Cancer Family Syndrome (Lynch Syndrome II): Preliminary Report of New Phenotypic Findings

Cristofaro, Giuseppe; Caruso, Maria Lucia; Attolini, A.; Matteo, G. Di; Giorgio, P. Di; Trabucco, Mario; Senator, S.; Nacci, Grazia; Argentieri, A.; Sbano, E; Giorgio, I

doi:10.1159/000412539

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“…Of pertinence to our kindred is the precancerous tendency of this pathology. Specifically, Correa's'' epidemiologic classification of CAG shows three divisions: (1) autoimmune, (2) hypersecretory, and (3) environmental.…”

Section: Chronic Atrophic Gastritismentioning

confidence: 99%

“…EREDITARY NONPOLYOSIS COLORECTAL CANCER H (HNPCC) is subdivided into (1) Lynch syndrome I: site-specific colonic cancer; and (2) Lynch syndrome 11: colonic cancer in association with other forms of cancer, particularly carcinoma of the endometrium and ovary;' and recently, carcinoma of the pancreas.' Both HNPCC disorders show a proximal predominance of nonpolyposis colonic cancer, vertical tranmission consonant with an autosomal dominant mode of inheritance as determined both clinically and through segregation analy~is,~ an excess of multiple primary cancer, and significantly improved survival when compared stage for stage with the American College of Surgeons Audit Serie~.~ Those facets of the natural history of HNPCC may be employed advantageously in the development of surveillance/management programs.…”

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confidence: 99%

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New phenotypic aspects in a family with lynch syndrome II

Cristofaro

Lynch²,

Caruso

et al. 1987

Cancer

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Increasing attention has been given to hereditary nonpolyposis colorectal cancer (HNPCC). This report provides medical genetic/pathologic findings on an HNPCC kindred from southern Italy that shows criteria consistent with Lynch syndrome II. An international collaborative effort led to extension of this kindred with disclosure of a potentially new spectrum of phenotypic findings: an excess of gastric carcinoma; complete intestinal metaplasia and chronic atrophic gastritis restricted to the antrum; an apparent excess of colonic mucosal macrophagia, which by special stain appeared to be positive for mucin, with a constant content of both sialo and sulfomucin, a lack of iron, and an inconstant positivity for lysozyme obtained by immunoperoxidase technique; and findings of crypt atrophy of the colonic mucosa. During the relatively short period of investigation of this family, an intensive educational and surveillance program has been mounted in the interest of improving cancer control through direct application of knowledge of natural history and the risk factor evidence through pedigree assessment.

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Section: Chronic Atrophic Gastritismentioning

confidence: 99%

mentioning

confidence: 99%