Cause of death* | |||
---|---|---|---|
Birth cohort (no. of deaths) | Non-vHL-related cause | vHL-related cause | Unknown cause |
Born 1840–1900 (n=11) | 3 (27%) | 4 (36%) CNS hb: 4 (100%) | 4 (36%) |
Born 1901–1955 (n=50) | 7 (14%) | 43 (86%) CNS hb: 18 (42%) RCC: 18 (42%) Pancreatic cancer: 2 (5%) Pheo: 1 (2%) Unspecified: 2 (2%) Suicide: 2 (2%) | 0 (0%) |
Born 1956–2010 (n=6) | 0 (0%) | 6 (100%) CNS hb: 5 (83%) RCC: 1 (17%)† | 0 (0%) |
Total (n=67) | 10 (15%) | 53 (79%) CNS hb: 27 (51%) RCC: 19 (36%) Pancreatic cancer: 2 (3%) Pheo: 1 (2%) Unspecified: 2 (3%) Suicide: 2 (3%) | 4 (6%) |
*vHL-related cause: defined as a death caused directly or indirectly by a vHL manifestation, treatment or sequelae.
Three suicides were classified based on events leading up to death described in medical records, autopsies and police reports. Two suicides were classified as vHL-related, because the patients were described as being depressed about their vHL diagnosis. The third suicide was classified as vHL-unrelated, as the vHL diagnosis was not made until after death and the patient was known to have bipolar disease.
†The one patient who died from RCC had dropped out of surveillance 16 years prior to death.
CNS, central nervous system; hb, hemangioblastoma; Pheo, pheochromocytoma; RCC, renal cell carcinoma; vHL, von Hippel-Lindau disease.