Document Detail


Malignant Pheochromocytoma Secreting Vasoactive Intestinal Peptide and Response to Sunitinib: A Case Report and Literature Review.
MedLine Citation:
PMID:  24936559     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Objective: Malignant pheochromocytoma is rare, may be sporadic or have a genetic basis. A vasoactive-intestinal-peptide-(VIP)-secreting pheochromocytoma has hardly been described in the literature, and treatment remains challenging in the absence of well-controlled randomized trials. The HIF-VEGF axis has been implicated in pheochromocytoma when associated with germ-line VHL or SDH mutations, suggesting potential clinical activity of sunitinib in this setting.Methods: We present a case-report of a patient with a VIP-secreting malignant pheochromocytoma manifested as severe watery diarrhea, with an exquisite clinical response to sunitinib. We review this rare clinical entity and the potential role of sunitinib in this context. Case report: A 51-year-old male initially presented with a pheochromocytoma causing symptoms related to norepinephrine excess. He underwent adrenalectomy which resulted in complete resolution of his symptoms. Subsequently, 3 years later, he developed multifocal metastatic disease from his primary tumor showing immunohistochemical evidence of VIP-production accompanied by severe watery diarrhea and hypokalemia. The patient had a rapid, complete and durable clinical response to sunitinib with only a minor radiological response ad significant toxicity. Genetic testing was negative for germline mutations in VHL, SDHB, SDHC, SDHD, TMEM127 and NF-1.Conclusion: To the best of our knowledge, a case of malignant VIP-producing pheochromocytoma that is responsive to sunitinib has not been described previously. We discuss this rare pathological entity and review the current literature on sunitinib in the management of these neoplasms.
Authors:
Raya Lebowitz-Amit; Ozgur Mete; Sylvia L Asa; Shereen Ezzat; Anthony M Joshua
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-6-16
Journal Detail:
Title:  Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists     Volume:  -     ISSN:  1934-2403     ISO Abbreviation:  Endocr Pract     Publication Date:  2014 Jun 
Date Detail:
Created Date:  2014-6-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9607439     Medline TA:  Endocr Pract     Country:  -    
Other Details:
Languages:  ENG     Pagination:  1-17     Citation Subset:  -    
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