Peutz-Jeghers syndrome. A clinicopathologic study of a large family with a 27-year follow-up

التفاصيل البيبلوغرافية
العنوان: Peutz-Jeghers syndrome. A clinicopathologic study of a large family with a 27-year follow-up
المؤلفون: John T. Prior, Daniel Burdick
المصدر: Cancer. 50:2139-2146
بيانات النشر: Wiley, 1982.
سنة النشر: 1982
مصطلحات موضوعية: Cancer Research, medicine.medical_specialty, Pathology, business.industry, Peutz–Jeghers syndrome, medicine.disease, Malignancy, Fibroadenoma, Gastroenterology, digestive system diseases, Jejunum, medicine.anatomical_structure, Oncology, Internal medicine, Carcinoma, Medicine, Adenocarcinoma, Mesenteric lymph nodes, skin and connective tissue diseases, business, Intestinal Cancer
الوصف: 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.
تدمد: 1097-0142
0008-543X
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_________::927de4212d1a01ccc90b4c96830a0767
https://doi.org/10.1002/1097-0142(19821115)50:10<2139::aid-cncr2820501028>3.0.co;2-k
حقوق: OPEN
رقم الأكسشن: edsair.doi...........927de4212d1a01ccc90b4c96830a0767
قاعدة البيانات: OpenAIRE