Dégénérescence inaugurale d’un polype hamartomateux au cours du syndrome de Peutz-Jeghers : à propos d’un cas avec revue de la littérature
Autor: | S. Mzabi-Regaya, S. Bouraoui, F. Lemaiem, H. Azouz, H. Kechrid |
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Rok vydání: | 2008 |
Předmět: |
congenital
hereditary and neonatal diseases and abnormalities Pathology medicine.medical_specialty Tumor suppressor gene business.industry Gastroenterology Cancer Peutz–Jeghers syndrome General Medicine medicine.disease digestive system diseases Germline mutation Hamartomatous Polyp otorhinolaryngologic diseases Medicine Adenocarcinoma Lentiginosis Gastrointestinal cancer skin and connective tissue diseases business |
Zdroj: | Gastroentérologie Clinique et Biologique. 32:250-254 |
ISSN: | 0399-8320 |
Popis: | The malignant potential of hamartomatous polyps in Peutz-Jeghers' (PPJ) syndrome has been debated. Although it is a very rare event, these polyps can become malignant, as demonstrated by this report. One case of colonic adenocarcinoma associated with Peutz-Jeghers' syndrome is described in a 62-year-old woman. The patient had colonic carcinoma which developed in a hamartomatous polyp. The malignant development of this colonic hamartomatous polyp arising in Peutz-Jeghers' syndrome was pathologically confirmed at surgery. This case also shows a sequence of hamartoma-dysplasia-carcinoma in a hamartomatous polyp without adenomatous changes. This suggests that hamartomatous polyps in Peutz-Jeghers' syndrome may develop into adenocarcinoma and may be a precursor of gastrointestinal carcinomas. STK 11 is a tumor suppressor gene regulating the development of hamartomas, and this somatic mutation promotes gastrointestinal cancer at later stages in Peutz-Jeghers' syndrome. |
Databáze: | OpenAIRE |
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