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胃恶性平滑肌瘤(上皮样平滑肌肉瘤)

[Malignant leiomyoblastoma of the stomach (epithelioid leiomyosarcoma)].

作者信息

Galifer R B, Margueritte G, Barneon G, Bosc O, Couture A, Rieu D

出版信息

Chir Pediatr. 1987;28(2):120-4.

PMID:3621389
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)

摘要

原发性胃恶性肿瘤在儿童中很少见。主要是肉瘤和淋巴瘤,癌则非常罕见。平滑肌瘤是起源于平滑肌的间叶性肿瘤。良性和恶性平滑肌瘤的组织学鉴别很困难。然而,可根据细胞增多程度、核异常、有丝分裂率、肿瘤大小及对邻近器官的侵犯情况为每个肿瘤确定组织病理学恶性分级。此外,其中一些肿瘤与卡尼三联征有关,伴有肺软骨瘤和肾上腺外副神经节瘤。尽管平滑肌瘤导致的胃肠道出血和贫血发生率比其他肿瘤高得多,但术前很少能做出正确诊断。只要有机会完全切除肿瘤,就必须进行广泛的手术切除。儿童对胃切除术耐受性良好,且不会显著改变儿童的正常生长发育。局部或转移复发的高风险以及出现卡尼三联征的可能性意味着预后不良,需要长期随访。我们在此描述一名15岁男孩的病例,他因严重黑便和贫血发现胃部肿瘤。胃镜检查显示胃小弯有溃疡性肿瘤,钡餐造影X线片显示胃体有一个大的不规则缺损,肿物上有中央溃疡。手术时,肿瘤似乎无法切除,遂行全胃切除术。组织学检查结果显示为上皮样平滑肌肉瘤,伴有一些不利的微观因素。(摘要截选至250字)

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