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1. Al-Harthy M, Al-Harthy S, Al-Otieschan A, Velagapudi S, Alzahrani AS: Comparison of pheochromocytomas and abdominal and pelvic paragangliomas with head and neck paragangliomas. Endocr Pract; 2009 Apr;15(3):194-202
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • We searched pathology and medical record databases under the terms pheochromocytoma, paraganglioma, head and neck tumors, carotid body tumors, glomus jugulare, and neuroendocrine tumors.
  • We compared clinical, radiologic, and pathologic characteristics, as well as management and outcomes, between patients with pheochromocytoma, abdominal and pelvic paraganglioma, and head and neck paraganglioma.
  • RESULTS: Eighty-six patients were included (46 with head and neck paraganglioma, 23 with pheochromocytoma, and 17 with abdominal or pelvic paraganglioma).
  • Compared with patients with head and neck paraganglioma, patients with pheochromocytoma or abdominal and pelvic paraganglioma were younger (35.7 +/- 16 years vs 43 +/- 17 years, P = .042) and were more likely to have the classic triad associated with catecholamine hypersecretion of palpitation, excessive sweating, and headache (40% vs 0%, P<.001); hypertension (70% vs 37%, P = .005); and benign tumors (65% vs 43%, P = .03).
  • Patients with head and neck paraganglioma and patients with pheochromocytoma/abdominal and pelvic paraganglioma were not different in female to male ratios (27:19 vs 29:11, respectively, P = .18), tumor size (5.8 +/- 2.7 cm vs 5.7 +/- 3 cm, respectively; P = .85), or remission rate (43% vs 60%, respectively, P = .13).
  • CONCLUSIONS: Head and neck paraganglioma are similar to pheochromocytoma and abdominal and pelvic paraganglioma in size and outcome, but occur at an older age, lack hyperadrenergic manifestations, and are more likely to have local pressure effects and result in persistent disease.
  • [MeSH-major] Abdominal Neoplasms / epidemiology. Adrenal Gland Neoplasms / epidemiology. Head and Neck Neoplasms / epidemiology. Paraganglioma / epidemiology. Pelvic Neoplasms / epidemiology. Pheochromocytoma / epidemiology

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  • (PMID = 19364686.001).
  • [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] Comparative Study; Journal Article
  • [Publication-country] United States
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2. Sharma R, Ganpule A, Veeramani M, Sabnis RB, Desai M: Laparoscopic management of adrenal lesions larger than 5 cm in diameter. Urol J; 2009;6(4):254-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Laparoscopic management of adrenal lesions larger than 5 cm in diameter.
  • They were compared with patients whose adrenal tumors were smaller than 5 cm.
  • There were no intra-operative complications except for 1 incidence of supraventricular tachycardia in a patient with a large pheochromocytoma.
  • Histopathology of large tumors revealed 16 benign tumors (8 pheochromocytomas, 4 adenomas, 2 ganglioneuromas, 1 pseudocyst, and 1 myelolipoma) and 3 malignancies, of which 1 was primary adrenocortical carcinoma and 2 were metastatic renal cell carcinoma.
  • CONCLUSION: In experienced hands, laparoscopic adrenalectomy is safe and feasible for large functioning adrenal tumors.
  • Large adrenal tumors suspicious of harboring malignancy with no peri-adrenal involvement can be tackled laparoscopically.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy

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  • (PMID = 20027553.001).
  • [ISSN] 1735-546X
  • [Journal-full-title] Urology journal
  • [ISO-abbreviation] Urol J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Iran
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3. Kupka S, Haack B, Zdichavsky M, Mlinar T, Kienzle C, Bock T, Kandolf R, Kroeber SM, Königsrainer A: Large proportion of low frequency microsatellite-instability and loss of heterozygosity in pheochromocytoma and endocrine tumors detected with an extended marker panel. J Cancer Res Clin Oncol; 2008 Apr;134(4):463-71
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Large proportion of low frequency microsatellite-instability and loss of heterozygosity in pheochromocytoma and endocrine tumors detected with an extended marker panel.
  • PURPOSE: Pheochromocytoma (PCC) is a usually benign tumor originated in the majority of patients from the adrenal medulla.
  • [MeSH-major] Endocrine Gland Neoplasms / genetics. Loss of Heterozygosity. Microsatellite Instability. Microsatellite Repeats. Pheochromocytoma / genetics

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  • (PMID = 17828419.001).
  • [ISSN] 0171-5216
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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4. Mischke K, Graf J, Rulands D, Koch KC, Hanrath P, Janssens U: [Cardiogenic shock in a 61 year old female with recurrent panic attacks]. Internist (Berl); 2005 May;46(5):580-5
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  • Conducting an abdominal ultrasound we detected a mass at the right adrenal gland.
  • The suspected diagnosis of pheochromocytoma was confirmed by elevated catecholamine levels in the urine and a CT scan.
  • After recompensation and subsequent administration of phenoxybenzamine a benign pheochromocytoma was resected.
  • A massive catecholamine secretion due to a pheochromocytoma can lead to a cardiogenic shock and multiple organ failure.
  • In patients with recurrent panic attacks and hypertension a pheochromocytoma should be included in the differential diagnosis.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Panic Disorder / diagnosis. Panic Disorder / prevention & control. Pheochromocytoma / diagnosis. Secondary Prevention. Shock, Cardiogenic / diagnosis. Shock, Cardiogenic / prevention & control
  • [MeSH-minor] Cardiomyopathies / diagnosis. Cardiomyopathies / etiology. Diagnosis, Differential. Female. Humans. Middle Aged

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  • (PMID = 15806412.001).
  • [ISSN] 0020-9554
  • [Journal-full-title] Der Internist
  • [ISO-abbreviation] Internist (Berl)
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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5. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Endoscopic treatment of large primary adrenal tumours.
  • BACKGROUND: Endoscopic adrenalectomy has become the treatment of choice for small benign adrenal tumours but should not be used for malignant lesions.
  • It is debatable whether large and therefore potentially malignant primary adrenal tumours should be removed by minimally invasive techniques.
  • METHODS: Three hundred and eighty primary adrenal tumours in 368 patients (142 male and 226 female; mean(s.d.) age 48.9(14.4) years) were excised by laparoscopic or retroperitoneoscopic adrenalectomy.
  • Adrenal neoplasias exceeded 6 cm in diameter (range 6-13 cm) in 33 patients (18 male and 15 female; age 42.6(14.2) years).
  • [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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6. Guillemot J, Compagnon P, Cartier D, Thouennon E, Bastard C, Lihrmann I, Pichon P, Thuillez C, Plouin PF, Bertherat J, Anouar Y, Kuhn JM, Yon L, Lefebvre H: Metoclopramide stimulates catecholamine- and granin-derived peptide secretion from pheochromocytoma cells through activation of serotonin type 4 (5-HT4) receptors. Endocr Relat Cancer; 2009 Mar;16(1):281-90
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metoclopramide stimulates catecholamine- and granin-derived peptide secretion from pheochromocytoma cells through activation of serotonin type 4 (5-HT4) receptors.
  • The aim of the study was to investigate the mechanism of action of metoclopramide and expression of serotonin type 4 (5-HT(4)) receptors in pheochromocytoma tissues.
  • Tissue explants, obtained from 18 pheochromocytomas including the tumor removed from a 46-year-old female patient who experienced life-threatening hypertension crisis after metoclopramide administration and 17 additional pheochromocytomas (9 benign and 8 malignant) were studied.
  • Cultured pheochromocytoma cells derived from the patient who previously received metoclopramide were incubated with metoclopramide and various 5-HT(4) receptor ligands.
  • Catecholamine- and granin-derived peptide concentrations were measured in pheochromocytoma cell incubation medium by HPLC and radioimmunological assays.
  • All 5-HT(4) receptor agonists must therefore be contraindicated in patients with proven or suspected pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Dopamine Antagonists / pharmacology. Metoclopramide / pharmacology. Pheochromocytoma / drug therapy. Receptors, Serotonin, 5-HT4 / genetics
  • [MeSH-minor] Adrenal Medulla / cytology. Adrenal Medulla / drug effects. Catecholamines / secretion. Chromogranins / secretion. Cisapride / pharmacology. Domperidone / pharmacology. Female. Humans. Middle Aged. RNA, Messenger / metabolism. Retrospective Studies. Reverse Transcriptase Polymerase Chain Reaction. Serotonin Receptor Agonists / pharmacology. Tumor Cells, Cultured

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  • (PMID = 18948374.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Chromogranins; 0 / Dopamine Antagonists; 0 / RNA, Messenger; 0 / Serotonin Receptor Agonists; 158165-40-3 / Receptors, Serotonin, 5-HT4; 5587267Z69 / Domperidone; L4YEB44I46 / Metoclopramide; UVL329170W / Cisapride
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7. Tömböl Z, Eder K, Kovács A, Szabó PM, Kulka J, Likó I, Zalatnai A, Rácz G, Tóth M, Patócs A, Falus A, Rácz K, Igaz P: MicroRNA expression profiling in benign (sporadic and hereditary) and recurring adrenal pheochromocytomas. Mod Pathol; 2010 Dec;23(12):1583-95
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  • [Title] MicroRNA expression profiling in benign (sporadic and hereditary) and recurring adrenal pheochromocytomas.
  • The objective of our study was to perform microRNA expression profiling in sporadic and hereditary benign, and recurring adrenomedullary tumors.
  • A total of 21 formalin-fixed paraffin-embedded samples (sporadic benign, multiple endocrine neoplasia 2, von Hippel-Lindau disease, sporadic recurring) were subjected to microRNA expression profiling using microarrays.
  • Furthermore, microRNA expression profiles of a malignant pheochromocytoma and a pair of primary and recurrent tumors were studied by TaqMan Human MicroRNA Cards.
  • Five of these were validated by real-time RT-PCR. miR-139-3p, miR-541 and miR-765 were significantly differentially expressed between sporadic benign and von Hippel-Lindau-related pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Profiling. MicroRNAs / genetics. Pheochromocytoma / genetics

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  • (PMID = 20818339.001).
  • [ISSN] 1530-0285
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MicroRNAs
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8. Pacak K, Ilias I, Adams KT, Eisenhofer G: Biochemical diagnosis, localization and management of pheochromocytoma: focus on multiple endocrine neoplasia type 2 in relation to other hereditary syndromes and sporadic forms of the tumour. J Intern Med; 2005 Jan;257(1):60-8
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  • [Title] Biochemical diagnosis, localization and management of pheochromocytoma: focus on multiple endocrine neoplasia type 2 in relation to other hereditary syndromes and sporadic forms of the tumour.
  • Approximately 50% of patients with multiple endocrine neoplasia (MEN) 2A or 2B develop pheochromocytoma.
  • These tumours are almost exclusively benign and localized in the adrenal glands.
  • About one-third are bilateral at initial diagnosis.
  • Amongst patients with pheochromocytoma, those with MEN 2A have subtler symptoms compared to those with sporadic disease.
  • Since pheochromocytomas in patients with MEN 2 often secrete catecholamines episodically (but metabolize them continuously to metanephrines), the first choice for biochemical diagnosis is the measurement of free metanephrines in plasma, with urinary fractionated metanephrines being the second choice.
  • In patients with biochemically proven MEN 2-related pheochromocytoma, anatomical imaging of the adrenals (with either computerized tomography or magnetic resonance) should be obtained next.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Multiple Endocrine Neoplasia / complications. Pheochromocytoma / diagnosis


9. 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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  • [Title] Adrenal cysts: an institutional experience.
  • INTRODUCTION: Adrenal cysts are rare clinical entities.
  • We report our institutional experience with adrenal cysts and also assess various management options.
  • MATERIAL AND METHODS: Over the past 15 years the Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, has had seven cases of adrenal cysts, of which two were functional: one patient had Cushing's syndrome and the other patient had pheochromocytoma.
  • It is generally agreed that a hormonal work-up is necessary in all cases of adrenal cysts to rule out a sub-clinical disease.
  • Adrenal neoplasms, including adrenocortical carcinomas, can be associated with cysts that are benign in appearance.
  • However, surgical excision provides a definite histopathological diagnosis and also removes the fear of future complications such as hemorrhage into the cyst and local pressure effects due to the tumor.
  • CONCLUSIONS: Given that the adrenals are a vascular gland and taking into consideration the possibilities of bleeding and complications in the cyst, our treatment of choice is the elective excision of adrenal cysts.
  • [MeSH-major] Academies and Institutes / statistics & numerical data. Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / surgery. Adrenalectomy / methods. Cysts / diagnosis. Cysts / surgery
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Follow-Up Studies. Humans. India. Laparoscopy. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed

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  • (PMID = 16983481.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
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10. Brouwers FM, Elkahloun AG, Munson PJ, Eisenhofer G, Barb J, Linehan WM, Lenders JW, De Krijger R, Mannelli M, Udelsman R, Ocal IT, Shulkin BL, Bornstein SR, Breza J, Ksinantova L, Pacak K: Gene expression profiling of benign and malignant pheochromocytoma. Ann N Y Acad Sci; 2006 Aug;1073:541-56
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  • [Title] Gene expression profiling of benign and malignant pheochromocytoma.
  • There are currently no reliable diagnostic and prognostic markers or effective treatments for malignant pheochromocytoma.
  • Other subgroups of tumors included those defined by tissue norepinephrine compared to epinephrine contents (i.e., noradrenergic versus adrenergic phenotypes), adrenal versus extra-adrenal locations, and presence of germline mutations of genes predisposing to the tumor.
  • Correcting for the confounding influence of noradrenergic versus adrenergic catecholamine phenotype by the analysis of variance revealed a larger and more accurate number of genes that discriminated benign from malignant pheochromocytomas than when the confounding influence of catecholamine phenotype was not considered.
  • Seventy percent of these genes were underexpressed in malignant compared to benign tumors.
  • Similarly, 89% of genes were underexpressed in malignant primary tumors compared to benign tumors, suggesting that malignant potential is largely characterized by a less-differentiated pattern of gene expression.
  • Further progress on the basis of this database can be made from follow-up confirmatory studies, application of bioinformatics approaches for data mining and pathway analyses, testing in pheochromocytoma cell culture and animal model systems, and retrospective and prospective studies of diagnostic markers.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Profiling. Pheochromocytoma / genetics

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  • (PMID = 17102123.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z99 CT999999; United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
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11. Welsh SJ, Khan S: Radiological localizing techniques in adrenal tumors. Minerva Endocrinol; 2009 Jun;34(2):161-9
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  • [Title] Radiological localizing techniques in adrenal tumors.
  • The characterisation of adrenal lesions is a common radiological dilemma.
  • Incidental adrenal lesions are commonly detected with computed tomography (CT), and lesion characterisation is critical.
  • The prevalence of incidental adrenal lesions has been reported to be 2.3% at autopsy and 0.5-2% with abdominal CT.
  • Although the majority of adrenal lesions are benign, in patients with an extra-adrenal primary cancer the probability of an adrenal mass being a metastasis is 52%.
  • Unfortunately, there may be significant overlap between the imaging appearances of benign lesions such as lipid-poor adenomas and malignant lesions, particularly metastases and small adrenal carcinomas.
  • This review highlights recent advances in radiological imaging of adrenal lesions and we discuss the relative merits of CT and magnetic resonance imaging to aid the identification of benign and malignant adrenal lesions and their roles, in combination with biochemical and clinical data, in recognizing common pathologies such as adrenal adenoma, phaeochromocytoma, carcinoma and metastases.
  • We also discuss the radiological characteristics of rarer adrenal lesions including lymphoma, neuroblastic tumours (neuroblastoma, ganglioneuroblastoma, and ganglioneuroma), lipomatous tumours (myelolipoma, angiolipoma, teratoma, lipoma and liposarcoma), in addition to hemangioma, hemangiosarcoma and leiomyosarcoma.
  • [MeSH-major] Adrenal Gland Neoplasms / radiography. Incidental Findings. Tomography, X-Ray Computed
  • [MeSH-minor] Adrenal Cortex Neoplasms / radiography. Adrenocortical Adenoma / radiography. Adrenocortical Carcinoma / radiography. Diagnosis, Differential. Ganglioneuroblastoma / radiography. Ganglioneuroma / radiography. Humans. Lymphoma / radiography. Magnetic Resonance Imaging. Neoplasm Metastasis. Neoplasms, Adipose Tissue / radiography. Neoplasms, Vascular Tissue / radiography. Neuroblastoma / radiography. Pheochromocytoma / radiography. Predictive Value of Tests. Sensitivity and Specificity. Teratoma / radiography

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  • (PMID = 19471240.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 42
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12. Lifante JC, Cenedese A, Fernandez Vila JM, Peix JL: [Impact of laparoscopy on the management of adrenal diseases. A retrospective study of 220 patients]. Ann Chir; 2005 Oct;130(9):547-52
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  • [Title] [Impact of laparoscopy on the management of adrenal diseases. A retrospective study of 220 patients].
  • OBJECTIVE: Soon after its introduction in 1992, laparoscopic adrenalectomy became the gold standard in the surgical management of most adrenal tumors.
  • RESULTS: The indications of adrenalectomy were: Cushing syndrome 18%, pheochromocytoma 31%, Conn syndrome 16%, incidentaloma 21%, and malignant tumours 13%.
  • CONCLUSIONS: This study is consistent with the findings of the literature supporting that there are no indications for the open procedure in case of small benign lesions.
  • The video-asisted adrenalectomy had not changed the management of the adrenal incidentaloma.
  • [MeSH-major] Adrenal Gland Diseases / surgery. Adrenalectomy / methods. Laparoscopy. Video-Assisted Surgery / methods

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  • (PMID = 15993374.001).
  • [ISSN] 0003-3944
  • [Journal-full-title] Annales de chirurgie
  • [ISO-abbreviation] Ann Chir
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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13. Ridho FE, Adam FM, Adam JM: Adrenal incidentaloma. Acta Med Indones; 2009 Apr;41(2):87-93
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  • [Title] Adrenal incidentaloma.
  • Adrenal Incidentaloma is an adrenal tumor, which is unidentified before the imaging procedures conducted for an abnormality which is initially unforeseen as an adrenal disease.
  • Symptoms and/or clinical signs of adrenal tumor do not have to be present prior to a diagnosis.
  • Adrenal Incidentaloma can be divided into non-hypersecreting adrenal adenoma, hypersecreting tumor, primary adrenal carcinoma, other adrenal mass, and metastases.
  • The majority of adrenal tumor is non-hypersecreting adrenal adenoma, but it is always considered as hypersecreting tumor until proven otherwise.
  • Some conditions that can be found due to hormonal activity of adrenal incidentaloma is subclinical Cushing's syndrome, pheochromocytoma, aldosteronoma (Conn's disease), and several tumors which secrete androgen and sex hormone.
  • Diagnostic approach of adrenal incidentaloma is focused on two main problems, which are, whether the lesion is hormonally active even though lacking characteristic clinical signs, and whether the lesion is benign or malignant; thus it needs hormonal and radiologic evaluation, even a fine needle biopsy.
  • The management for adrenal incidentaloma includes surgical removal for hormonally active adrenal tumors, or inactive tumors with size less than 4 cm.
  • [MeSH-major] Adrenal Gland Neoplasms. Aldosterone / blood. Catecholamines / urine. Incidental Findings. Renin / blood
  • [MeSH-minor] Biopsy, Fine-Needle. Diagnosis, Differential. Humans. Indonesia / epidemiology. Morbidity / trends

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  • (PMID = 19390128.001).
  • [ISSN] 0125-9326
  • [Journal-full-title] Acta medica Indonesiana
  • [ISO-abbreviation] Acta Med Indones
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Indonesia
  • [Chemical-registry-number] 0 / Catecholamines; 4964P6T9RB / Aldosterone; EC 3.4.23.15 / Renin
  • [Number-of-references] 38
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14. Feng C, Li HZ, Yan WG, Luo YF, Cao JL: [The expression and significance of chromogranin A and synaptophysin in adrenal gland tumors]. Zhonghua Zhong Liu Za Zhi; 2005 Aug;27(8):486-8
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  • [Title] [The expression and significance of chromogranin A and synaptophysin in adrenal gland tumors].
  • OBJECTIVE: To investigate the expression of chromogranin A (CgA) and synaptophysin (Syn) for differential diagnosis of different kinds of adrenal gland tumors.
  • METHODS: The samples of 69 adrenal gland tumors and 4 normal adrenal glands were immunohistochemically analyzed for the expression of chromogranin A and synaptophysin.
  • RESULTS: In the normal adrenal gland, CgA and Syn was exclusively detected in the medulla.
  • Syn was detected in adrenocortical adenomas 27/28 (96.4%), adrenocortical carcinoma 7/8 (87.5%), pheochromocytoma 24/25 (96.0%) and adrenal metastatic carcinoma 6/8 (75.0%), respectively.
  • CONCLUSION: There is statistically significant difference of CgA expression between adrenalcortical and adrenal medullary tumors, and also between benign and malignant pheochromocytomas.
  • CgA and Syn are immunohistochemically reliable markers in the differential diagnosis of various kinds of adrenal gland tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Chromogranin A / biosynthesis. Pheochromocytoma / metabolism. Synaptophysin / biosynthesis
  • [MeSH-minor] Adrenocortical Adenoma / diagnosis. Adrenocortical Adenoma / metabolism. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / metabolism. Diagnosis, Differential. Female. Humans. Male

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  • (PMID = 16188147.001).
  • [ISSN] 0253-3766
  • [Journal-full-title] Zhonghua zhong liu za zhi [Chinese journal of oncology]
  • [ISO-abbreviation] Zhonghua Zhong Liu Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Chromogranin A; 0 / Synaptophysin
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15. Bjornsson B, Birgisson G, Oddsdottir M: Laparoscopic adrenalectomies: A nationwide single-surgeon experience. Surg Endosc; 2008 Mar;22(3):622-6
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  • Since then, all procedures for presumed benign lesions of the adrenals have been performed laparoscopically in a single center.
  • The preoperative diagnosis was documented, as well as the pathologic diagnosis, operative details, complications, and length of hospital stay.
  • RESULTS: In 49 operations, 53 adrenal glands were removed from 48 patients (37 women and 11 men).
  • The left adrenal was removed from 29 patients, the right adrenal from 14 patients, and both adrenals from 5 patients.
  • The most common indications and diagnoses included 17 nonsecreting tumors (12 adenomas, 3 hyperplasias, 1 complex adrenal cyst, and 1 hemangioma), 12 aldosteronomas (10 aldosteronomas and 2 nodular hyperplasias), and 10 pheochromocytomas (9 confirmed, 1 adrenal hyperplasia).
  • CONCLUSION: The results of laparoscopic adrenalectomies in Iceland for benign lesions of the adrenals are comparable with published results from large referral centers.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy / methods. Laparoscopy / statistics & numerical data. Pheochromocytoma / surgery

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  • (PMID = 18163169.001).
  • [ISSN] 1432-2218
  • [Journal-full-title] Surgical endoscopy
  • [ISO-abbreviation] Surg Endosc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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16. 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).
  • In normal adrenal medulla, Snail, Twist, and E-cadherin expression could not be detected.
  • [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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17. Pham TH, Moir C, Thompson GB, Zarroug AE, Hamner CE, Farley D, van Heerden J, Lteif AN, Young WF Jr: Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center. Pediatrics; 2006 Sep;118(3):1109-17
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  • [Title] Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center.
  • METHODS: A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients < 18 years of age with histologically confirmed pheochromocytoma or paraganglioma.
  • Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma.
  • Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease.
  • Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of > 6 cm.
  • All patients with benign disease appeared cured after resection.
  • CONCLUSIONS: The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of > 6 cm.
  • Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates.
  • Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Paraganglioma / surgery. Pheochromocytoma / surgery


18. Kuruba R, Gallagher SF: Current management of adrenal tumors. Curr Opin Oncol; 2008 Jan;20(1):34-46
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  • [Title] Current management of adrenal tumors.
  • PURPOSE OF REVIEW: Adrenal tumors evoke considerable interest and diagnostic challenges.
  • RECENT FINDINGS: Significant advances have been made in diagnostic imaging modalities for identifying malignancy risk in adrenal incidentalomas.
  • SUMMARY: Laparoscopic adrenalectomy has emerged as standard of care in the treatment of functional benign adenomas and nonfunctional tumors larger than 4 cm when adrenocortical carcinoma is not suspected.
  • International consensus conferences have attempted to standardize diagnostic and treatment approaches in the management of adrenal tumors; further research is necessary.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / drug therapy
  • [MeSH-minor] Adrenal Glands / pathology. Adrenal Glands / radiography. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / drug therapy. Adrenocortical Carcinoma / genetics. Chemotherapy, Adjuvant. Humans. Pheochromocytoma / diagnosis. Pheochromocytoma / drug therapy. Prognosis. Tomography, X-Ray Computed

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  • (PMID = 18043254.001).
  • [ISSN] 1040-8746
  • [Journal-full-title] Current opinion in oncology
  • [ISO-abbreviation] Curr Opin Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 68
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19. Nieman LK: Approach to the patient with an adrenal incidentaloma. J Clin Endocrinol Metab; 2010 Sep;95(9):4106-13
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  • [Title] Approach to the patient with an adrenal incidentaloma.
  • Unsuspected adrenal masses, or incidentalomas, are increasingly found with the widespread use of thoracic and abdominal imaging.
  • These masses may be hormonally active or nonfunctional and malignant or benign.
  • All patients should undergo biochemical testing for pheochromocytoma, either with plasma or urinary catecholamine measurements.
  • This is particularly important before surgical resection, which is routinely recommended for masses larger than 4 cm in diameter without a clear-cut diagnosis and for others with hormonal secretion or ominous imaging characteristics.
  • For masses that appear to be benign (<10 HU; washout, >50%), small (<3 cm), and completely nonfunctioning, imaging and biochemical reevaluation (pheochromocytoma and hypercortisolism only) at 1-2 yr (or more) is appropriate.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Algorithms. Carcinoma / diagnosis. Carcinoma / therapy. Incidental Findings
  • [MeSH-minor] Biopsy, Fine-Needle. Cushing Syndrome / complications. Cushing Syndrome / diagnosis. Diagnosis, Differential. Female. Humans. Middle Aged. Practice Guidelines as Topic

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  • (PMID = 20823463.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 50
  • [Other-IDs] NLM/ PMC2936073
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20. Blanes A, Sanchez-Carrillo JJ, Diaz-Cano SJ: Topographic molecular profile of pheochromocytomas: role of somatic down-regulation of mismatch repair. J Clin Endocrinol Metab; 2006 Mar;91(3):1150-8
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  • CONTEXT AND OBJECTIVE: Despite extensive molecular investigation of adrenal pheochromocytomas, no information is available on their molecular and mismatch repair (MMR) profiles by topographic compartments.
  • PATIENTS, OUTCOME MEASURES, AND INTERVENTIONS: Pheochromocytomas were classified as malignant (16 sporadic tumors with distant metastases), locally invasive (30 sporadic tumors showing retroperitoneal infiltration only), and benign (all remaining tumors).
  • More genetic abnormalities involving the peripheral compartment were revealed in 34 pheochromocytomas (23.8%): 12 of 16 malignant, 10 of 30 locally invasive, and 12 of 97 benign.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / pathology. Base Pair Mismatch / genetics. DNA Repair / genetics. Gene Expression Regulation, Neoplastic. Pheochromocytoma / genetics. Pheochromocytoma / pathology
  • [MeSH-minor] Adrenal Medulla / pathology. Base Sequence. DNA Primers. DNA, Neoplasm / genetics. DNA, Satellite / genetics. Genes, p53. Humans. Loss of Heterozygosity. Multiple Endocrine Neoplasia Type 2a / genetics. Polymorphism, Single Nucleotide. Retinoblastoma Protein / genetics


21. Pacak K, Eisenhofer G, Ahlman H, Bornstein SR, Gimenez-Roqueplo AP, Grossman AB, Kimura N, Mannelli M, McNicol AM, Tischler AS, International Symposium on Pheochromocytoma: Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endocrinol Metab; 2007 Feb;3(2):92-102
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  • [Title] Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005.
  • The First International Symposium on Pheochromocytoma, held in October 2005, included discussions about developments concerning these rare catecholamine-producing tumors.
  • Recommendations were made during the symposium for biochemical diagnosis, localization, genetics, and treatment.
  • Measurement of plasma or urinary fractionated metanephrines, the most accurate screening approach, was recommended as the first-line test for diagnosis; reference intervals should favor sensitivity over specificity.
  • Inadequate methods to distinguish malignant from benign tumors and a lack of effective treatments for malignancy are important problems requiring further resolution.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Paraganglioma / diagnosis. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 17237836.001).
  • [ISSN] 1745-8366
  • [Journal-full-title] Nature clinical practice. Endocrinology & metabolism
  • [ISO-abbreviation] Nat Clin Pract Endocrinol Metab
  • [Language] eng
  • [Publication-type] Consensus Development Conference; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0J45DE6B88 / Normetanephrine; 5001-33-2 / Metanephrine; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret
  • [Number-of-references] 83
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22. Feng C, Li HZ, Yan WG, Gao JG, Xu WF, Luo YF, Cao JL: [The significance of Ki-67 antigen expression in the distinction between benign and malignant pheochromocytomas]. Zhonghua Wai Ke Za Zhi; 2007 Dec 15;45(24):1697-700
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  • [Title] [The significance of Ki-67 antigen expression in the distinction between benign and malignant pheochromocytomas].
  • OBJECTIVES: To investigate the expression of Ki-67 antigen in benign and malignant pheochromocytomas, and to evaluate whether the expression of Ki-67 antigen could serve as a diagnostic marker for predicting the biological behaviour of these tumors.
  • METHODS: Ki-67 antigen were detected by immunohistochemical technique and image analysis in 57 cases of clinically documented benign and malignant pheochromocytomas were analyzed.
  • RESULTS: Ki-67 index was low in benign pheochromocytomas (average 0.98%), and high in malignant pheochromocytomas (average 3.78%).
  • There was statistically significant difference in expressions of Ki-67 antigen between benign and malignant pheochromocytomas.
  • The Ki-67 index of 2 cases in benign pheochromocytomas (5.1%, 2/39) and 10 cases in malignant pheochromocytomas (55.6%, 10/18) was higher than 3%.
  • The accuracy, sensitivity, specificity, positive predictive value and negative predictive value of Ki-67 index higher than 3% to diagnosis malignant pheochromocytomas was 82.5%, 55.6%, 94.9%, 83.3% and 82.2%.
  • Immunohistochemical assessment of Ki-67 antigen can be useful in the differential diagnosis of malignant from benign pheochromocytomas.
  • Ki-67 index (> 3%) is a useful marker for distinguishing benign from malignant tumors or for predicting the malignant potential of pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Biomarkers, Tumor / analysis. Ki-67 Antigen / analysis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Immunohistochemistry. Male. Middle Aged. Sensitivity and Specificity

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  • (PMID = 18476530.001).
  • [ISSN] 0529-5815
  • [Journal-full-title] Zhonghua wai ke za zhi [Chinese journal of surgery]
  • [ISO-abbreviation] Zhonghua Wai Ke Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen
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23. Frazzetta M, Tornambè A, Barrera T, Sciortino A, Lucania M, Frazzetta F, Sammartano A, Bonventre S, Vetri R: [Adrenal incidentaloma: case report and synthesis of indication and thresholds of surgery therapy]. G Chir; 2010 Nov-Dec;31(11-12):534-6
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  • [Title] [Adrenal incidentaloma: case report and synthesis of indication and thresholds of surgery therapy].
  • In 1982, the term "incidentaloma" was introduced to indicate lesions detected on adrenal regions, found unexpectedly.
  • Incidentalomas with diameters under 1 cm seem to not have a pathologic significance, and are considered like manifestation of the gland involution in advanced-age subjects, and can remain unseen for a long time.
  • The mass dimensions are a fundamental parameter used to distinguish benign lesions from malign ones (2, 3).
  • Most Authors suspect malignity in masses above 5-6 cm, while considering those with a diameter under 3 cm to be benign.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy. Diabetes Mellitus, Type 2 / complications. Incidental Findings. Pheochromocytoma / surgery


24. Stelow EB, Debol SM, Stanley MW, Mallery S, Lai R, Bardales RH: Sampling of the adrenal glands by endoscopic ultrasound-guided fine-needle aspiration. Diagn Cytopathol; 2005 Jul;33(1):26-30
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  • [Title] Sampling of the adrenal glands by endoscopic ultrasound-guided fine-needle aspiration.
  • Endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) has proven to be a valuable modality for the primary diagnosis and staging of gastrointestinal, and perigastrointestinal malignancy.
  • Aside from assessing thoracic and abdominal lymph nodes and the liver for metastases, EUS can assess and sample the adrenal glands, which are frequently involved by metastatic disease, but can also harbor benign primary neoplasms.
  • The cytology files at our institution were reviewed for all cases of EUS-guided FNA of the adrenal glands.
  • Results were compared with overall EUS-guided FNA performance and the performance of non-EUS-guided FNA of the adrenal.
  • Between 1/1/00 and 5/15/04 there were 24 cases of EUS-guided FNA of the adrenal gland from 22 different patients (13 men; 9 women) at our institution.
  • This represented 1.4% of overall EUS-guided FNA and 77% of adrenal gland FNA.
  • Almost all FNAs were of the left adrenal gland (23 of 24).
  • Final diagnoses were as follows: cortical tissue consistent with cortical adenoma (19), metastatic adenocarcinoma (3), pheochromocytoma (1), and adrenal cortical carcinoma (1).
  • EUS-guided FNA of the adrenal gland is primarily used in the staging of other malignancies when lesions of the left adrenal are recognized sonographically.
  • Diagnostic tissue is easily obtained, including material for cell block IHC, which allows definitive diagnosis in cases that present difficult differential diagnoses.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Glands / pathology. Biopsy, Fine-Needle / methods. Endosonography / methods
  • [MeSH-minor] Adenocarcinoma / diagnosis. Adenoma / diagnosis. Adrenocortical Carcinoma / diagnosis. Adult. Aged. Aged, 80 and over. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Pheochromocytoma / diagnosis. Sensitivity and Specificity

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  • [Copyright] 2005 Wiley-Liss, Inc
  • (PMID = 15945088.001).
  • [ISSN] 8755-1039
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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25. 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
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  • [Other-IDs] NLM/ NIHMS420223; NLM/ PMC3505859
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26. Kino M, Suzuki H, Naya Y, Komiya A, Imamoto T, Ichikawa T, Tatsuno I, Ishida H, Shindo T, Seki N: Comparative genomic hybridization reveals frequent losses of 1p and 3q in benign pheochromocytomas of Japanese patients. Cancer Genet Cytogenet; 2007 Jun;175(2):169-72
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  • [Title] Comparative genomic hybridization reveals frequent losses of 1p and 3q in benign pheochromocytomas of Japanese patients.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Chromosome Deletion. Chromosomes, Human, Pair 1 / genetics. Chromosomes, Human, Pair 3 / genetics. Pheochromocytoma / genetics

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  • (PMID = 17556075.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Comparative Study; Letter
  • [Publication-country] United States
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27. Sandgren J, Andersson R, Rada-Iglesias A, Enroth S, Akerstrom G, Dumanski JP, Komorowski J, Westin G, Wadelius C: Integrative epigenomic and genomic analysis of malignant pheochromocytoma. Exp Mol Med; 2010 Jul 31;42(7):484-502
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  • [Title] Integrative epigenomic and genomic analysis of malignant pheochromocytoma.
  • Pheochromocytoma is a rare endocrine tumor of the adrenal gland that mostly occurs sporadic with unknown epigenetic/genetic cause.
  • The majority of cases are benign.
  • Here we aimed to combine the genome-wide profiling of H3K4me3 and H3K27me3, obtained by the ChIP-chip methodology, and DNA copy number data with global gene expression examination in a malignant pheochromocytoma sample.
  • The integrated analysis of the tumor expression levels, in relation to normal adrenal medulla, indicated that either histone modifications or chromosomal alterations, or both, have great impact on the expression of a substantial fraction of the genes in the investigated sample.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / pathology. Epigenesis, Genetic. Genome, Human / genetics. Genomics. Pheochromocytoma / genetics. Pheochromocytoma / pathology

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  • (PMID = 20534969.001).
  • [ISSN] 2092-6413
  • [Journal-full-title] Experimental & molecular medicine
  • [ISO-abbreviation] Exp. Mol. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Histones; 0 / Tumor Suppressor Proteins; K3Z4F929H6 / Lysine
  • [Other-IDs] NLM/ PMC2912476
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28. 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.
  • Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites.
  • While medical management plays a significant role in the treatment of pheochromocytoma patients, surgical excision remains the only cure.
  • Improved medical management and surgical techniques and an increased understanding of hereditary disease have improved the outcome of pheochromocytoma patients with benign disease; however, the outcome of patients with malignant disease remains poor.
  • In this review, we discuss the presentation, diagnosis, management, and future directions in the management of this disease.
  • [MeSH-major] Adrenal Gland Neoplasms. Pheochromocytoma
  • [MeSH-minor] Adrenal Glands / surgery. Chemotherapy, Adjuvant. Genetic Predisposition to Disease. Humans. Neoplasm Metastasis / prevention & control. Radiotherapy, Adjuvant


29. Musholt TJ: [Diagnosis of and surgical therapy for pheochromocytoma and paraganglioma]. Zentralbl Chir; 2010 Jun;135(3):226-32
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  • [Title] [Diagnosis of and surgical therapy for pheochromocytoma and paraganglioma].
  • However, in the absence of metastasis, reliable differentiation between -benign and malignant growth is preoperatively and even histopathologically rarely possible.
  • Minimally invasive techniques using a transabdominal or retroperitoneal approach have become the gold standard for the resection of unifocal benign pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Paraganglioma / surgery. Pheochromocytoma / surgery
  • [MeSH-minor] Adrenal Glands / pathology. Adrenalectomy / methods. Diagnosis, Differential. Humans. Minimally Invasive Surgical Procedures. Multiple Endocrine Neoplasia / diagnosis. Multiple Endocrine Neoplasia / pathology. Multiple Endocrine Neoplasia / surgery

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  • [Copyright] Georg Thieme Verlag Stuttgart, New York.
  • (PMID = 20549585.001).
  • [ISSN] 1438-9592
  • [Journal-full-title] Zentralblatt für Chirurgie
  • [ISO-abbreviation] Zentralbl Chir
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
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30. 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
  • [MeSH-minor] Adolescent. Adrenal Gland Diseases / surgery. Adrenal Glands / pathology. Adult. Aged. Cysts / surgery. Female. Humans. Length of Stay. Male. Middle Aged. Postoperative Care. Time Factors. Tomography, X-Ray Computed

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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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31. 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
  • [MeSH-minor] Adolescent. Adrenal Medulla / metabolism. Adrenal Medulla / pathology. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Cyclin-Dependent Kinase Inhibitor p27 / metabolism. Female. Follow-Up Studies. Humans. In Situ Hybridization. Ki-67 Antigen / metabolism. Male. Middle Aged. Neoplasm Proteins / genetics. Neoplasm Proteins / metabolism

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

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  • (PMID = 15947101.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins; 0 / Proteome
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33. Havekes B, Romijn JA, Eisenhofer G, Adams K, Pacak K: Update on pediatric pheochromocytoma. Pediatr Nephrol; 2009 May;24(5):943-50
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  • [Title] Update on pediatric pheochromocytoma.
  • Pheochromocytomas are rare tumors in children arising from chromaffin cells of adrenal medullary or extra-adrenal paraganglionic tissue.
  • The formerly used guidelines for pheochromocytoma have been changed by recent discoveries, implementation of new approaches, and understanding of biochemistry, genetics, imaging, pathophysiology, and nomenclature of these tumors.
  • In children, pheochromocytomas are more frequently familial, extra-adrenal, bilateral, and multifocal than in adults.
  • Because of a highly variable clinical presentation, pheochromocytoma is often referred to as the great mimic.
  • Measurements of plasma or urinary fractionated metanephrines are recommended as first-line biochemical tests for diagnosis, with optimum diagnostic sensitivity to be preferred over specificity.
  • This article reviews recent advances in biochemistry, genetics, and imaging and outlines recommendations for improved evaluation and treatment of children with benign or malignant pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology

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  • (PMID = 18566838.001).
  • [ISSN] 1432-198X
  • [Journal-full-title] Pediatric nephrology (Berlin, Germany)
  • [ISO-abbreviation] Pediatr. Nephrol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 5001-33-2 / Metanephrine
  • [Number-of-references] 66
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34. 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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35. Nürnberg D: [Ultrasound of adrenal gland tumours and indications for fine needle biopsy (uFNB)]. Ultraschall Med; 2005 Dec;26(6):458-69
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  • [Title] [Ultrasound of adrenal gland tumours and indications for fine needle biopsy (uFNB)].
  • The normal adrenal glands can be detected by high-resolution-sonography in a high percentage of cases.
  • Sonography is also highly sensitive in the diagnosis of tumours of the adrenal glands.
  • Amongst the benign tumours, adenomas are the most frequent (up to 8%).
  • The adrenal glands are the fourth most common location of metastases.
  • Much less frequent are infiltrates of lymphomas in adrenal glands tumours (3.4%).
  • Incidentalomas are accidentally detected tumours of the adrenal glands without clinical symptoms.
  • The uFNB of the adrenal glands has a high sensitivity (90-95%).
  • Recommendations for this procedure in accidentally detected tumours of the adrenal glands: < 3 cm and hormonally inactive --> US-follow up, > 5 cm + suspected tumour --> surgery, 3-5 cm uFNB after laboratory diagnostics.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / ultrasonography. Biopsy, Fine-Needle
  • [MeSH-minor] Adrenal Glands / cytology. Adrenal Glands / pathology. Adrenal Glands / ultrastructure. Humans. Lymphoma / pathology. Lymphoma / ultrasonography. Pheochromocytoma / pathology. Pheochromocytoma / ultrasonography

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  • (PMID = 16453217.001).
  • [ISSN] 0172-4614
  • [Journal-full-title] Ultraschall in der Medizin (Stuttgart, Germany : 1980)
  • [ISO-abbreviation] Ultraschall Med
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 116
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36. González JI, Angoso M, García JA, Clemen JJ, Prado A, Angoso F: [Extra-adrenal pheochromocytoma: update of the most controversial features. Apropos of two cases]. Cir Esp; 2005 Mar;77(3):166-9
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  • [Title] [Extra-adrenal pheochromocytoma: update of the most controversial features. Apropos of two cases].
  • They are usually symptomatic and sporadic and are generally located in the adrenal medulla.
  • We present two cases of extra-adrenal nonfunctional pheochromocytomas located in the retroperitoneum.
  • The tumors were apparently benign and sporadic and were not associated with other neoplasms or hereditary diseases.
  • Several immunohistochemical techniques were performed to reach the definitive diagnosis.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / radiography. Pheochromocytoma / pathology. Pheochromocytoma / radiography

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  • (PMID = 16420910.001).
  • [ISSN] 0009-739X
  • [Journal-full-title] Cirugía española
  • [ISO-abbreviation] Cir Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 16
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37. Waldmann J, Langer P, Habbe N, Fendrich V, Ramaswamy A, Rothmund M, Bartsch DK, Slater EP: Mutations and polymorphisms in the SDHB, SDHD, VHL, and RET genes in sporadic and familial pheochromocytomas. Endocrine; 2009 Jun;35(3):347-55
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  • Malignant PC revealed multiple (>2) genetic alterations more frequently than benign PC (4/7 vs. 4/29, P = 0.03).
  • Interestingly intronic variants of the SDHB gene occur more frequently in malignant than in benign PC (3/7 vs. 2/29, P = 0.04).
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics. Proto-Oncogene Proteins c-ret / genetics. Succinate Dehydrogenase / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • (PMID = 19399650.001).
  • [ISSN] 1355-008X
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / SDHD protein, human; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase; EC 2.3.2.27 / Von Hippel-Lindau Tumor Suppressor Protein; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 6.3.2.- / VHL protein, human
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38. Gazymov MM, Filipov DS: [Adrenal tumors: topical diagnosis and surgical outcomes]. Urologiia; 2005 Sep-Oct;(5):9-12
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  • [Title] [Adrenal tumors: topical diagnosis and surgical outcomes].
  • A total of 128 adrenalectomies were made for catechol-producing tumor (n = 69), mineralocorticism (n = 27), primary and metastatic adrenal cancer (n = 20), other tumors (n = 12).
  • 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.
  • If cancer involves the upper segment of the kidney, the tumor often invades the adrenal.
  • Therefore, pre- and intraoperative search for adrenal tumor must be made.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Adrenalectomy. Hypertension / surgery

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

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  • (PMID = 17102081.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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40. Zografos GN, Markou A, Ageli C, Kopanakis N, Koutmos S, Kaltsas G, Piaditis G, Papastratis G: Laparoscopic surgery for adrenal tumors. A retrospective analysis. Hormones (Athens); 2006 Jan-Mar;5(1):52-6
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  • [Title] Laparoscopic surgery for adrenal tumors. A retrospective analysis.
  • OBJECTIVE: Laparoscopic adrenalectomy has rapidly replaced open adrenalectomy as the procedure of choice for benign adrenal tumors.
  • DESIGN: A retrospective analysis of patients operated on for adrenal tumors was conducted.
  • One patient with pheochromocytoma succumbed following pulmonary embolus.
  • In one patient with morbid obesity, Cushing's syndrome, and bilateral adrenal macronodular hyperplasia, the left laparoscopic adrenalectomy was complicated by a low output pancreatic fistula, conservatively treated.
  • CONCLUSIONS: Laparoscopic adrenalectomy should be the treatment of choice for all benign adrenal tumors.
  • Laparoscopic resection of large adrenal tumors necessitates experience in open surgery and advanced laparoscopic surgery.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Laparoscopy
  • [MeSH-minor] Adolescent. Adrenalectomy. Adult. Aged. Aged, 80 and over. Female. Humans. Length of Stay. Male. Middle Aged. Neoplasm Metastasis. Neoplasm Recurrence, Local. Pheochromocytoma / surgery. Retrospective Studies. Time Factors. Treatment Outcome

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  • (PMID = 16728385.001).
  • [ISSN] 1109-3099
  • [Journal-full-title] Hormones (Athens, Greece)
  • [ISO-abbreviation] Hormones (Athens)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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41. 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-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy. Laparoscopy
  • [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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42. 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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  • BACKGROUND AND PURPOSE: Laparoscopic adrenalectomy has become the gold standard in the surgical management of adrenal pathology.
  • 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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43. Chervin RA, Danilowicz K, Pitoia F, Gómez RM, Bruno OD: [A study of 34 cases of adrenal incidentaloma]. Medicina (B Aires); 2007;67(4):341-50
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  • [Title] [A study of 34 cases of adrenal incidentaloma].
  • Adrenal incidentaloma, a tumor discovered unexpectedly during imaging performed for non-adrenal related causes, has become a frequent clinical concern.
  • Although in most cases they are benign and hormonally nonfunctioning, it is important to appropriately identify those few cases of malignant or hyperfunctioning lesions of surgical resolution.
  • In the present retrospective study we analyzed 34 patients with adrenal incidentaloma.
  • Of these, 32% of the patients displayed hyperfunctioning pathologies that included subclinical Cushing's syndrome in four patients, probable primary hyperaldosteronism in two, late onset congenital adrenal hyperplasia in one, adrerocortical carcinoma in one and pheochromocytoma in three.
  • CT and/or MRI permitted the identification of adenomas and were crucial to decide on surgery in two patients with nonfunctioning pheochromocytomas and in a patient carrying a primitive adrenocortical carcinoma, a diagnosis also suggested by a mixed pattern of hypersecretion of androgens and cortisol.
  • Nonfunctioning tumors were mainly adenomas (87%) with individual cases of histoplasmosis, pseudocyst, idiopathic adrenal hyperplasia and mielolipoma.
  • [MeSH-major] Adenoma / diagnosis. Adrenal Gland Neoplasms / diagnosis. Cushing Syndrome / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Aged. Androgens / secretion. Child. Diagnosis, Differential. Female. Humans. Incidental Findings. Male. Middle Aged. Retrospective Studies. Treatment Outcome

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  • (PMID = 17891929.001).
  • [ISSN] 0025-7680
  • [Journal-full-title] Medicina
  • [ISO-abbreviation] Medicina (B Aires)
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Argentina
  • [Chemical-registry-number] 0 / Androgens
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44. Liao CH, Chueh SC, Lai MK, Hsiao PJ, Chen J: Laparoscopic adrenalectomy for potentially malignant adrenal tumors greater than 5 centimeters. J Clin Endocrinol Metab; 2006 Aug;91(8):3080-3
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  • [Title] Laparoscopic adrenalectomy for potentially malignant adrenal tumors greater than 5 centimeters.
  • We report a series of LA or hand-assisted LA for large (>5 cm) adrenal tumors.
  • Final pathology revealed eight malignant (four adrenocortical carcinomas and four metastatic carcinomas) and 31 benign tumors (14 cortical adenomas, eight pheochromocytomas, six myelolipomas, and three ganglioneuromas).
  • CONCLUSIONS: LA is a reasonable option for selected large adrenal tumors when complete resection is technically feasible and there is no evidence of local invasion.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy
  • [MeSH-minor] Adenoma / pathology. Adenoma / surgery. Adolescent. Adult. Aged. Child. Child, Preschool. Ganglioneuroma / pathology. Ganglioneuroma / surgery. Humans. Middle Aged. Myelolipoma / pathology. Myelolipoma / surgery. Neoplasm Metastasis. Pheochromocytoma / pathology. Pheochromocytoma / surgery. Prognosis. Survival Rate

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  • [CommentIn] Nat Clin Pract Endocrinol Metab. 2007 Mar;3(3):210-1 [17262068.001]
  • (PMID = 16720665.001).
  • [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
  • [Publication-country] United States
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45. 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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  • RESULTS: Of the 115 patients, 90 had adrenal tumours, 18 extra-adrenal and seven combined adrenal and extra-adrenal tumours.
  • Ten patients had malignant and 105 had benign phaeochromocytoma.
  • 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.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Hypertension / etiology. Pheochromocytoma / pathology


46. 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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  • AIMS: Phaeochromocytomas are rare but potentially life-threatening neuroendocrine tumours of the adrenal medulla or sympathetic nervous system ganglia.
  • There are no histological features which reliably differentiate benign from malignant phaeochromocytomas.
  • 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.
  • [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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47. Vilar L, Freitas Mda C, Canadas V, Albuquerque JL, Botelho CA, Egito CS, Arruda MJ, Moura e Silva L, Coelho CE, Casulari LA, Naves LA: Adrenal incidentalomas: diagnostic evaluation and long-term follow-up. Endocr Pract; 2008 Apr;14(3):269-78
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  • [Title] Adrenal incidentalomas: diagnostic evaluation and long-term follow-up.
  • OBJECTIVE: To evaluate the cause and the clinical and laboratory features of adrenal incidentalomas (AI) in 52 patients and to assess the evolution of nonsurgically treated lesions during long-term follow-up.
  • Carcinomas were the largest adrenal masses (mean diameter, 11.7 +/- 1.3 cm).
  • With the exception of 1 pheochromocytoma, 1 cyst, and 1 myelolipoma, all AI larger than 6 cm were carcinomas.
  • After 12 months of follow-up, however, a 45-year-old woman had adrenal mass enlargement from 3.2 cm to 4.4 cm; the excised lesion proved to be an adenoma.
  • Moreover, evidence of cortisol hypersecretion developed after 24 months of follow-up in a 30-year-old man with a 3.5-cm adenoma in the left adrenal gland.
  • CONCLUSION: Our findings demonstrate that most AI are nonfunctioning benign lesions and emphasize the need for long-term follow-up of patients with conservatively managed lesions, in light of the potential for evolution to hormonal hypersecretion or tumor growth.
  • [MeSH-major] Adenoma / diagnosis. Adrenal Gland Neoplasms / diagnosis. Incidental Findings
  • [MeSH-minor] Adrenal Cortex Neoplasms / blood. Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / surgery. Adrenocortical Adenoma / blood. Adrenocortical Adenoma / diagnosis. Adrenocortical Adenoma / surgery. Adult. Brazil. Female. Follow-Up Studies. Humans. Hydrocortisone / blood. Longitudinal Studies. Male. Middle Aged. Prognosis. Retrospective Studies

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  • [CommentIn] Endocr Pract. 2008 Apr;14(3):267-8 [18463031.001]
  • (PMID = 18463032.001).
  • [ISSN] 1934-2403
  • [Journal-full-title] Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
  • [ISO-abbreviation] Endocr Pract
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] WI4X0X7BPJ / Hydrocortisone
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48. Will OC, Hansmann A, Phillips RK, Palazzo FF, Meeran K, Marshall M, Clark SK: Adrenal incidentaloma in familial adenomatous polyposis: a long-term follow-up study and schema for management. Dis Colon Rectum; 2009 Sep;52(9):1637-44
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  • [Title] Adrenal incidentaloma in familial adenomatous polyposis: a long-term follow-up study and schema for management.
  • PURPOSE: Adrenal incidentaloma is often diagnosed in patients with familial adenomatous polyposis, because they frequently undergo abdominal imaging and have a raised incidence of adrenal incidentaloma.
  • This study investigates the natural history of adrenal incidentaloma in familial adenomatous polyposis, and suggests a schema for management.
  • METHODS: An original cohort of 14 familial adenomatous polyposis patients with adrenal incidentaloma, identified prospectively 12 years ago, was followed up clinically and radiologically.
  • For both cohorts, characteristics of patients (genotype, age at diagnosis, concomitant diagnoses) and incidentaloma (size, laterality, rate of growth, outcome) are described.
  • RESULTS: Overall, 3 of 30 patients underwent adrenalectomy; one patient had pheochromocytoma and another had an adenoma of borderline malignancy.
  • A further three lesions were radiologically suspicious for malignancy at the time of diagnosis; one was in a patient who was unfit for surgery but died of nonadrenal causes after nine years.
  • None of the lesions radiologically benign at diagnosis showed an aggressive course, but one patient required referral for surgery after 12 years because of a slow increase in size of the lesion.
  • CONCLUSIONS: Familial adenomatous polyposis-associated adrenal incidentaloma may warrant long-term follow-up.
  • [MeSH-major] Adenoma / diagnosis. Adenoma / therapy. Adenomatous Polyposis Coli / pathology. Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Incidental Findings


49. Agarwal A, Mehrotra PK, Jain M, Gupta SK, Mishra A, Chand G, Agarwal G, Verma AK, Mishra SK, Singh U: Size of the tumor and pheochromocytoma of the adrenal gland scaled score (PASS): can they predict malignancy? World J Surg; 2010 Dec;34(12):3022-8
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  • [Title] Size of the tumor and pheochromocytoma of the adrenal gland scaled score (PASS): can they predict malignancy?
  • The goal of this study was to find a correlation between the tumor size and malignant potential of PCC and determine whether the "Pheochromocytoma of the adrenal gland scaled score" (PASS) proposed by Thompson can be applied to predict malignancy.
  • RESULTS: Of the benign cases, none developed recurrence or metastasis.
  • Of the sporadic benign cases, 21 (41%) patients with tumor size > 6 cm had a PASS of >4, and none of them developed metastasis.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Glands / pathology. Neoplasm Staging. Pheochromocytoma / pathology

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  • (PMID = 20703467.001).
  • [ISSN] 1432-2323
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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50. Gonsior A, Pfeiffer H, Führer D, Liatsikos E, Schwalenberg T, Stolzenburg JU: [Adrenal tumors. Principles of diagnostics and operative treatment]. Urologe A; 2010 May;49(5):659-68; quiz 669-70
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  • [Title] [Adrenal tumors. Principles of diagnostics and operative treatment].
  • Adrenal masses are very heterogeneous and comprise benign or malignant tumors, unilateral or bilateral masses and variable endocrine activity.
  • Because of these attributes adrenal gland masses are a clinical challenge.
  • This article gives a summary of diagnostic steps and indications for adrenal surgery including perioperative management.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery
  • [MeSH-minor] Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / surgery. Adrenal Hyperplasia, Congenital / diagnosis. Adrenal Hyperplasia, Congenital / surgery. Adrenalectomy / instrumentation. Adrenalectomy / methods. Adrenogenital Syndrome / diagnosis. Adrenogenital Syndrome / surgery. Chemotherapy, Adjuvant. Combined Modality Therapy. Cushing Syndrome / diagnosis. Cushing Syndrome / surgery. Diagnosis, Differential. Diagnostic Imaging. Humans. Hyperaldosteronism / diagnosis. Hyperaldosteronism / surgery. Laparoscopes. Neoplasms, Multiple Primary / diagnosis. Neoplasms, Multiple Primary / drug therapy. Neoplasms, Multiple Primary / radiotherapy. Neoplasms, Multiple Primary / surgery. Pheochromocytoma / diagnosis. Pheochromocytoma / surgery. Radiotherapy, Adjuvant. Sensitivity and Specificity

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  • (PMID = 20449781.001).
  • [ISSN] 1433-0563
  • [Journal-full-title] Der Urologe. Ausg. A
  • [ISO-abbreviation] Urologe A
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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51. Fariña LA, Antón I, Fernández GC: [Large adrenal pseudocyst mimicking cystic pheochromocytoma: excision minimising laparoscopic access]. Actas Urol Esp; 2009 Sep;33(8):913-6
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  • [Title] [Large adrenal pseudocyst mimicking cystic pheochromocytoma: excision minimising laparoscopic access].
  • [Transliterated title] Seudoquiste adrenal grande que simula feocromocitoma quístico: exéresis minimizando el acceso laparoscópico.
  • INTRODUCTION: Adrenal cysts and pseudocysts are rare and usually benign and asymptomatic; they are discovered either accidentally or during examination for non-specific abdominal discomfort.
  • The differential diagnosis for adrenal cysts must consider cystic adrenal carcinoma and cystic pheochromocytoma, which are both extremely rare, but more severe and difficult to treat.
  • PATIENTS AND RESULTS: A 55 year-old woman reported right-side flank pain; imaging studies discovered a 10 cm cystic adrenal tumour, with radiological indications of cystic pheochromocytoma.
  • Pathological study showed benign pseudocyst.
  • CONCLUSIONS: As less invasive laparoscopic procedures become widespread, an increasing number of case reports and series of cases regarding adrenal cyst operations have recently been reported.
  • A review of the literature shows that the cysts are generally benign, but it is not always possible to identify cystic adrenal carcinoma or cystic pheochromocytoma from the radiology image.
  • [MeSH-major] Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / surgery. Adrenal Gland Neoplasms / diagnosis. Cysts / diagnosis. Cysts / surgery. Laparoscopy. Pheochromocytoma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Middle Aged


52. St Julien J, Ball D, Schulick R: Robot-assisted cortical-sparing adrenalectomy in a patient with Von Hippel-Lindau disease and bilateral pheochromocytomas separated by 9 years. J Laparoendosc Adv Surg Tech A; 2006 Oct;16(5):473-7
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  • Von Hippel-Lindau disease is a heritable syndrome that confers an increased risk of developing various benign and malignant tumors to those with a germline mutation of the tumor suppressor gene.
  • He was found to have a left pheochromocytoma which was successfully managed with open total adrenalectomy.
  • He presented again at age 18 with a second pheochromocytoma in the right adrenal gland.
  • [MeSH-major] Adrenal Gland Neoplasms / etiology. Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Neoplasms, Second Primary / etiology. Neoplasms, Second Primary / surgery. Pheochromocytoma / etiology. Pheochromocytoma / surgery. Robotics. von Hippel-Lindau Disease / complications
  • [MeSH-minor] Adolescent. Adrenal Cortex. Child. Humans. Male. Time Factors

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  • (PMID = 17004871.001).
  • [ISSN] 1557-9034
  • [Journal-full-title] Journal of laparoendoscopic & advanced surgical techniques. Part A
  • [ISO-abbreviation] J Laparoendosc Adv Surg Tech A
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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53. Imanaka M, Iida K, Takahashi K, Tsuji K, Nishizawa H, Fukuoka H, Takeno R, Takahashi Y, Okimura Y, Kaji H, Chihara K: The N131S mutation in the von Hippel-Lindau gene in a Japanese family with pheochromocytoma and hemangioblastomas. Endocr J; 2006 Dec;53(6):819-27
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  • [Title] The N131S mutation in the von Hippel-Lindau gene in a Japanese family with pheochromocytoma and hemangioblastomas.
  • von Hippel-Lindau (VHL) disease (VHLD) is a hereditary autosomal dominant syndrome that causes various benign and malignant tumors.
  • Here, we report a mutation in the VHL gene in a Japanese family with VHLD type 2A, characterized by pheochromocytoma (PHE), and hemangioblastomas (HAB) in both the retina and thoracic spinal cord but without renal cell carcinoma (RCC).
  • We also identified somatic loss of heterozygosity (LOH) at chromosome 3p25-26 in the adrenal tumor of the patient.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Cerebellar Neoplasms / genetics. Hemangioblastoma / genetics. Mutation. Pheochromocytoma / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • (PMID = 17001110.001).
  • [ISSN] 0918-8959
  • [Journal-full-title] Endocrine journal
  • [ISO-abbreviation] Endocr. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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54. Fassnacht M, Weismann D, Ebert S, Adam P, Zink M, Beuschlein F, Hahner S, Allolio B: AKT is highly phosphorylated in pheochromocytomas but not in benign adrenocortical tumors. J Clin Endocrinol Metab; 2005 Jul;90(7):4366-70
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  • [Title] AKT is highly phosphorylated in pheochromocytomas but not in benign adrenocortical tumors.
  • Immunohistochemistry for total AKT and pAKT was performed in pheochromocytomas (n = 8), ACC (n = 4), and normal adrenal glands (n = 2).
  • MAIN OUTCOME MEASURES: Determination of pAKT/total AKT ratio in adrenal tissues was the main outcome.
  • [MeSH-major] Adrenal Cortex Neoplasms / metabolism. Adrenal Gland Neoplasms / metabolism. Pheochromocytoma / metabolism. Protein-Serine-Threonine Kinases / metabolism. Proto-Oncogene Proteins / metabolism

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  • (PMID = 15855265.001).
  • [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 / Proto-Oncogene Proteins; 0 / Tumor Suppressor Proteins; EC 2.7.1.- / Phosphatidylinositol 3-Kinases; EC 2.7.11.1 / AKT1 protein, human; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt; EC 3.1.3.- / Phosphoric Monoester Hydrolases; EC 3.1.3.48 / PTEN protein, human; EC 3.1.3.67 / PTEN Phosphohydrolase
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55. Marazuela M, Domínguez-Gadea L, Larrañaga E, Rodríguez-Ramos R, López-Gallardo G, Rodríguez-Eyre JL, Gómez-Pan A: [The use of suprarenal scintigraphy in the differential diagnosis of suprarenal incidentaloma]. Rev Clin Esp; 2005 Jul;205(7):316-21
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  • [Title] [The use of suprarenal scintigraphy in the differential diagnosis of suprarenal incidentaloma].
  • OBJECTIVES: To assess the usefulness of adrenal scintigraphy for clinical evaluation of adrenal incidentalomas, and its relation with pathological diagnosis and follow-up.
  • PATIENTS AND METHODS: We have studied 46 patients with unilateral adrenal incidentaloma of size between 10 and 100 mm (average 30.5 +/- 19 mm).
  • The lesions were discovered with abdominal computerized tomography in the study of a primary tumor (22%) or in the evaluation of benign pathology (78%).
  • Adrenal scintigraphy assessed uptake in adrenal incidentaloma.
  • Hormonal study included urinary catecholamines, plasma cortisol after dexamethasone, adrenal androgens, and renin and aldosterone in hypertensive patients.
  • Adrenal incidentaloma was considered nonfunctional if the lesion did not modified its size nor showed analytical alterations along a follow-up higher than 8 months.
  • RESULTS: Of 46 adrenal lesions, seven didn't show uptake (three metastases, one cyst, one adrenal carcinoma, one pheochromocytoma, and one angiomyolipoma), 34 showed excessive uptake (29 nonfunctional adrenal nodules and 5 hyperfunctional adrenal nodules), and five had normal uptake (nonfunctional adrenal nodules).
  • Adrenal scintigraphy was compatible in all cases with cytological study or the response to chemotherapy.
  • CONCLUSIONS: Detection of a lesion with no uptake in adrenal scintigraphy forces to carry out complementary explorations in order to rule out malignant pathology.
  • A lesion with excessive uptake is indicative of a benign process and should be assessed with hormonal determinations.
  • [MeSH-major] Adrenal Gland Neoplasms / radionuclide imaging

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  • (PMID = 16029757.001).
  • [ISSN] 0014-2565
  • [Journal-full-title] Revista clínica española
  • [ISO-abbreviation] Rev Clin Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
  • [Chemical-registry-number] WI4X0X7BPJ / Hydrocortisone
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56. 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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57. Han XN, Chen B, Ye XD, Wang J, Liu GH: [Clinical manifestation and multiphasic spiral CT scanning features of abdominal pheochromocytoma: report of 70 cases]. Zhonghua Zhong Liu Za Zhi; 2009 Feb;31(2):139-42
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  • [Title] [Clinical manifestation and multiphasic spiral CT scanning features of abdominal pheochromocytoma: report of 70 cases].
  • OBJECTIVE: The purpose of this study was to assess the imaging characteristics of abdominal pheochromocytoma in multiphasic spiral CT scanning, and to determine whether these image characteristics can aid in differentiating pheochromocytoma from other types of tumors or not.
  • RESULTS: Among the 70 patients, there were 41 patients with endocrine symptoms related to pheochromocytoma, 15 had a latent pheochromocytoma and the remaining 14 cases presented with a non-functioning pheochromocytoma.
  • Sixty cases had a tumor located in the adrenal gland, while 8 in retroperitoneal space, and 2 cases had both intraadrenal and ectopic lesions simultaneously.
  • Sixty patients had a benign pheochromocytoma, the other 10 had a malignant one or relapse after operation.
  • However, when a small tumor has moderate blood supply, it should be differentiated with an adrenal adenoma; when a big one has moderate blood supply, it should be differentiated with other malignant tumors.
  • Furthermore, a part of malignant pheochromocytomas is really difficult to be differentiated from some benign lesions by spiral CT images alone.
  • [MeSH-major] Adrenal Gland Neoplasms / radiography. Pheochromocytoma / radiography. Retroperitoneal Neoplasms / radiography. Tomography, Spiral Computed / methods
  • [MeSH-minor] Adenoma / blood supply. Adenoma / radiography. Adenoma / surgery. Adolescent. Adult. Aged. Aged, 80 and over. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Radiographic Image Enhancement. Retrospective Studies. Young Adult

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

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  • (PMID = 16882497.001).
  • [ISSN] 1055-3207
  • [Journal-full-title] Surgical oncology clinics of North America
  • [ISO-abbreviation] Surg. Oncol. Clin. N. Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Number-of-references] 79
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59. 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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  • [Title] Laparoscopic surgery for malignant adrenal tumors.
  • Advances in imaging have improved early detection of primary and metastatic adrenal tumors.
  • The laparoscopic approach, the gold standard for benign adrenal diseases, is controversial for malignant adrenal tumors.
  • A prospective randomized study of the role of laparoscopic surgery in adrenal cancer is not feasible because of the rarity of the disease.
  • A review of the literature demonstrates the safety and efficacy of laparoscopic adrenalectomy for solitary adrenal tumors.
  • In primary adrenal malignancies, the laparoscopic approach should be considered cautiously, only when it can achieve complete tumor resection with an intact adrenal capsule.

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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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60. Michalakis K, Ilias I: Medical management of adrenal disease: a narrative review. Endocr Regul; 2009 Jul;43(3):127-35
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  • [Title] Medical management of adrenal disease: a narrative review.
  • Adrenal diseases comprise for a variety of medical endocrine issues, ranging from partial or complete gland insufficiency, to several kinds of adrenal hyperfunction, either of congenital or neoplastic etiology.
  • Patients with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency are treated with glucocorticoids to control androgen excess.
  • Most benign neoplastic adrenal diseases that cause hyperfunction of the gland are surgically treated, however this may not be always feasible or effective.
  • [MeSH-major] Adrenal Gland Diseases / drug therapy
  • [MeSH-minor] Adrenal Gland Neoplasms / drug therapy. Adrenal Gland Neoplasms / surgery. Adrenal Glands / surgery. Adrenal Hyperplasia, Congenital / drug therapy. Adrenal Insufficiency / drug therapy. Adrenocortical Carcinoma / drug therapy. Clinical Trials as Topic. Cushing Syndrome / drug therapy. Humans. Hyperaldosteronism / drug therapy. Pheochromocytoma / drug therapy. Pheochromocytoma / surgery

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  • (PMID = 19817507.001).
  • [ISSN] 1210-0668
  • [Journal-full-title] Endocrine regulations
  • [ISO-abbreviation] Endocr Regul
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Slovakia
  • [Number-of-references] 51
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61. 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.
  • METHODS: Different from the other cardiac pheochromocytomas, its symptoms and signs are so nonspecific that it is easy to make a misdiagnosis or missed diagnosis.
  • One patient with nonfunctioning benign cardiac pheochromocytoma was treated surgically and relevant cases data were collected.
  • Clinical features, diagnosis and treatment of pheochromocytoma were discussed.
  • The prognosis for patients of benign cardiac pheochromocytomas was excellent, but for those of malignant ones was very poor.
  • CONCLUSION: Reviewing the few reported cases, most nonfunctioning benign cardiac pheochromocytomas can be cured completely by operation with good prognosis.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Heart Neoplasms / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Echocardiography. Humans. Male. Radiography, Thoracic

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  • (PMID = 18563442.001).
  • [ISSN] 0171-5216
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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62. Wängberg B, Muth A, Khorram-Manesh A, Jansson S, Nilsson O, Forssell-Aronsson E, Tisell L, Ahlman H: Malignant pheochromocytoma in a population-based study: survival and clinical results. Ann N Y Acad Sci; 2006 Aug;1073:512-6
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  • [Title] Malignant pheochromocytoma in a population-based study: survival and clinical results.
  • One hundred fifty-four consecutive patients with pheochromocytoma (PC, n=137) or paraganglioma (PG, n=17) were treated at our unit.
  • Within 4 years two patients died of tumor progression. (2) Locally advanced disease (n=4), all resected for cure. (3) Malignancy disclosed during follow-up after adrenalectomy with "benign" histopathology (n=4).
  • Active surgical treatment and phenoxybenzamine resulted in low tumor-related mortality in groups 2 and 3; five patients died 8-30 years after diagnosis, four of PC/PG (three from group 2 and one from group 3) and one of other causes.
  • To diagnose PC/PG early, screening of adrenal incidentalomas has been suggested.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology


63. 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
  • [MeSH-minor] Diagnosis, Differential. Genome, Human. Humans

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  • (PMID = 18651863.001).
  • [ISSN] 1442-2042
  • [Journal-full-title] International journal of urology : official journal of the Japanese Urological Association
  • [ISO-abbreviation] Int. J. Urol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Australia
  • [Number-of-references] 58
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64. 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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  • Concomitantly, a chest-abdominal computed tomography was performed and revealed a 22 mm right adrenal incidentaloma.
  • 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.
  • The diagnosis of Men2a at the age of 80 years and the absence of lymph node metastasis of the multiple MTC in a carrier of C634G mutation were unusual and argued for the possible role of genetic modifier(s) in this MEN 2a patient.
  • [MeSH-minor] Adenoma / radionuclide imaging. Adrenalectomy. Aged, 80 and over. Calcitonin / blood. Catecholamines / urine. Goiter, Nodular / ultrasonography. Humans. Male. Parathyroid Neoplasms / radionuclide imaging. Parathyroidectomy. Radiopharmaceuticals. Technetium Tc 99m Sestamibi. Thyroid Gland / radionuclide imaging. Thyrotropin / blood

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

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  • (PMID = 19451490.001).
  • [ISSN] 1538-3644
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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66. Amar L, Peyrard S, Rossignol P, Zinzindohoue F, Gimenez-Roqueplo AP, Plouin PF: Changes in urinary total metanephrine excretion in recurrent and malignant pheochromocytomas and secreting paragangliomas. Ann N Y Acad Sci; 2006 Aug;1073:383-91
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  • We quantified urinary excretion of total metanephrines (metanephrine plus normetanephrine) (UETM) in 261 patients with pheochromocytoma (PH) or secreting paraganglioma, before primary tumor resection.
  • The tumor was considered benign in 242 patients and malignant in 19 patients.
  • Patients with malignant tumors had higher UETM and tumor diameters, lower plasma epinephrine concentrations, and were more likely to have secreting paragangliomas than patients with benign tumors.
  • In the 215 patients with a single-benign primary tumor, preoperative UETM and tumor diameter were significantly correlated.
  • Urinary metanephrine excretion is a marker of tumor activity and a surrogate of tumor burden in patients with pheochromocytoma or secreting paraganglioma.
  • [MeSH-major] Adrenal Gland Neoplasms / urine. Metanephrine / urine. Paraganglioma / urine. Pheochromocytoma / urine

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  • (PMID = 17102107.001).
  • [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] 5001-33-2 / Metanephrine
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67. De Toma G, Letizia C, Cavallaro G, Polistena A, Cotesta D, Petramala L, Porciello R, Giustini S, Calvieri S: Adrenal pheochromocytoma and jejunal neurofibroma in type 1 neurofibromatosis: report of a case. G Chir; 2007 May;28(5):199-202
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  • [Title] Adrenal pheochromocytoma and jejunal neurofibroma in type 1 neurofibromatosis: report of a case.
  • Diagnosis, despite extreme clinical variability, is defined by established diagnostic criteria.
  • CASE REPORT: A case of a patient affected by NF1, with hypertension due to adrenal pheochromocytoma and with jejunal neurofibroma, is reported.
  • DISCUSSION AND CONCLUSIONS: Variability in clinical presentation of NF1 with possible manifestation of severe systemic benign and malignant diseases requires strict follow-up and specific screening of extra-cutaneous lesions.
  • [MeSH-major] Adrenal Gland Neoplasms. Jejunal Neoplasms. Neoplasms, Multiple Primary. Neurofibromatosis 1. Pheochromocytoma


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


69. 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.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy

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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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70. Suh I, Shibru D, Eisenhofer G, Pacak K, Duh QY, Clark OH, Kebebew E: Candidate genes associated with malignant pheochromocytomas by genome-wide expression profiling. Ann Surg; 2009 Dec;250(6):983-90
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: To improve our understanding of the molecular mechanisms involved in malignant pheochromocytoma by examining differences in the gene expression profile between benign and malignant tumors.
  • BACKGROUND: The molecular events involved in the malignant transformation of pheochromocytoma are poorly understood.
  • There are also no reliable and uniformly accepted histopathologic criteria to distinguish benign from malignant pheochromocytoma.
  • METHODS: We performed genome-wide expression profiling of 58 pheochromocytomas (29 benign and sporadic, 16 benign and hereditary, 13 malignant) with technical and biologic replication.
  • Supervised cluster analysis showed almost completely separate clustering between benign and malignant tumors.
  • Ten differentially expressed genes had high diagnostic accuracy, and 5 of these genes (CFC1, FAM62B, HOMER1, LRRN3, TBX3, ADAMTS) in combination had an area under the receiver operating characteristic (ROC) curve of 0.96 for distinguishing benign versus malignant tumors.
  • CONCLUSIONS: Differentially expressed genes between benign and malignant pheochromocytomas distinguish between these tumors with high diagnostic accuracy.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Regulation, Neoplastic. Genes, Neoplasm / genetics. Genetic Association Studies / methods. Pheochromocytoma / genetics. RNA, Neoplasm / genetics

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  • (PMID = 19661783.001).
  • [ISSN] 1528-1140
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / RNA, Neoplasm
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71. Shen SJ, Cheng HM, Chiu AW, Chou CW, Chen JY: Perioperative hypertensive crisis in clinically silent pheochromocytomas: report of four cases. Chang Gung Med J; 2005 Jan;28(1):44-50
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  • Improvements in imaging have resulted in an increase in incidentally discovered adrenal tumors.
  • The adrenal incidentalomas have been identified in at least 2%-3% of patients receiving abdominal computed tomography (CT), and pheochromocytomas are reported to occur in about 5.1%-23.0% of those patients.
  • Herein we present 4 cases of pheochromocytomas as unforeseen adrenal masses on abdominal images.
  • The characteristics of these tumors were that they (1) were noted to have no particular symptoms, (2) were confirmed biochemically and pathologically with immunophenotypic staining, (3) were benign in nature, and (4) produced apparent blood-pressure fluctuations during the operation.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Hypertension / etiology. Pheochromocytoma / complications


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

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  • (PMID = 15642205.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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73. Reisch N, Peczkowska M, Januszewicz A, Neumann HP: Pheochromocytoma: presentation, diagnosis and treatment. J Hypertens; 2006 Dec;24(12):2331-9
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  • [Title] Pheochromocytoma: presentation, diagnosis and treatment.
  • Pheochromocytomas are rare, mostly benign catecholamine-producing tumors of chromaffin cells of the adrenal medulla or of a paraganglion.
  • The diagnosis of pheochromocytomas depends mainly upon the demonstration of catecholamine excess by 24-h urinary catecholamines and metanephrines or plasma metanephrines.
  • They are localized by a computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen; complementary 123I-metaiodobenzylguanidine scintigraphy and 18F-dihydroxyphenylalanine-positron emission tomography are available.
  • Laparoscopic and adrenal sparing surgical intervention following preoperative alpha-blockade is the treatment of choice and usually curative.
  • This review provides an update on identification and management of pheochromocytomas, emphasizing current developments in diagnosis, including genetic testing, pathophysiology and treatment of pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy


74. Meyer-Rochow GY, Jackson NE, Conaglen JV, Whittle DE, Kunnimalaiyaan M, Chen H, Westin G, Sandgren J, Stålberg P, Khanafshar E, Shibru D, Duh QY, Clark OH, Kebebew E, Gill AJ, Clifton-Bligh R, Robinson BG, Benn DE, Sidhu SB: MicroRNA profiling of benign and malignant pheochromocytomas identifies novel diagnostic and therapeutic targets. Endocr Relat Cancer; 2010 Sep;17(3):835-46
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  • [Title] MicroRNA profiling of benign and malignant pheochromocytomas identifies novel diagnostic and therapeutic targets.
  • There is increasing evidence to suggest that miRNAs could be useful in cancer diagnosis, prognosis, and therapy.
  • We performed miRNA microarray expression profiling on a cohort of 12 benign and 12 malignant pheochromocytomas and identified a number of differentially expressed miRNAs.
  • These results were validated in a separate cohort of ten benign and ten malignant samples using real-time quantitative RT-PCR; benign samples had a minimum follow-up of at least 2 years.
  • It was found that IGF2 as well as its intronic miR-483-5p was over-expressed, while miR-15a and miR-16 were under-expressed in malignant tumours compared with benign tumours.
  • These miRNAs were found to be diagnostic and prognostic markers for malignant pheochromocytoma.
  • The functional role of miR-15a and miR-16 was investigated in vitro in the rat PC12 pheochromocytoma cell line, and these miRNAs were found to regulate cell proliferation via their effect on cyclin D1 and apoptosis.
  • These data indicate that miRNAs play a pivotal role in the biology of malignant pheochromocytoma, and represent an important class of diagnostic and prognostic biomarkers and therapeutic targets warranting further investigation.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Biomarkers, Tumor / genetics. Gene Expression Profiling. Gene Expression Regulation, Neoplastic. MicroRNAs / physiology. Pheochromocytoma / genetics
  • [MeSH-minor] Adrenal Glands / metabolism. Adrenal Glands / pathology. Animals. Apoptosis. Blotting, Western. Cell Cycle. Cohort Studies. Follow-Up Studies. Humans. Oligonucleotide Array Sequence Analysis. Prognosis. RNA, Messenger / genetics. Rats. Reverse Transcriptase Polymerase Chain Reaction. Survival Rate. Tumor Cells, Cultured


75. Legmann P: [Adrenal incidentaloma: management approaches: CT - MRI]. J Radiol; 2009 Mar;90(3 Pt 2):426-43
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  • [Title] [Adrenal incidentaloma: management approaches: CT - MRI].
  • The adrenal gland may be affected by several pathologies, and the detection of an adrenal nodule may occur during the work-up of a biological abnormality, oncologic work-up, or be incidental.
  • Cross-sectional imaging has had a great impact on the diagnostic work-up of adrenal nodules.
  • CT, without and with intravenous contrast, is the first line imaging study for evaluation of adrenal nodules.
  • A spontaneous density below 10 HU confirms the benign nature of a nodule.
  • When lesions cannot be characterized, postcontrast CT or MR imaging, including in and out of phase imaging, may allow diagnosis.
  • [MeSH-major] Adenoma / diagnosis. Adenoma / radiography. Adrenal Gland Diseases / diagnosis. Adrenal Gland Diseases / radiography. Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / radiography. Cushing Syndrome / diagnosis. Cushing Syndrome / radiography. Incidental Findings. Magnetic Resonance Imaging / methods. Sarcoma, Ewing / diagnosis. Sarcoma, Ewing / radiography. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / radiography. Adrenal Glands / pathology. Adult. Algorithms. Biopsy. Cysts / diagnosis. Cysts / radiography. Diagnosis, Differential. Female. Hemangioma / diagnosis. Hemangioma / radiography. Hematoma / diagnosis. Hematoma / radiography. Humans. Lymphoma / diagnosis. Lymphoma / radiography. Pheochromocytoma / diagnosis. Pheochromocytoma / radiography. Sarcoma / diagnosis. Sarcoma / radiography


76. 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


77. Timmers HJ, Gimenez-Roqueplo AP, Mannelli M, Pacak K: Clinical aspects of SDHx-related pheochromocytoma and paraganglioma. Endocr Relat Cancer; 2009 Jun;16(2):391-400
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  • [Title] Clinical aspects of SDHx-related pheochromocytoma and paraganglioma.
  • Paragangliomas (PGLs) derive from either sympathetic chromaffin tissue in adrenal and extra-adrenal abdominal or thoracic locations, or from parasympathetic tissue of the head and neck.
  • SDHB mutations mainly predispose to extra-adrenal, and to a lesser extent, adrenal PGLs, with a high malignant potential, but also head and neck paragangliomas (HNPGL).
  • SDHD mutations are typically associated with multifocal HNPGL and usually benign adrenal and extra-adrenal PGLs.
  • The identification of SDHx mutations in patients with PGL is warranted for a tailor-made approach to the biochemical diagnosis, imaging, treatment, follow-up, and family screening.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics. Succinate Dehydrogenase / genetics

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  • (PMID = 19190077.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 HD008735-08
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] EC 1.3.99.1 / Succinate Dehydrogenase
  • [Number-of-references] 54
  • [Other-IDs] NLM/ NIHMS750209; NLM/ PMC4711350
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78. 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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  • INTRODUCTION: In the last few years, laparoscopic adrenalectomy has become widely used in the management of adrenal disease.
  • RESULTS: Surgery was indicated for Cushing's syndrome in 46% of the patients, aldosteronoma in 25%, incidentaloma in 21% and pheochromocytoma in 8%.
  • The mean size of the gland was 4 cm.
  • 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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79. 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.
  • A total of 79 adrenal glands were resected, 44 right sided and 35 left sided.
  • 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).
  • While long-term results after LA for cortical carcinomas were poor and LA is not recommended in such cases, long-term results after LA for adrenal metastases were encouraging.
  • [MeSH-major] Adrenal Gland Diseases / surgery. Adrenalectomy / methods. Laparoscopy / methods

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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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80. 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.
  • RIZ1 mRNA appeared to be significantly under-expressed in the tumour samples compared to normal adrenal controls (mean 0.6 vs. 1.0, p<0.001).
  • [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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81. 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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  • [Title] Differential diagnosis of incidentally detected adrenal masses revealed on routine abdominal CT.
  • PURPOSE: The aim of this study was to compare CT findings of adrenal incidentalomas with a definitive histological diagnosis in order to establish CT features characteristic for individual types of lesions.
  • PATIENTS AND METHODS: The retrospective study comprised of patients with adrenal lesions detected on abdominal CT.
  • The study consisted of 62 adrenal expansions found in 55 patients (in seven patients bilateral lesions were found).
  • 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.
  • [MeSH-major] Adrenal Gland Neoplasms / radiography. Radiographic Image Interpretation, Computer-Assisted / methods. Radiography, Abdominal / methods. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Adult. Aged. Diagnosis, Differential. Female. Humans. Incidental Findings. Male. Middle Aged. Radiographic Image Enhancement / methods. Reproducibility of Results. Sensitivity and Specificity. Young Adult

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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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82. Korpershoek E, Loonen AJ, Corvers S, van Nederveen FH, Jonkers J, Ma X, Ziel-van der Made A, Korsten H, Trapman J, Dinjens WN, de Krijger RR: Conditional Pten knock-out mice: a model for metastatic phaeochromocytoma. J Pathol; 2009 Mar;217(4):597-604
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  • Phaeochromocytomas (PCCs) are neuro-endocrine tumours of the adrenal medulla that are usually benign, but approximately 10% of patients develop metastases.
  • Here we describe adrenal tumours generated in a Pten conditional knock-out (KO) mouse model.
  • Thirty-two of 41 (78%) male Psa-Cre;Pten-loxP/loxP mice presented adrenal tumours that were shown to be PCC by histology and by immunohistochemical staining for enzymes in the catecholamine biosynthetic pathway.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Disease Models, Animal. Lung Neoplasms / secondary. Neoplasm Proteins / genetics. PTEN Phosphohydrolase / genetics. Pheochromocytoma / secondary


83. Bissada NK, Safwat AS, Seyam RM, Al Sobhi S, Hanash KA, Jackson RJ, Sakati N, Bissada MA: Pheochromocytoma in children and adolescents: a clinical spectrum. J Pediatr Surg; 2008 Mar;43(3):540-3
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  • [Title] Pheochromocytoma in children and adolescents: a clinical spectrum.
  • PURPOSE: The purpose of the study was to identify the spectrum of disease characteristics of pheochromocytoma in children.
  • METHODS: Records of 21 consecutive children diagnosed with pheochromocytoma were reviewed.
  • Patients included 17 with adrenal and 4 with extra-adrenal tumors including 1 in the urinary bladder.
  • Seventeen had sporadic and 4 had familial pheochromocytoma.
  • Associated manifestations were the predominant features in 1 of the 4 patients with familial pheochromocytoma.
  • The patient with bladder pheochromocytoma presented with gross hematuria.
  • Two patients (1 sporadic and 1 familial) had malignant pheochromocytoma.
  • One patient with benign pheochromocytoma had multiple recurrences in chromaffin-containing sites.
  • One patient with malignant pheochromocytoma died of the disease, and 1 with recurrent pheochromocytoma had neurologic consequences.
  • CONCLUSIONS: Pheochromocytoma in children has unique characteristics.
  • To our knowledge, this series is one of the largest reports of adrenal pheochromocytoma in children.
  • It also reflects the spectrum of pheochromocytoma in this age group.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Pheochromocytoma / diagnosis. Pheochromocytoma / surgery


84. McHugh K: Renal and adrenal tumours in children. Cancer Imaging; 2007;7:41-51
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  • [Title] Renal and adrenal tumours in children.
  • The differential diagnosis of renal and supra-renal masses firstly depends on the age of the child.
  • Benign renal masses predominate in early infancy but beyond the first year of life Wilms' tumour is the most common renal malignancy, until adolescence when renal cell carcinoma has similar or increased frequency as children get older.
  • Adrenal adenomas and carcinomas also occur in childhood; these tumours are indistinguishable on imaging but criteria for the diagnosis of adrenal carcinoma include size larger than 5 cm, a tendency to invade the inferior vena cava and to metastasise.
  • The most topical dilemmas in the radiological assessment of renal and adrenal tumours are presented.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Diagnostic Imaging / methods. Kidney Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Age of Onset. Carcinoma, Renal Cell / diagnosis. Carcinoma, Renal Cell / epidemiology. Child. Child, Preschool. Diagnosis, Differential. Ganglioneuroblastoma / diagnosis. Ganglioneuroblastoma / epidemiology. Ganglioneuroblastoma / pathology. Humans. Infant. Infant, Newborn. Lung Neoplasms / radiography. Lung Neoplasms / secondary. Magnetic Resonance Imaging. Mass Screening. Neoplasm Staging. Neuroblastoma / diagnosis. Neuroblastoma / epidemiology. Neuroblastoma / pathology. Neuroblastoma / secondary. Neuroblastoma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / epidemiology. Positron-Emission Tomography. Tomography, X-Ray Computed. Wilms Tumor / diagnosis. Wilms Tumor / epidemiology. Wilms Tumor / pathology. Wilms Tumor / therapy

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  • (PMID = 17339140.001).
  • [ISSN] 1470-7330
  • [Journal-full-title] Cancer imaging : the official publication of the International Cancer Imaging Society
  • [ISO-abbreviation] Cancer Imaging
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 19
  • [Other-IDs] NLM/ PMC1828369
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85. Guillemot J, Barbier L, Thouennon E, Vallet-Erdtmann V, Montero-Hadjadje M, Lefebvre H, Klein M, Muresan M, Plouin PF, Seidah N, Vaudry H, Anouar Y, Yon L: Expression and processing of the neuroendocrine protein secretogranin II in benign and malignant pheochromocytomas. Ann N Y Acad Sci; 2006 Aug;1073:527-32
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  • [Title] Expression and processing of the neuroendocrine protein secretogranin II in benign and malignant pheochromocytomas.
  • The aim of the present study was to compare the expression levels of secretogranin II (SgII), prohormone convertases (PC)1 and PC2, and the proteolytic processing of SgII in benign versus malignant pheochromocytomas.
  • Quantitative (Q)-PCR experiments indicated that SgII, PC1, and PC2 mRNAs were overexpressed in pheochromocytoma compared to non-tumoral chromaffin cells (P<0.001) and in benign compared to malignant tumors (P<0.01).
  • Western blot analysis using a human SgII antiserum revealed the occurrence of a 97-kDa band corresponding to the expected size of SgII, with significantly higher quantities in benign than in malignant tumors (P<0.05).
  • Using antisera directed against sequential regions of SgII (N-terminal, secretoneurin [SN], EM66, internal, and C-terminal sequences), we observed distinct processing profiles between benign and malignant pheochromocytomas.
  • RIA measurement showed that EM66 median values between benign and malignant chromaffin cell tumors were significantly different (128.5 vs. 6.3 ng/mg protein, respectively; P<0.001).
  • Taken together, these results indicate that, in pheochromocytoma, malignancy is associated with reduced PC1, PC2, and SgII mRNA expression and decreased levels of processing products of SgII, in line with the low concentrations of EM66 that occur in malignant tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Gene Expression Profiling. Pheochromocytoma / diagnosis

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  • (PMID = 17102121.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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86. Singh PK, Buch HN: Adrenal incidentaloma: evaluation and management. J Clin Pathol; 2008 Nov;61(11):1168-73
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  • [Title] Adrenal incidentaloma: evaluation and management.
  • Adrenal incidentalomas are adrenal masses discovered incidental to imaging studies performed for reasons unrelated to adrenal pathology.
  • Although most adrenal incidentalomas are non-functioning benign adenomas, their increasing prevalence presents diagnostic and therapeutic challenges.
  • The assessment of adrenal incidentalomas is aimed at deciding whether or not the tumour should be surgically removed.
  • Biochemical screening for tumour hypersecretion is mandatory in all adrenal incidentalomas, since hormone secreting tumours may be clinically silent.
  • The diagnosis of phaeochromocytoma is of paramount importance because of its life-threatening complications.
  • Non-functioning adrenal incidentalomas need assessment for risk of malignancy, and this is based on the size of the tumour and its imaging characteristics.
  • The duration and frequency of reassessment remains unclear, as the natural history of adrenal incidentalomas has yet to be clearly defined, and there is a lack of controlled studies comparing surgical intervention with observation.
  • However, the possibility of acquiring autonomous hypersecretion or conversion to malignancy in an incidentaloma diagnosed to be a benign non-functioning lesion is very low, and most patients may be safely discharged after an initial follow-up period of 2 years.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis
  • [MeSH-minor] Adenoma / diagnosis. Diagnosis, Differential. Humans. Incidental Findings. Long-Term Care / methods. Pheochromocytoma / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 18955573.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 117
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87. Nap RR, Meinardi JR, van den Berg G, Dullaart RP, de Vries J, Wolffenbuttel BH: [Long-term follow-up is indicated after surgery for a phaeochromocytoma]. Ned Tijdschr Geneeskd; 2006 May 13;150(19):1045-9
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  • Studies report recurrence ofphaeochromocytoma in 8-20% of cases, and such a recurrence may be either benign or malignant.
  • Factors predictive for recurrence are older age, a malignant primary tumour, tumour weight above 60 g, extra-adrenal origin of the tumour and peroperative tumour spill.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Pheochromocytoma / surgery

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  • (PMID = 16733977.001).
  • [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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88. Chrisoulidou A, Kaltsas G, Ilias I, Grossman AB: The diagnosis and management of malignant phaeochromocytoma and paraganglioma. Endocr Relat Cancer; 2007 Sep;14(3):569-85
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  • [Title] The diagnosis and management of malignant phaeochromocytoma and paraganglioma.
  • Although a subset of these tumours has metastatic disease at initial presentation, a significant number develops metastases during follow-up after excision of an apparently benign tumour.
  • Clinical, biochemical and histological features cannot reliably distinguish malignant from benign tumours.
  • Several imaging modalities have been utilised for the diagnosis and staging of these tumours.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Paraganglioma / diagnosis. Paraganglioma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 17914089.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Radiopharmaceuticals
  • [Number-of-references] 159
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89. 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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90. 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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  • [Title] [Adrenal gland surgery].
  • The authors of the article analyze more than 30-year experience of surgical treatment of adrenal gland diseases, offering a detailed description of clinical and morphological features of the main nosological forms such as Itsenko-Cushing disease and syndrome, primary hyperaldosteronism, pheochromocytoma, incidentaloma, and malignant adrenal tumors.
  • The paper contains basic diagnostic criteria, indications to operative treatment, and extents of surgery for each of the diseases considered, as well as comparative analysis of peculiarities of conventional ("open"), and endoscopic adrenal gland surgeries.
  • The article demonstrates significance of various immunohistochemical markers in differentiation between malignant and benign adrenal tumors.

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  • (PMID = 16408654.001).
  • [ISSN] 0869-6047
  • [Journal-full-title] Vestnik Rossiiskoi akademii meditsinskikh nauk
  • [ISO-abbreviation] Vestn. Akad. Med. Nauk SSSR
  • [Language] RUS
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Russia (Federation)
  • [Number-of-references] 16
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91. Juan-Sallés C, Ramos-Vara JA, Garner MM: Pheochromocytoma in six New World primates. Vet Pathol; 2009 Jul;46(4):662-6
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  • [Title] Pheochromocytoma in six New World primates.
  • Six New World primates, including 2 golden lion tamarins (Leontopithecus rosalia), 2 cotton-top tamarins (Saguinus o. oedipus), 1 black howler monkey (Alouatta caraya), and 1 black-handed spider monkey (Ateles g. geoffroyi), were diagnosed with unilateral (4/6) or bilateral (1/6) adrenal or extra-adrenal (1/6) pheochromocytoma by light microscopy and immunohistochemical staining for chromogranin A.
  • Overt invasive behavior or metastases were not observed in any primate, and thus these neoplasms were considered benign.
  • [MeSH-major] Adrenal Gland Neoplasms / veterinary. Animals, Zoo. Monkey Diseases / pathology. Pheochromocytoma / veterinary. Platyrrhini

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  • (PMID = 19276065.001).
  • [ISSN] 1544-2217
  • [Journal-full-title] Veterinary pathology
  • [ISO-abbreviation] Vet. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chromogranin A
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92. 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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  • All mutation carriers had adrenal tumors, including 7 bilateral (P = 2.7 × 10(-4)) and/or with familial disease (5 of 20 samples; P = .005).
  • 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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93. 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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  • [Title] Unexpected histological findings of lesions diagnosed in the adrenal region in a series of 420 patients submitted to adrenal surgery. Review of our experience.
  • INTRODUCTION: The incidence of adrenal incidentalomas is reported to be up to 30% in the current literature; nevertheless, in some patients undergoing surgery, a final diagnosis of non-adrenal origin of the mass is performed.
  • In this paper we present our experience of 13 patients with unexpected histological findings of lesions diagnosed in the adrenal region.
  • 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%).
  • RESULTS: Final histology revealed an unexpected diagnosis of non-adrenal origin of the mass in 13 patients (3.1%).
  • Histology demonstrated a benign neurogenic tumor in 10 patients.
  • In the other 3 patients diagnosis was respectively of lymphnode, hemangioma and a gastric metastasis of melanoma.
  • Mean operative time in this group was higher compared to laparoscopic resection for adrenal lesion (95.3 min vs 73.2 min).
  • CONCLUSION: A small percentage of our patients (3.1%) demonstrated unexpected findings of the lesion pre-operatively misinterpreted as an adrenal mass.
  • Despite a complete pre-operative assessment, adrenal lesions might reveal a different origin, increasing the surgical challenge as well as the morbidity for the patient.
  • [MeSH-major] Adrenal Gland Diseases / diagnosis. Adrenal Gland Neoplasms / diagnosis. Adrenalectomy / methods
  • [MeSH-minor] Adrenal Glands / pathology. Adrenal Glands / surgery. Adult. Diagnostic Errors. Female. Humans. Incidental Findings. Laparoscopy. Male. Pregnancy. Tomography, X-Ray Computed

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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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94. Geli J, Kiss N, Karimi M, Lee JJ, Bäckdahl M, Ekström TJ, Larsson C: Global and regional CpG methylation in pheochromocytomas and abdominal paragangliomas: association to malignant behavior. Clin Cancer Res; 2008 May 1;14(9):2551-9
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  • EXPERIMENTAL DESIGN: A panel of 53 primary tumors (42 benign, 11 malignant) was analyzed by quantitative bisulfite pyrosequencing.
  • Global hypomethylation of LINE-1 elements was observed in tumors compared with normal adrenal samples (P < 0.02).
  • [MeSH-major] Abdominal Neoplasms / genetics. Adrenal Gland Neoplasms / genetics. CpG Islands / genetics. DNA Methylation. Paraganglioma / genetics. Pheochromocytoma / genetics. Promoter Regions, Genetic


95. Johnson PT, Horton KM, Fishman EK: Adrenal mass imaging with multidetector CT: pathologic conditions, pearls, and pitfalls. Radiographics; 2009 Sep-Oct;29(5):1333-51
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  • [Title] Adrenal mass imaging with multidetector CT: pathologic conditions, pearls, and pitfalls.
  • The adrenal gland is involved by a range of neoplasms, including primary and metastatic malignant tumors; however, the most common tumor detected is the incidental benign adenoma.
  • Although computed tomographic (CT) findings will not always yield a definitive diagnosis, attention to these findings provides a road map to guide image interpretation.
  • Adrenocortical carcinoma typically has an RPW of less than 40%; however, large size and heterogeneity are more reliable indicators of the diagnosis than are washout values.
  • Washout characteristics of pheochromocytoma are variable; in conjunction with high levels of dynamic enhancement, pheochromocytomas may mimic adenoma (ie, APW > 60%, RPW > 40%).
  • After contrast material administration, metastases usually demonstrate slower washout on delayed images (APW < 60%, RPW < 40%) than do adenomas, although hypervascular metastases may enhance similarly to pheochromocytoma.
  • Finally, a number of nonadrenal pathologic conditions have been reported to mimic adrenal masses at CT.
  • [MeSH-major] Adrenal Gland Neoplasms / radiography. Adrenal Glands / radiography. Diagnostic Errors / prevention & control. Image Enhancement / methods. Tomography, X-Ray Computed / methods

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  • [Copyright] (c) RSNA, 2009.
  • (PMID = 19755599.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 56
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96. Neri V, Ambrosi A, Fersini A, Valentino TP: Laparoscopic adrenalectomy: transperitoneal lateral approach. Cases study. Ann Ital Chir; 2005 Mar-Apr;76(2):123-6
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  • MATERIAL AND METHOD: In the period 1997-September 2004 we treated 17 patients with adrenal pathology: eight patients underwent to an open anterior transperitoneal adrenalectomy and nine patients underwent a laparoscopic adrenalectomy with lateral transperitoneal approach.
  • DISCUSSION: Both procedures allow to achieve the complete resolution of the adrenal pathology if it is confined within the gland and no more than 8 cm. in size.
  • Therefore, the AA can believe that the laparoscopic approach is at present the gold standard in the treatment of all benign adrenal pathologies with a no more than 8 cm. size.
  • [MeSH-minor] Adenoma / surgery. Adrenal Cortex Neoplasms / surgery. Adrenal Gland Neoplasms / surgery. Adrenocortical Adenoma / surgery. Female. Humans. Laparotomy. Male. Middle Aged. Pheochromocytoma / surgery. Safety. Time Factors. Treatment Outcome

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  • (PMID = 16302650.001).
  • [ISSN] 0003-469X
  • [Journal-full-title] Annali italiani di chirurgia
  • [ISO-abbreviation] Ann Ital Chir
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Italy
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97. 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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98. Iihara M, Obara T: [Diagnosis and surgical treatment of adrenal tumors]. Nihon Geka Gakkai Zasshi; 2005 Aug;106(8):479-83
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  • [Title] [Diagnosis and surgical treatment of adrenal tumors].
  • Adrenal surgery is necessary for the management of functioning adrenal tumors, such as aldosterone-producing adenoma, cortisol-producing adenoma, and pheochromocytoma.
  • The role of adrenal imaging in primary hyperaldosteronism is to separate the surgically resectable unilateral aldosteronoma from bilateral hyperplasia.
  • Once the clinical diagnosis of primary hyperaldosteronism is confirmed, adrenal computed tomography (CT) with 3-mm sections should be the first imaging study.
  • If the results of CT and NP-59 scintigraphy are equivocal, adrenal venous sampling is necessary.
  • Cortisol-producing adrenocortical adenomas are seen as adrenal masses 2.5 cm or larger in diameter in CT scanning.
  • When an adrenal mass measures more than 5 cm in diameter, a functioning adrenal carcinoma should be considered.
  • In the past decade, laparoscopic adrenalectomy has replaced open adrenalectomy as a standard operative procedure for benign adrenal tumors.
  • Adrenal-sparing laparoscopic surgery has recently become a feasible option in patients with hereditary bilateral pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery
  • [MeSH-minor] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / surgery. Adrenal Medulla. Humans

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  • (PMID = 16119111.001).
  • [ISSN] 0301-4894
  • [Journal-full-title] Nihon Geka Gakkai zasshi
  • [ISO-abbreviation] Nihon Geka Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 17
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99. Pohl PP, Meyer A, Lammers BJ, Goretzki PE: [Abdominal preoperation. No contraindication for laparoscopic transabdominal adrenalectomy]. Chirurg; 2008 Jun;79(6):571-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Benign adrenal gland tumors smaller than 6 cm are nowadays the indication for minimally invasive surgery.
  • [MeSH-major] Abdomen / surgery. Adrenal Gland Neoplasms / surgery. Adrenalectomy / contraindications. Adrenocortical Adenoma / surgery. Carcinoma / surgery. Cushing Syndrome / surgery. Laparoscopy / contraindications. Pheochromocytoma / surgery

  • MedlinePlus Health Information. consumer health - Adrenal Gland Cancer.
  • MedlinePlus Health Information. consumer health - Cushing's Syndrome.
  • MedlinePlus Health Information. consumer health - Pheochromocytoma.
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  • (PMID = 17879074.001).
  • [ISSN] 0009-4722
  • [Journal-full-title] Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
  • [ISO-abbreviation] Chirurg
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Germany
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100. Reisch N, Walz MK, Erlic Z, Neumann HP: [Pheochromocytoma - still a challenge]. Internist (Berl); 2009 Jan;50(1):27-35
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Pheochromocytoma - still a challenge].
  • Pheochromocytomas are rare, mostly benign catecholamine-producing tumors arising from the chromaffin cells of the adrenal medulla or in the paraganglia.
  • Computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen as well as functional imaging with (123)Iod-MIBG scintigraphy and (18)F-dopa positron emission tomography are used for tumor localization.
  • The therapy of choice is the endoscopic adrenal sparing surgery following preoperative alpha-blockade.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Hypertension / diagnosis. Hypertension / prevention & control. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy






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