title: [Familial tumor syndrome and ectopic hormone-producing tumors].
abstract: It has been thought that endocrine tumors occurred through interactions of multiple environments factors and a personal genetic factor. A normal somatic cell having an intrinsic function is able to obtain the character of a malignant cell by influence of many factors. Several percent of all tumors have obvious familial aggregation. These entity are called familial cancer. Familial cancer syndrome is well defined for colorectal cancer and breast cancer, but an endocrine neoplasia is the one, too. Ectopic hormone producing tumors are kinds of endocrine tumors, and have the characteristics, which they generate in many organs multicentrically. The phenomena suggest that the patient with these disorders may possess strong genetic predisposition. Among endocrine neoplasia, multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau disease(VHL) are well defined genetic disorder with autosomal dominant inheritance, and the reliable genes were previously identified as MEN1, RET, and VHL, respectively. At this opportunity, we interpret for these three disorders.
authors.affiliation: Laboratory of Hereditary Tumor, Institute for Advanced Medical Sciences, Hyogo College of Medicine.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Tamura
authors.forename: Joji
authors.initials: J
authors.lastname: Utsunomiya
authors.forename: Takehira
authors.initials: T
authors.lastname: Yamamura
mesh_terms: D020137:Base Pair Mismatch / Q000235:genetics; D004260:DNA Repair / Q000235:genetics; D016147:Genes, Tumor Suppressor; D006729:Hormones, Ectopic / Q000378:metabolism*; D006801:Humans; D025202:Molecular Diagnostic Techniques; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism; D009369:Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism; D009857:Oncogenes / Q000235:genetics; D006623:von Hippel-Lindau Disease / Q000235:genetics
chemical_list: D006729:Hormones, Ectopic