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.
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.
Authors: F Latif; K Tory; J Gnarra; M Yao; F M Duh; M L Orcutt; T Stackhouse; I Kuzmin; W Modi; L Geil Journal: Science Date: 1993-05-28 Impact factor: 47.728
Authors: E R Maher; L Iselius; J R Yates; M Littler; C Benjamin; R Harris; J Sampson; A Williams; M A Ferguson-Smith; N Morton Journal: J Med Genet Date: 1991-07 Impact factor: 6.318
Authors: Russell R Lonser; Gladys M Glenn; McClellan Walther; Emily Y Chew; Steven K Libutti; W Marston Linehan; Edward H Oldfield Journal: Lancet Date: 2003-06-14 Impact factor: 79.321