Adrenal adenoma in von Hippel-Lindau syndrome: A case report with review of literature.

Abstract:

:A 29-year-old hypertensive male with von Hippel-Lindau (VHL) syndrome came to the Endocrinology department for evaluation. Contrast-enhanced computed tomography of the abdomen revealed an adrenal mass, bilateral renal cell carcinoma, and multiple pancreatic cysts. The hormonal investigations for adrenal mass were normal. He underwent left-sided adrenalectomy, and the histopathological report was suggestive of an adrenocortical adenoma. Genetic analysis of VHL gene in this patient revealed a heterogeneous 5' splice site variation of intron 1 of the VHL gene that affects splice site of exon 1 (c. 340 + 1G > A). Adrenocortical adenoma is very rare in VHL syndrome. Only two cases of adrenocortical adenoma in VHL have been reported in the literature, and both were associated with pheochromocytoma. This is probably the first reported case of adrenocortical adenoma in VHL syndrome without accompanying pheochromocytoma.

journal_name

J Cancer Res Ther

authors

Palui R,Kamalanathan S,Sahoo J,Dorairajan LN,Badhe B,Gochhait D

doi

10.4103/jcrt.JCRT_127_18

subject

Has Abstract

pub_date

2019-03-01 00:00:00

pages

S163-S166

issue

Supplement

eissn

0973-1482

issn

1998-4138

pii

JCanResTher_2019_15_8_163_244437

journal_volume

15

pub_type

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