title: [Hereditary adenocarcinomatosis in 4 generations of a Valais family].
abstract: An account is given of a family from the Canton of Valais, suffering from hereditary adenocarcinomatosis. The pedigree extends over four generations; the first three comprise 47 individuals (28 males, 19 females), of whom 21 (16 males and 5 females), i.e. 44.6%, are affected with malignant tumours. Of the 32 people in the fourth generation, only one individual is affected to date (a girl aged 21, IV/14). There were 27 tumours in all: 16 adenocarcinomas of the colon, two gastric adenocarcinomas, one duodenal adenocarcinoma, one rectal adenocarcinoma, one papillary carcinoma of the ovary, one osseous sarcoma, one cutaneous fibrosarcoma, a multiform glioblastoma of the basal nuclei of the brain, a basocellular epithelioma, also a cerebral metastasis from an adenocarcinoma, the origin of which has not been established, and a tumour invading the biliary tract. Three members of the family suffered from multiple tumours. In three of the patients, the colonic adenocarcinoma was accompanied by one or two polyps. The average age at the onset for all the tumours was 45 years. It was definitely lower in the third than the second generation (anticipation). The transmission was autosomal dominant, with predilection for the male sex (57.1% male and 26.3% female patients). The penetrance was about 80%. The author finally discusses the diagnostic criteria for hereditary adenocarcinoma and reviews the different familial forms of cancer.
mesh_terms: D000230:Adenocarcinoma / Q000235:genetics*; D000328:Adult; D003110:Colonic Neoplasms / Q000235:genetics; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000235:genetics*; D006801:Humans; D007417:Intestinal Polyps / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000235:genetics; D010375:Pedigree; D012737:Sex Factors; D013557:Switzerland
vernacular_title: Adenocarcinomatose héréditaire dans quatre générations d'une famille Valaisanne.