Metastatic Pheochromocytoma Diagnosed with 131 I-MIBG SPECT/CT Imaging in a Patient with Pathogenic VHL Mutation

Pheochromocytoma is a rare catecholamine secreting neuroendocrine tumor arising from chromaffin cells of adrenal medulla with approximate prevalence of 0.1 to 0.6% in patients suffering from hypertension. Hypertensive control followed by surgical resection remains the primary treatment of choice. Al...

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Bibliographic Details
Published inWorld journal of nuclear medicine Vol. 21; no. 1; pp. 73 - 75
Main Authors Vankadari, Kousik, Boddula, Raman, Hegde, Aditya Gajanan, Chinte, Chimutai
Format Journal Article
LanguageEnglish
Published Germany Thieme Medical and Scientific Publishers Pvt. Ltd 30.04.2022
Subjects
Case Report
metastatic pheochromocytoma
missense VHL mutation
Arg167Gln
MIBG
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Summary:Pheochromocytoma is a rare catecholamine secreting neuroendocrine tumor arising from chromaffin cells of adrenal medulla with approximate prevalence of 0.1 to 0.6% in patients suffering from hypertension. Hypertensive control followed by surgical resection remains the primary treatment of choice. Although it is considered a slow growing benign tumor, it rarely leads to recurrence of tumor in the lymph nodes, liver, and lungs. Association of benign pheochromocytoma with familial or de novo Von Hippel-Lindau (VHL) mutations is well reported in literature. Here, we report a case of metastatic pheochromocytoma arising from commonly seen benign VHL mutation.
ISSN:1450-1147
1607-3312
DOI:10.1055/s-0042-1746177