Familial blood vessel tumors and subsequent cancers

Background: Population-based data on the familial risk for vascular tumors are largely lacking. Such data are important for clinical counseling and cancer genetics. Methods: We used the Swedish Family-Cancer Database to calculate standardized incidence ratios for specific subtypes of vascular tumors in offspring using parents as probands. In addition, risks for second cancers were analyzed. Results: Offspring hemangioblastoma in the nervous system was associated with parental kidney cancer and nervous system hemangioblastoma and hemangioma. Offspring nervous system hemangiopericytoma was associated with parental pituitary adenomas. Offspring angiosarcoma in the trunk and extremities was associated with maternal breast cancer. Second Kaposi's sarcoma, non-Hodgkin's lymphoma, Hodgkin's disease and myeloma were increased following primary skin Kaposi's sarcoma. Kidney and endocrine gland tumors and nervous system hemangioblastomas and hemangiomas were in excess following primary nervous system hemangioblastoma and hemangioma. Conclusions: Our data showed that familial clustering of nervous system hemangioblastoma and hemangioma and the risks of subsequent cancers were primarily related to von-Hippel-Lindau disease. As a novel association, offspring nervous system hemangiopericytomas were in excess when parents were diagnosed with pituitary adenoma. Similarly, offspring angiosarcoma is associated with maternal breast cancer. Immunodeficiency may explain the excess of lymphoproliferative diseases after skin Kaposi's sarcoma.