[Digestive polyposis. Retrospective study of 20 cases]. / Les polyposes digestives. Etude rétrospective d'une série de 20 patients.

BACKGROUND: Three main polyposis syndromes are transmitted as an autosomal dominant disorder: familial adenomatous polyposis (FAP), juvenile polyposis syndrome (JPS) and Peutz-Jeghers syndrome. AIM: Evaluate this management of digestive polyposis. METHODS: Our study included 20 patients which were collected in th departements of pathology surgery and garstroenterology of MT Maarmouri's Hospital, Nabeul city. RESULTS: We reported 15 cases of adenomatous polyposis with 2 family groups. We identified a family group of JPS with 3 members and 2 cases of Peutz-Jeghers syndrome. We found 11 cases of colonic adenocarcinoma out of the 15 patients affected by adenomatous polyposes. FAP is a generalized disorder involving the entire colorectum segment with numerous extra-colonic manifestations. The risk to develop colonic cancer is 100%. JPS is characterised by the development of numerous gastrointestinal juvenile polyps and occurs usually before 20 years old, the progression to cancer is rarely observed. CONCLUSION: Peutz-Jeghers syndrome consists in hamartomatous polyps associated to a characteristic mucosal pigmentation. The patients are usually young adults and have an increased incidence of cancer in extradigestive sites.

Bilateral orbital myeloid sarcoma as initial manifestation of acute myeloid leukemia.

BACKGROUND: Granulocytic sarcoma is a rare orbital complication of acute leukemia. It concerns primarily children under 10 years of age suffering from primitive acute myeloid leukemia. The diagnosis is made by clinical examination, computed tomography and confirmed by haematological investigations. The treatment approach is based on chemotherapy associated with intravenous steroid therapy. CASE REPORT: We report the case of a 6-year-old girl who presented with bilateral proptosis revealing acute myeloid leukemia. The patient was treated by a combination of chemotherapeutic drugs in two phases, associated with intravenous steroids. After a follow-up period of 24 months, the patient was in complete remission. CONCLUSION: The diagnosis of granulocytic sarcoma should be considered in any orbital mass of uncertain origin, particularly if it is bilateral. Special stains and immunohistochemistry play an important role in the diagnosis.