Von hippel-lindau disease associated pulmonary carcinoid with cranial metastasis

Document Type

Article

Abstract

CONTEXT: Carcinoids have rarely been described in von Hippel-Lindau (VHL) disease. OBJECTIVE: We describe the first reported case of a patient with VHL who developed a pulmonary carcinoid that subsequently metastasized to a pre-existent cranial hemangioblastoma. RESULTS: Histological and immunohistochemical features of the metastatic lesion were similar to the primary carcinoid. Both lesions demonstrated heterozygous VHL gene deletions with fluorescence in situ hybridization analysis. CONCLUSIONS: This case provides direct molecular genetic evidence of an association between VHL and carcinoids.

Medical Subject Headings

Adult; Brain Neoplasms (diagnosis, pathology); Carcinoid Tumor (diagnosis, etiology, pathology); Hemangioblastoma (diagnosis, pathology); Humans; Lung Neoplasms (diagnosis, etiology, pathology); Lymphatic Metastasis; Male; Neoplasms, Second Primary (diagnosis, pathology); von Hippel-Lindau Disease (complications, diagnosis)

Publication Date

8-1-2014

Publication Title

The Journal of clinical endocrinology and metabolism

E-ISSN

1945-7197

Volume

99

Issue

8

First Page

2633

Last Page

6

PubMed ID

24878057

Digital Object Identifier (DOI)

10.1210/jc.2014-1732

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