[Recurrent inflammatory myofibroblastic tumor with renal, retroperitoneal and lymph node involvement]. / Tumeur myofibroblastique inflammatoire récurrente avec triple localisation, rénale, rétropéritonéale et ganglionnaire.

Inflammatory myofibroblastic tumors are uncommon and benign tumors with unknown aetiology. First reported in the lungs, the inflammatory myofibroblastic tumors have been observed in other locations, especially in the abdomen and the pelvis. We report a 14-year-old adolescent female, who presented sequentially an inflammatory pseudotumor of lymph node, the left kidney and the retroperitoneum. Extrapulmonary inflammatory myofibroblastic tumors are mesenchymal solid tumors. They are frequently circumscribed and confined to a single organ. The recurrence of some inflammatory myofibroblastic tumors and their expression of chromosomal abnormalities found in some types of lymphoma suggest that some of these lesions constitute a true neoplastic process.

[Pediatric Hodgkin disease in south Tunisia: a study of 23 cases]. / Maladie de Hodgkin de l'enfant dans le sud tunisien: étude de 23 cas.

PURPOSE: To discuss, through a retrospective study, the epidemiologic and anatomo-clinic aspects of paediatric Hodgkin disease (HD) in south Tunisia and to study the faisability of the French protocol MDH 90 for our patients. PATIENTS AND METHODS: Between January 1995 and December 2000, we treated 23 children with HD. Initial work-up included clinical examination, radiological explorations (chest X ray, abdomen ultrasonography and computed tomography scan of chest, abdomen and pelvis), biological explorations (full blood count, erythrocyte sedimentation rate, serum albumin, liver functions tests) and a bone marrow biopsy. Treatment was inspired from the SFOP MDH 90 protocol. Overall survivals and disease free survivals were estimated with Kaplan-Meier method and compared with the log-rank test. RESULTS: Mean age was 8.6 years (4-14 years), sex-ratio was 3.6. Histological subtypes 2 and 3 had the same frequency. Localised stages (I-II) were as frequent as advanced stages (III-IV). At the end of the treatment, the 18 evaluables patients were in complete remission. The 5-year overall survival (OS) and disease free survival (DFS) were 82 and 76% respectively. Localised stages and advanced stages had the same 5-year overall survival (82%). There were no statistical differences in term of OS between the different prognosis factors studied. CONCLUSIONS: The particularity of our study was the identical frequency of the two histological subtypes 2 and 3 and of localised and advanced stages. The MDH 90 protocol is feasable for our patients with satisfactory results similar to those found in the literature.

Iatrogenic colorectal Kaposi's sarcoma complicating a refractory ulcerative colitis in a human immunodeficiency negative-virus patient.

Kaposi sarcoma is an unusual tumor associated to a human herpes virus-8 infection involving the skin or internal organs. Iatrogenic Kaposi's sarcoma often occurs in patients receiving immunosuppressive therapy. So far, a few Kaposi's sarcoma cases have been reported in the literature associated with inflammatory bowel diseases. We report a 53-year-old male diagnosed with a severe refractory ulcerative colitis who was treated with corticosteroids and azathioprine. The patient underwent a colectomy after the failure of medical treatment. Histological examination of the colon showed findings suggestive of Kaposi's sarcoma. Immunohistochemistry for human herpes virus-8 was positive in the colonic lesions. Correspondence.

[Embryonal carcinoma of the submandibular gland. Apropos of a case]. / Le carcinome embryonnaire de la loge sous-maxillaire. A propos d'un cas.

The authors report a case of infantile embryonal carcinoma of the submandibular gland in a nine months old infant. The diagnosis was based on typical histopathological features, and positive reaction of tumor cells with alpha-foetoprotein. The patient was treated by wide surgical excision and chemotherapy. The clinical course was favourable two years later with normalization of serum alpha-foetoprotein levels and with no abnormality on the thoraco-abdomino-facial CT scan.

Cutaneous lymphomas of phenotypically undetermined lineage: contribution of genotypic analysis.

Genotypic analyses were performed in six primary cutaneous lymphomas whose lineage could not be assessed on the basis of histologic and phenotypic data. By immunophenotyping, these neoplasms expressed leukocyte common antigen and HLA-DR but did not show consistent immunostaining for B-cell or T-cell differentiation antigens. Expression of nonspecific histiocytic markers such as lysozyme and alpha 1-antitrypsin was found in three cases. By genotyping, three cases retained a germline configuration and immunoglobulin gene rearrangement was observed in one case, T-cell receptor gene rearrangement was found in one case, and both types of rearrangements in one case. Of the three patients in whom gene rearrangements were noted, two rapidly died and the other patient, with a dual genotype, is still alive 15 years after diagnosis. The three patients without gene rearrangements are alive and well after a mean follow-up of 2.5 years. It appears that cutaneous lymphomas with an uncertain phenotype include at least some cases of authentic B-cell or T-cell lymphomas. The germline configuration that we observed in cases with a chronic course remains difficult to explain. It may be related to a low malignancy form of histiocytic lymphoma, an atypical polyclonal hyperplasia, or even a low-grade lymphoma arising from a primitive cell without established commitment to either B- or T-cell lineage.