Von hippel-lindau disease associated pulmonary carcinoid with cranial metastasis
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)
The Journal of clinical endocrinology and metabolism
Digital Object Identifier (DOI)
Zhang, Chao; Yang, Andrew I.; Vasconcelos, Lucas; Moon, Seog; Yang, Chunzhang; Nesvick, Cody L.; Saidkhodjaeva, Lola; Abdullaev, Ziedulla; Pack, Svetlana D.; Ghosh, Arunima; Chittiboina, Prashant; Heiss, John D.; Zhuang, Zhengping; Quezado, Martha M.; and Zaghloul, Kareem A., "Von hippel-lindau disease associated pulmonary carcinoid with cranial metastasis" (2014). Neurosurgery. 1891.