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1. Timmers HJ, Brouwers FM, Hermus AR, Sweep FC, Verhofstad AA, Verbeek AL, Pacak K, Lenders JW: Metastases but not cardiovascular mortality reduces life expectancy following surgical resection of apparently benign pheochromocytoma. Endocr Relat Cancer; 2008 Dec;15(4):1127-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastases but not cardiovascular mortality reduces life expectancy following surgical resection of apparently benign pheochromocytoma.
  • The treatment of choice for non-metastatic pheochromocytoma is surgical resection.
  • Data on long-term mortality and morbidity after pheochromocytoma surgery are limited.
  • We here report a retrospective study on the long-term outcome after surgery for apparently benign pheochromocytoma at the Radboud University Nijmegen Medical Centre.
  • Two patients experienced a benign recurrence. Mean+/-s.d. follow-up was 10.2+/-7.5 (median 9, range 1-38) years.
  • In conclusion, compared with the general population patients have a reduced life expectancy following pheochromocytoma surgery, due to their risk of developing metastatic disease.
  • Only one-third becomes normotensive without antihypertensive medication.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Cardiovascular Diseases / mortality. Life Expectancy. Pheochromocytoma / secondary. Pheochromocytoma / surgery

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  • (PMID = 18824558.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Catecholamines
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2. Terzolo M, Bovio S, Pia A, Reimondo G, Angeli A: Management of adrenal incidentaloma. Best Pract Res Clin Endocrinol Metab; 2009 Apr;23(2):233-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Cortical adenoma is the most frequent tumour detected incidentally, but adrenocortical cancer, phaeochromocytoma and metastasis are not rare.
  • (2) identify phaeochromocytoma.
  • Radiological evaluation is the key to the differential diagnosis of benign and malignant tumours.
  • Endocrine testing is necessary to exclude phaeochromocytoma in all patients with an adrenal incidentaloma because this tumour may remain undiagnosed after imaging studies.
  • [MeSH-minor] Adenoma / diagnosis. Adenoma / drug therapy. Adenoma / therapy. Animals. Humans. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 19500766.001).
  • [ISSN] 1878-1594
  • [Journal-full-title] Best practice & research. Clinical endocrinology & metabolism
  • [ISO-abbreviation] Best Pract. Res. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 46
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3. Bembo SA, Elimian A, Waltzer W, Carlson HE: Pheochromocytoma in a pregnant woman with a history of intracerebral aneurysms. Am J Med Sci; 2005 Jun;329(6):317-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma in a pregnant woman with a history of intracerebral aneurysms.
  • Pheochromocytoma in pregnancy is rare; if unrecognized, potentially fatal hypertensive crises may occur.
  • A benign pheochromocytoma was removed without incident.
  • This case illustrates the importance of early diagnosis and management of pheochromocytoma in pregnancy and is also an example of the rare association of pheochromocytoma with intracerebral aneurysms.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pheochromocytoma / diagnosis. Pregnancy Complications, Neoplastic / diagnosis
  • [MeSH-minor] Adult. Antihypertensive Agents / therapeutic use. Female. Humans. Hypertension / drug therapy. Hypertension / etiology. Intracranial Aneurysm. Pregnancy


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4. Druce MR, Kaltsas GA, Fraenkel M, Gross DJ, Grossman AB: Novel and evolving therapies in the treatment of malignant phaeochromocytoma: experience with the mTOR inhibitor everolimus (RAD001). Horm Metab Res; 2009 Sep;41(9):697-702
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Novel and evolving therapies in the treatment of malignant phaeochromocytoma: experience with the mTOR inhibitor everolimus (RAD001).
  • Phaeochromocytoma and paraganglioma are rare neuroendocrine tumours (NETS).
  • They may be benign or malignant but the pathological distinction is mainly made when metastases are present.
  • This short review outlines the main molecular and histological features of malignant phaeochromocytoma and the difficulties in differentiating between benign and malignant disease.
  • We list current therapies used for malignant pheochromocytoma; however, these generally achieve relatively low success rates.
  • In vitro studies have implicated the PI3/Akt/mTOR pathway in the pathogenesis of malignant NETS, including phaeochromocytoma.
  • We have used RAD001 in four patients with progressive malignant paraganglioma/phaeochromocytoma in addition to other therapies (with institutional approval for compassionate use), and evaluated the effects of this treatment.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Pheochromocytoma / drug therapy. Protein Kinase Inhibitors / therapeutic use. Sirolimus / analogs & derivatives
  • [MeSH-minor] Adolescent. Adult. Everolimus. Female. Humans. Male. Protein Kinases / metabolism. Signal Transduction / drug effects. TOR Serine-Threonine Kinases. Young Adult

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  • (PMID = 19424940.001).
  • [ISSN] 1439-4286
  • [Journal-full-title] Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et métabolisme
  • [ISO-abbreviation] Horm. Metab. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Protein Kinase Inhibitors; 9HW64Q8G6G / Everolimus; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; W36ZG6FT64 / Sirolimus
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5. Imai T, Kikumori T, Shibata A, Fujiwara M, Hibi Y, Nakao A: Laparoscopic adrenalectomy for incidentaloma and bilateral adrenal disease. Asian J Surg; 2003 Apr;26(2):64-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Adrenalectomy is ideally suited to minimally invasive surgery based on the characteristics of adrenal tumours, which are usually small and benign.
  • From October 1995 through August 2002, 133 patients underwent adrenal surgery at the Department of Surgery II, Nagoya University School of Medicine.
  • The average size of a non-functioning adrenal tumour was 5.8 cm in diameter, which was equal to the average size of a phaeochromocytoma.
  • The benefits of the laparoscopic approach to adrenalectomy should not result in a more aggressive attitude toward the excision of clinically silent, benign-appearing adrenal incidentalomas.

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  • (PMID = 12732488.001).
  • [ISSN] 1015-9584
  • [Journal-full-title] Asian journal of surgery
  • [ISO-abbreviation] Asian J Surg
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] China
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6. Pucar D, Marković S: [Nuclear medicine diagnosis of pheochromocytoma with metaiodobenzylguanidine]. Srp Arh Celok Lek; 2002 Jul;130 Suppl 2:20-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Nuclear medicine diagnosis of pheochromocytoma with metaiodobenzylguanidine].
  • They may result from benign or malignant adrenal tumours, adrenal hyperplasia or, least frequently, from extra-adrenal disease.
  • Phaeochromocytoma is rare in hypertensive population, affecting only an estimated of 0.1%.
  • The purpose of this study is to point that metaiodobenzylguanidine (mlBG) has proved to be a safe, sensitive and highly specific agent for the location of phaeochromocytoma.
  • Hepatic uptake is maximal at 24 h, declining to very low levels by 72 h (even more rapid in patients with phaeochromocytoma.
  • Dosimetric corlsiderations limit the amount of 131l-mlBG that is administered for diagnostic studies.
  • This, coupled with the low detection efficiency of gamma cameras for the 364 keV photon of 131l, led to the introduction of 131l-mlBG as an adrenomedullary scintigraphic agent of choice.
  • Like the other worldwide experience with this agent our sensitivity was 88.58% and specificity of 98.46%.
  • After all we can say that mlBG has proved to be a safe, sensitive and highly specific agent for the location of phaeochromocytoma and neuroblastoma.
  • Other radiolabelled aralkylamines have been examined as potential adrenal medullary scintigraphic agents.
  • 131l-mlBG in high doses is successfully used in therapy of malignant phaeochromocytoma and especially in nuroblastoma.
  • [MeSH-major] 3-Iodobenzylguanidine. Adrenal Gland Neoplasms / radionuclide imaging. Pheochromocytoma / radionuclide imaging. Radiopharmaceuticals


7. Kaman L, Behera A, Singh R, Katariya RN: Surgical management of phaeochromocytoma. Asian J Surg; 2002 Apr;25(2):139-44
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical management of phaeochromocytoma.
  • OBJECTIVES: Phaeochromocytoma is a rare tumour, which is benign but metabolically functional, with a potential for malignancy.
  • The purpose of this study was to identify the presenting features, diagnostic tests and appropriate surgical approaches for phaeochromocytoma.
  • METHODS: Retrospective analysis was performed on 26 patients with phaeochromocytoma who were admitted to General Surgery, Department of Postgraduate Institute of Medical Education and Research, Chandigarh, India from 1993 to 1999.
  • Their ages ranged from 13 to 60 years, with a peak phaeochromocytoma incidence in the second decade of life.
  • Two patients had phaeochromocytoma as a manifestation of multiple endocrine neoplasia type 2 syndrome (MEN-2).
  • Six (23%) patients had extra-adrenal phaeochromocytoma.
  • The incidence of bilateral tumour was 25% of adrenal tumour and 11.5% had malignant phaeochromocytoma.
  • The diagnostic measurements were:.
  • Computed tomography of the abdomen was extremely accurate in diagnosing phaeochromocytoma (92% accuracy).
  • Preoperatively, all the patients were put on alpha adrenergic blocking agents to control blood pressure for varying periods of time.
  • All the resected specimens were subjected to histopathological examination and phaeochromocytoma was subsequently confirmed.
  • CONCLUSION: If preoperative diagnosis and localization of phaeochromocytoma is accurate, surgical resection can be done with minimal morbidity and mortality.

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  • (PMID = 12376234.001).
  • [ISSN] 1015-9584
  • [Journal-full-title] Asian journal of surgery
  • [ISO-abbreviation] Asian J Surg
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] China
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8. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • Functional imaging using radiolabelled metaiodobenzylguanidine (MIBG) and more recently, (18)F-fluorodopamine and (18)F-fluorodopa positron emission tomography offer substantial sensitivity and specificity to correctly detect metastatic phaeochromocytoma and paraganglioma and helps identify patients suitable for treatment with radiopharmaceuticals.
  • Ongoing microarray studies may provide novel intracellular pathways of importance for proliferation/cell cycle control, and lead to the development of novel pharmacological agents.
  • [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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9. Phaeochromocytoma unmasked by drug therapy. Prescrire Int; 2003 Oct;12(67):181-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phaeochromocytoma unmasked by drug therapy.
  • (1) Phaeochromocytoma is a benign or malignant tumour that stores and produces catecholamines (adrenaline and noradrenaline, and their metabolites metanephrine and normetanephrine). (2) Phaeochromocytoma can be unmasked by some drugs. (3) Metoclopramide and other neuroleptics are most often implicated.
  • Other drugs include antidepressants (such as selective serotonin reuptake inhibitors, monoamine oxidase inhibitors, and imipramine), betablockers, opioids, and curare.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Drug-Related Side Effects and Adverse Reactions. Metoclopramide / adverse effects. Pheochromocytoma / diagnosis
  • [MeSH-minor] Adrenergic beta-Antagonists / adverse effects. Antidepressive Agents / adverse effects. Antipsychotic Agents / adverse effects. Humans. Monoamine Oxidase Inhibitors / adverse effects. Narcotics / adverse effects. Serotonin Uptake Inhibitors / adverse effects

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  • (PMID = 14619898.001).
  • [ISSN] 1167-7422
  • [Journal-full-title] Prescrire international
  • [ISO-abbreviation] Prescrire Int
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Adrenergic beta-Antagonists; 0 / Antidepressive Agents; 0 / Antipsychotic Agents; 0 / Monoamine Oxidase Inhibitors; 0 / Narcotics; 0 / Serotonin Uptake Inhibitors; L4YEB44I46 / Metoclopramide
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