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Laryngeal carcinoma in a lynch syndrome II kindred
Author(s) -
Lynch Henry T.,
Kriegler Mary,
Christiansen Thomas A.,
Smyrk Tom,
Lynch Jane F.,
Watson Patrice
Publication year - 1988
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19880901)62:5<1007::aid-cncr2820620528>3.0.co;2-x
Subject(s) - lynch syndrome , medicine , colorectal cancer , cancer , endometrial cancer , carcinoma , adenocarcinoma , cervix , uterine cancer , cervical cancer , oncology , ovarian cancer , gynecology , gastroenterology , dna mismatch repair
Abstract Hereditary nonpolyposis colorectal cancer (HNPCC) accounts for about 4% to 6% of the total colorectal cancer burden. It is subdivided into Lynch syndrome I and II. Lynch syndrome I is characterized by an autosomal dominant inheritance pattern for site‐specific, early onset, adenocarcinoma of the colon, with proximal predominance and an excess of synchronous and metachronous colonic cancers. Lynch syndrome II (cancer family syndrome) shows these same colon cancer characteristics, but differs in that there is an excess proclivity of other forms of cancer, particularly of the endometrium and ovary. This article documents a family that shows features of Lynch syndrome II. Unique aspects pertain to a patient who is in the direct genetic lineage (whose five brothers manifested colonic cancer), but who developed carcinoma of the uterine cervix at age 34 and laryngeal cancer at 60. The pedigree also shows uterine cervical carcinoma among other patients at genetic risk. Her son, who is a nonsmoker and nondrinker, manifested laryngeal cancer at age 31. These observations appear to add new information about tumor heterogeneity in HNPCC.