title: [Malignant leiomyoblastoma of the stomach (epithelioid leiomyosarcoma)].
abstract: Primary malignant tumors of the stomach are rare in children. They are chiefly sarcomas and lymphomas, carcinomas being quite exceptional. Leiomyoblastomas are mesenchymal tumors arising from smooth muscles. Histologic differentiation between benign and malignant leiomatous tumor is difficult. However, an histopathologic grade of malignancy can be assigned to each tumor according to the degree of hypercellularity, nuclear abnormality, mitotic rate, tumor size and invasion of adjacent organs. Moreover, some of these tumors are noted in conjunction with Carney's triad, associated with a pulmonary chondroma and an extra-adrenal para-ganglioma. Pre-operative correct diagnosis is seldom made, although the incidence of gastro-intestinal bleeding and anemia is much higher in smooth muscle tumors than in others. Wide surgical excision must be performed, whenever a chance to completely remove the tumor exists. Gastric resection is well tolerated in children and does not significantly alter the child's normal growth and development. The high risk of local or metastatic recurrence, the chance for a Carney's triad to appear imply a poor prognosis and neccesitate a long follow-up period. We here describe the case of a 15-year-old boy, whose gastric tumor was found on the fact of an important melena and anemia. Gastroscopy showed an ulcerative tumor of the lesser curvature and roentgenogram from a barium study described an important and irregular defect in gastric body with a central ulcer overlying the mass. At operation, the tumor appeared to be unlimited and total gastrectomy was performed. Histologic findings showed an epithelioid leiomyosarcoma with some unfavorable microscopic factors.(ABSTRACT TRUNCATED AT 250 WORDS)
mesh_terms: D000293:Adolescent; D006801:Humans; D007890:Leiomyosarcoma / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D008297:Male; D011379:Prognosis; D013274:Stomach Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery
vernacular_title: Léiomyoblastome malin de l'estomac (léiomyosarcome épithélioïde).