of long-term follow up in 10 patients with malignant pheochromocytoma.
  • METHODS: The clinical charts of 10 patients with malignant pheochromocytoma from a single institute were reviewed.
  • The diagnosis of pheochromocytoma was confirmed at surgery.
  • Close long-term follow up for more than 10 years after surgery is necessary in patients with pheochromocytoma.
  • The possibility of malignancy should be kept in mind even though the initial pathology is benign.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / therapy. Adrenalectomy. Antineoplastic Agents / therapeutic use. Pheochromocytoma

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  • (PMID = 17430251.001).
  • [ISSN] 0919-8172
  • [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
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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79. Gao B, Sun Y, Liu Z, Meng F, Shi B, Liu Y, Xu Z: A logistic regression model for predicting malignant pheochromocytomas. J Cancer Res Clin Oncol; 2008 Jun;134(6):631-4
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  • In all 130 cases with malignant or benign PCCs, 15 predictive variables were observed and entered in the logistic regression analysis in a backward stepwise way.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis

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  • (PMID = 17999082.001).
  • [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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80. Igaz P, Wiener Z, Szabó P, Falus A, Gaillard RC, Horányi J, Rácz K, Tulassay Z: Functional genomics approaches for the study of sporadic adrenal tumor pathogenesis: clinical implications. J Steroid Biochem Mol Biol; 2006 Oct;101(2-3):87-96
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Some studies have already been published describing gene expression profiles of benign and malignant adrenocortical tumors and phaeochromocytomas.
  • [MeSH-minor] Gene Expression Profiling. Gene Expression Regulation, Neoplastic. Humans. Models, Biological. Molecular Biology / methods. Pheochromocytoma / genetics

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  • (PMID = 16891114.001).
  • [ISSN] 0960-0760
  • [Journal-full-title] The Journal of steroid biochemistry and molecular biology
  • [ISO-abbreviation] J. Steroid Biochem. Mol. Biol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 89
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81. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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

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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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82. de Bruijne EL, Burgmans JP, Krestin GP, Pols HA, van den Meiracker AH, de Herder WW: [Adrenal incidentaloma: a clinical problem related to imaging]. Ned Tijdschr Geneeskd; 2005 Aug 13;149(33):1821-6
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  • Hormonal evaluation and imaging showed a benign non-hyperactive functioning adenoma in one patient and a pheochromocytoma in the other.
  • [MeSH-major] Adenoma / diagnosis. Adrenal Gland Neoplasms / diagnosis. Incidental Findings. Pheochromocytoma / diagnosis

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  • (PMID = 16128177.001).
  • [ISSN] 0028-2162
  • [Journal-full-title] Nederlands tijdschrift voor geneeskunde
  • [ISO-abbreviation] Ned Tijdschr Geneeskd
  • [Language] dut
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Netherlands
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83. Iacconi P, Donatini G, Iacconi C, De Bartolomeis C, Cucinotta M, Puccini M, Miccoli P: Unexpected histological findings of lesions diagnosed in the adrenal region in a series of 420 patients submitted to adrenal surgery. Review of our experience. J Endocrinol Invest; 2008 Oct;31(10):873-6
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  • Pre-operative diagnosis was: incidentaloma (34.0% of patients), Conn's adenoma (29.0%), Cushing's adenoma (13.9%), pheochromocytoma (8.8%), suspicious metastasis (7.3%), Cushing's disease (6.0%), other (1.0%).
  • Histology demonstrated a benign neurogenic tumor in 10 patients.

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  • (PMID = 19092291.001).
  • [ISSN] 1720-8386
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
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84. Fariña Pérez LA: [Laparoscopic adrenalectomy]. Actas Urol Esp; 2006 May;30(5):510-2
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  • Laparoscopic extirpation of the suprarenal gland is considered the 'gold standard' of surgery for benign conditions, but its indication in suprarenal cancer is still controversial.
  • In this article, we review the pros and cons of the laparoscopic approach in the different disorders that affect the adrenal gland, pheochromocytoma, cancer, partial and bilateral adrenalectomy, etc.

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  • (PMID = 16884103.001).
  • [ISSN] 0210-4806
  • [Journal-full-title] Actas urologicas españolas
  • [ISO-abbreviation] Actas Urol Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 15
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85. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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86. Conlon JM: Granin-derived peptides as diagnostic and prognostic markers for endocrine tumors. Regul Pept; 2010 Nov 30;165(1):5-11
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  • Measurement of concentrations of a second secretogranin II-derived peptide, EM-66 in tumor tissue has been used to differentiate between benign and malignant pheochromocytoma.

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  • [Copyright] Copyright © 2009 Elsevier B.V. All rights reserved.
  • (PMID = 19931574.001).
  • [ISSN] 1873-1686
  • [Journal-full-title] Regulatory peptides
  • [ISO-abbreviation] Regul. Pept.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Chromogranin A; 0 / Chromogranin B; 0 / Chromogranins; 0 / Secretogranin II
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87. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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88. Cadden IS, Atkinson AB, Johnston BT, Pogue K, Connolly R, McCance D, Ardill JE, Russell CF, McGinty A: Cyclooxygenase-2 expression correlates with phaeochromocytoma malignancy: evidence for a Bcl-2-dependent mechanism. Histopathology; 2007 Dec;51(6):743-51
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  • [Title] Cyclooxygenase-2 expression correlates with phaeochromocytoma malignancy: evidence for a Bcl-2-dependent mechanism.
  • There are no histological features which reliably differentiate benign from malignant phaeochromocytomas.
  • The aim of the study was to evaluate cyclooxygenase (COX)-2 and Bcl-2 as tissue-based biomarkers of phaeochromocytoma prognosis.
  • METHODS AND RESULTS: COX-2 and Bcl-2 expression were examined immunohistochemically in tissue from 41 sporadic phaeochromocytoma patients followed up for a minimum of 5 years after diagnosis.
  • CONCLUSIONS: COX-2 and Bcl-2 may promote phaeochromocytoma malignancy, and these oncoproteins may be valuable surrogate markers of an aggressive tumour phenotype.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Biomarkers, Tumor / analysis. Cyclooxygenase 2 / biosynthesis. Pheochromocytoma / metabolism. Proto-Oncogene Proteins c-bcl-2 / biosynthesis

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  • (PMID = 17916073.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Proto-Oncogene Proteins c-bcl-2; EC 1.14.99.1 / Cyclooxygenase 2
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89. Liao CH, Lai MK, Li HY, Chen SC, Chueh SC: Laparoscopic adrenalectomy using needlescopic instruments for adrenal tumors less than 5cm in 112 cases. Eur Urol; 2008 Sep;54(3):640-6
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  • METHODS: Transperitoneal laparoscopic adrenalectomy with needlescopic instruments for 112 patients with presumptively benign adrenal tumors < 5cm were enrolled from July 2000 to February 2005.
  • Larger tumors, previous abdominal surgery, and pheochromocytoma group were independent risk factors of a longer operative time.
  • Except for one leiomyosarcoma, all other tumors were benign adrenal pathologies (57 aldosterone-producing adenomas, 23 Cushing's adenomas, 12 pheochromocytomas, and 20 incidentalomas).

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  • [CommentIn] Eur Urol. 2008 Sep;54(3):493-5; author reply 495-7 [18281144.001]
  • [CommentIn] Nat Clin Pract Urol. 2009 Jan;6(1):10-1 [19048004.001]
  • (PMID = 18164803.001).
  • [ISSN] 0302-2838
  • [Journal-full-title] European urology
  • [ISO-abbreviation] Eur. Urol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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90. Gimm O, Krause U, Brauckhoff M, Hoang-Vu C, Dralle H: Distinct expression of galectin-3 in pheochromocytomas. Ann N Y Acad Sci; 2006 Aug;1073:571-7
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  • Unless distant metastases or local invasion are present, the diagnosis of malignant pheochromocytoma is challenging.
  • Four malignant and 24 benign (10 sporadic, 14 hereditary) pheochromocytomas were analyzed for the expression of galectin-3.
  • One malignant pheochromocytoma with distant metastases showed strong and one malignant undifferentiated pheochromocytoma with local invasion showed partly strong cytoplasmic staining.
  • Nine of 10 sporadic and all hereditary benign pheochromocytomas had absent/weak staining.
  • One benign sporadic pheochromocytoma had moderate cytoplasmic staining.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Galectin 3 / genetics. Pheochromocytoma / genetics

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  • (PMID = 17102125.001).
  • [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
  • [Chemical-registry-number] 0 / Galectin 3
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91. Pugliese R, Boniardi M, Sansonna F, Maggioni D, De Carli S, Costanzi A, Scandroglio I, Ferrari GC, Di Lernia S, Magistro C, Loli P, Grossrubatscher E: Outcomes of laparoscopic adrenalectomy. Clinical experience with 68 patients. Surg Oncol; 2008 Jul;17(1):49-57
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  • Adrenal masses averaged 5.4cm in size (range 1.2-13cm) and 56.7g in weight (range 10-265) including 71 benign and 8 malignant lesions.
  • Three left adrenalectomies for pheochromocytoma were robot-assisted.
  • Patients affected by hormone secreting or bilateral lesions, by unilateral or bilateral pheochromocytoma and by bilateral Cushing's disease were transferred to the endocrinological ward so that their overall hospital stay was prolonged to 9+/-2.8 days on average (range 7-17).

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  • (PMID = 17949973.001).
  • [ISSN] 0960-7404
  • [Journal-full-title] Surgical oncology
  • [ISO-abbreviation] Surg Oncol
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Netherlands
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92. Imani F, Agopian VG, Auerbach MS, Walter MA, Imani F, Benz MR, Dumont RA, Lai CK, Czernin JG, Yeh MW: 18F-FDOPA PET and PET/CT accurately localize pheochromocytomas. J Nucl Med; 2009 Apr;50(4):513-9
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  • Successful treatment of pheochromocytoma requires accurate diagnosis and localization of tumors.
  • Herein, we investigated the accuracy of PET using 3,4-dihydroxy-6-(18)F-fluoro-phenylalanine ((18)F-FDOPA), an amino acid transporter substrate, as an independent marker for detection of benign and malignant pheochromocytomas.
  • METHODS: The study comprised 25 consecutive patients (9 men, 16 women) whose median age was 51 y (range, 25-68 y), with known or suspected pheochromocytoma.
  • SUVmax of the benign and malignant tumors was compared.
  • Histology confirmed pheochromocytoma or paraganglioma in 11 cases (8 adrenal, including 2 malignant tumors, and 3 extraadrenal, including 1 malignant tumor).
  • The diagnosis of pheochromocytoma was established by follow-up in 2 additional patients (1 adrenal and 1 unknown location) and ruled out in 6 patients.
  • Visual analysis detected and localized pheochromocytoma in 11 of 13 patients without false-positive results (sensitivity, 84.6%; specificity, 100%; accuracy, 92%).
  • The Mann-Whitney nonparametric test did not demonstrate a statistically significant difference between the SUVmax of (18)F-FDOPA in malignant and benign tumors.
  • CONCLUSION: (18)F-FDOPA PET and PET/CT are highly sensitive and specific tools that can provide additional independent information for diagnosis and localization of benign and malignant pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Dihydroxyphenylalanine / analogs & derivatives. Image Enhancement / methods. Pheochromocytoma / diagnosis. Positron-Emission Tomography / methods. Subtraction Technique. Tomography, X-Ray Computed / methods

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  • (PMID = 19289420.001).
  • [ISSN] 0161-5505
  • [Journal-full-title] Journal of nuclear medicine : official publication, Society of Nuclear Medicine
  • [ISO-abbreviation] J. Nucl. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 2C598205QX / fluorodopa F 18; 63-84-3 / Dihydroxyphenylalanine
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93. Oller AR, Kirkpatrick DT, Radovsky A, Bates HK: Inhalation carcinogenicity study with nickel metal powder in Wistar rats. Toxicol Appl Pharmacol; 2008 Dec 1;233(2):262-75
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  • Adrenal gland pheochromocytomas (benign and malignant) in males and combined cortical adenomas/carcinomas in females were induced in a dose-dependent manner by the nickel metal exposure.
  • [MeSH-minor] Adrenal Cortex Neoplasms / chemically induced. Adrenal Gland Neoplasms / chemically induced. Adrenocortical Adenoma / chemically induced. Adrenocortical Carcinoma / chemically induced. Animals. Dose-Response Relationship, Drug. Female. Male. Models, Animal. Occupational Exposure / adverse effects. Pheochromocytoma / chemically induced. Powders. Rats. Rats, Wistar. Respiratory Tract Neoplasms / epidemiology. Respiratory Tract Neoplasms / etiology. Sex Factors

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  • (PMID = 18822311.001).
  • [ISSN] 1096-0333
  • [Journal-full-title] Toxicology and applied pharmacology
  • [ISO-abbreviation] Toxicol. Appl. Pharmacol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Carcinogens; 0 / Powders; 7OV03QG267 / Nickel
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94. 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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95. Kanthan R, Gomez D, Senger JL, Kanthan SC: Endoscopic biopsies of duodenal polyp/mass lesions: a surgical pathology review. J Clin Pathol; 2010 Oct;63(10):921-5
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  • The accurate diagnoses of metastatic renal cell carcinoma, metastatic phaeochromocytoma and metastatic malignant melanoma, respectively, were confirmed with retrospective analysis of previous clinical and pathological records.
  • 33% of these lesions were malignant and 67% were benign/normal.
  • 41% of the benign cases had no significant pathological abnormalities.
  • [MeSH-minor] Adolescent. Adult. Aged, 80 and over. Biopsy. Carcinoma, Renal Cell / pathology. Carcinoma, Renal Cell / secondary. Child. Child, Preschool. Duodenoscopy / methods. Humans. Infant. Kidney Neoplasms. Male. Melanoma / pathology. Melanoma / secondary. Middle Aged. Pheochromocytoma / pathology. Pheochromocytoma / secondary. Retrospective Studies. Young Adult

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  • (PMID = 20876326.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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96. Strong VE, Kennedy T, Al-Ahmadie H, Tang L, Coleman J, Fong Y, Brennan M, Ghossein RA: Prognostic indicators of malignancy in adrenal pheochromocytomas: clinical, histopathologic, and cell cycle/apoptosis gene expression analysis. Surgery; 2008 Jun;143(6):759-68
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  • The histologic differentiation between benign and malignant tumors is difficult, the latter diagnosed by the presence of metastatic disease or recurrence.
  • AIM: To determine if postoperative histologic evaluation using the previously proposed Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and cell cycle/apoptosis markers can predict patients at risk for recurrence.
  • This pheochromocytoma scoring system is based on the presence of 12 different histologic parameters, including tumor necrosis, mitotic rate, tumor cell spindling, and the presence of large cell nests.
  • In addition, we constructed a tissue microarray of all 5 malignant tumors and 41 of the benign tumors.
  • RESULTS: Forty-three patients had a benign clinical course while 5 patients harbored a clinically malignant pheochromocytoma.
  • Tumor necrosis (focal or confluent) was a particularly powerful indicator of malignancy present in 4 of 5 patients (80%) with malignant tumors, but only in 3 of 42 cases (7%) with benign neoplasms (P = .0009).
  • There was a highly significant difference in PASS scores between benign and malignant cases (P = .0003).
  • There was no difference in staining between benign and malignant pheochromocytomas using p53, Bcl-2, mdm-2, cyclin D1, p21, and p27. CONCLUSIONS:.
  • (1) A PASS score of <4 predicted benign pheochromocytomas. (2) All malignant pheochromocytomas had a PASS score >/=6, which was significantly higher compared with the benign lesions.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / pathology. Apoptosis. Cell Cycle. Gene Expression Regulation, Neoplastic. Neoplasm Recurrence, Local / genetics. Pheochromocytoma / diagnosis. Pheochromocytoma / pathology


97. Zografos GN, Vasiliadis G, Farfaras AN, Aggeli C, Digalakis M: Laparoscopic surgery for malignant adrenal tumors. JSLS; 2009 Apr-Jun;13(2):196-202
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  • The laparoscopic approach, the gold standard for benign adrenal diseases, is controversial for malignant adrenal tumors.

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  • (PMID = 19660215.001).
  • [ISSN] 1086-8089
  • [Journal-full-title] JSLS : Journal of the Society of Laparoendoscopic Surgeons
  • [ISO-abbreviation] JSLS
  • [Language] ENG
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 63
  • [Other-IDs] NLM/ PMC3015945
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98. Waguespack SG, Rich T, Grubbs E, Ying AK, Perrier ND, Ayala-Ramirez M, Jimenez C: A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab; 2010 May;95(5):2023-37
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  • [Title] A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma.
  • National Library of Medicine by using the search terms pheochromocytoma and paraganglioma.
  • Although most pediatric PHEO/PGL are benign, these tumors can occasionally metastasize, a condition for which no curative treatment exists.
  • [MeSH-major] Paraganglioma / etiology. Pheochromocytoma / etiology

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  • (PMID = 20215394.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein; VTD58H1Z2X / Dopamine
  • [Number-of-references] 139
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99. Thouënnon E, Pierre A, Tanguy Y, Guillemot J, Manecka DL, Guérin M, Ouafik L, Muresan M, Klein M, Bertherat J, Lefebvre H, Plouin PF, Yon L, Anouar Y: Expression of trophic amidated peptides and their receptors in benign and malignant pheochromocytomas: high expression of adrenomedullin RDC1 receptor and implication in tumoral cell survival. Endocr Relat Cancer; 2010 Sep;17(3):637-51
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  • [Title] Expression of trophic amidated peptides and their receptors in benign and malignant pheochromocytomas: high expression of adrenomedullin RDC1 receptor and implication in tumoral cell survival.
  • Pheochromocytomas are catecholamine-producing tumors which are generally benign, but which can also present as or develop into malignancy.
  • Here, we investigated the expression of trophic amidated peptides, including pituitary adenylate cyclase-activating polypeptide (PACAP), neuropeptide Y (NPY), and adrenomedullin (AM), and their receptors in benign and malignant pheochromocytomas in order to assess their potential role in chromaffin cell tumorigenesis and malignant transformation.
  • Although median gene expression or peptide levels were systematically lower in malignant compared to benign tumors, no statistically significant difference was found.
  • Among all the receptors of these peptides that were analyzed, only the AM receptor RDC1 displayed a differential expression between benign and malignant pheochromocytomas.
  • This receptor exhibited a fourfold higher expression in malignant than in benign tumors.
  • AM and stromal cell-derived factor 1, which has also been described as a ligand for RDC1, increased the number of human pheochromocytoma cells in primary culture and exerted anti-apoptotic activity on rat pheochromocytoma PC12 cells.
  • This study shows the expression of several trophic peptides and their receptors in benign and malignant pheochromocytomas, and suggests that AM and its RDC1 receptor could be involved in chromaffin cell tumorigenesis through pro-survival effects.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Pheochromocytoma / metabolism. Pituitary Adenylate Cyclase-Activating Polypeptide / metabolism. Receptors, Peptide / biosynthesis

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  • (PMID = 20483910.001).
  • [ISSN] 1479-6821
  • [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 / Formazans; 0 / Neuropeptide Y; 0 / Pituitary Adenylate Cyclase-Activating Polypeptide; 0 / RNA, Messenger; 0 / RNA, Small Interfering; 0 / Receptors, Adrenomedullin; 0 / Receptors, Neuropeptide Y; 0 / Receptors, Peptide; 0 / Receptors, Pituitary Adenylate Cyclase-Activating Polypeptide; 0 / Tetrazolium Salts; 148498-78-6 / Adrenomedullin; 23305-68-2 / MTT formazan
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100. Vezzosi D, Bouisson M, Escourrou G, Laurell H, Selves J, Seguin P, Pradayrol L, Caron P, Buscail L: Clinical utility of telomerase for the diagnosis of malignant well-differentiated endocrine tumours. Clin Endocrinol (Oxf); 2006 Jan;64(1):63-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: The distinction between benign and malignant well-differentiated endocrine tumours is hard to achieve.
  • The aim of the present study was to determine whether detection of telomerase or quantification of human telomerase reverse transcriptase protein subunit (hTERT) differ between benign and malignant endocrine tumours.
  • Based on clinical and histopathological criteria, tumours were categorized with the most recent WHO classification as 'benign' (n = 14), 'uncertain' (n = 5) or 'malignant' (n = 12) with (n = 7) or without (n = 5) metastasis after a mean follow-up of 40.4 +/- 25.8 months (4-122 months).
  • RESULTS: Telomerase activity was detected in 7 malignant and metastatic tumours, in 1 malignant tumour without metastases, in 1 uncertain tumour and in 1 benign tumour. hTERT mRNA levels were significantly higher in malignant endocrine tumours with or without metastases (P = 0.001) when compared to benign tumours.
  • [MeSH-minor] Adrenal Gland Neoplasms / diagnosis. Adult. Aged. Carcinoid Tumor / diagnosis. Female. Gastrinoma / diagnosis. Glucagonoma / diagnosis. Humans. Insulinoma / diagnosis. Intestinal Neoplasms / diagnosis. Lung Neoplasms / diagnosis. Male. Middle Aged. Pancreatic Neoplasms / diagnosis. Pheochromocytoma / diagnosis. Predictive Value of Tests. Retrospective Studies. Reverse Transcriptase Polymerase Chain Reaction. Tumor Suppressor Protein p53 / analysis

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  • (PMID = 16402930.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA-Binding Proteins; 0 / Tumor Suppressor Protein p53; EC 2.7.7.49 / Telomerase
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