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Peutz‐Jeghers syndrome. A clinicopathologic study of a large family with a 27‐year follow‐up
Author(s) -
Burdick Daniel,
Prior John T.
Publication year - 1982
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(19821115)50:10<2139::aid-cncr2820501028>3.0.co;2-k
Subject(s) - peutz–jeghers syndrome , medicine , jejunum , malignancy , intestinal cancer , adenocarcinoma , fibroadenoma , family history , gastroenterology , cancer , breast cancer , general surgery , pathology , colorectal cancer
A family group of ten patients with the Peutz‐Jeghers syndrome has been followed for a 27‐year period. Eight members of the family had one or more manifestations of the syndrome. One member of the family died following a bypass procedure of a nonresectable carcinoma of the jejunum. Postmortem examination revealed this to be an adenocarcinoma of the jejunum arising in a Peutz‐Jeghers polyp and metastases were present in the mesenteric lymph nodes. Two members of the family developed breast carcinomas, one arising in a fibroadenoma; both patients died, one of metastatic breast carcinoma, the other of a second primary malignancy (adenocarcinoma of the jejunum arising in a Peutz‐Jeghers polyp). Three family members had benign ovarian tumors, one patient had a benign breast tumor and another patient had a benign colloid thyroid nodule. While the authors of this report believe that they have added a documented case of an adenocarcinoma of the jejunum arising in a Peutz‐Jeghers polyp to the literature, the exact risk of intestinal cancer in the Peutz‐Jeghers syndrome is unknown, but probably very small. Gastrointestinal surgery should continue to be performed in symptomatic patients and all patients should be followed closely at regular intervals.