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

Abstract 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 c...

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Bibliographic Details
Published inWorld journal of nuclear medicine Vol. 21; no. 1; pp. 073 - 075
Main Authors Vankadari, Kousik, Boddula, Raman, Hegde, Aditya Gajanan, Chinte, Chimutai
Format Journal Article
LanguageEnglish
Published A-12, 2nd Floor, Sector 2, Noida-201301 UP, India Thieme Medical and Scientific Publishers Pvt. Ltd 01.03.2022
Subjects
Case Report
metastatic pheochromocytoma
missense VHL mutation
MIBG
Arg167Gln
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Summary:Abstract 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