Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0017636 (glioblastoma)
 18,345 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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 comprised 47 individuals (28 males, 19 females), of whom 21 (16 males and 5 females), i.e. 44.6%, have malignant tumors. Of the 32 people in the fourth generation, only one individual is affected to date (a girl age 21, IV/4). There were 27 tumors 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, a cerebral metastasis from an adenocarcinoma, the origine of which has not been established, and a tumor invading the biliary tract. Three members of the family had multiple tumors. In three of the patient the colonic adenocarcinoma was accompanied by one or two polyps. The average age at onset for all tumors 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%. Finally, the diagnostic criteria for hereditary adenocarcinoma are discussed and the different familial forms of cancer are reviewed.
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PMID:[Familial cancer syndrome studies in 4 generations of a family]. 19 29

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.
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PMID:[Hereditary adenocarcinomatosis in 4 generations of a Valais family]. 59 30

Preliminary electron microprobe studies of a small number of meningiomas, oligodendrogliomas, glioblastoma multiformae, craniopharyngiomas, and metastatic epithelioma of the breast suggest that the elemental composition of the deposits associated with these tumors is characteristic for each type of tumor.
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PMID:Electron microprobe study of calcifications in human brain tumors. 71 42