Familial aggregation of neuroectodermal and gastrointestinal tumors

A family with multiple tumors is presented. Four cases were childhood neoplasms derived from the neuroectoderm, as was a malignant melanoma in a young female and a branchiogenic cyst in her brother. He also had an early onset of an adenocarcinoma of the transverse colon. Four further cases of gastrointestinal tumors and one case of acute myelogenous leukemia had occurred in the family. One of the childhood tumors was a bilateral retinoblastoma. It is discussed as to whether the tumors in the studied family were due to a genetic factor resulting in a neuroectodermal embryopathy, or were caused by a retinoblastoma gene with a pleiotropic effect.