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儿童胃平滑肌瘤(上皮样平滑肌瘤):一例报告

Gastric leiomyoblastoma (epithelioid leiomyoma) occurring in a child: a case report.

作者信息

Rogers B B, Grishaber J E, Mahoney D H, McGill C W, Wagner M L

机构信息

Department of Pathology, Texas Children's Hospital, Houston.

出版信息

Pediatr Pathol. 1989;9(1):79-85. doi: 10.3109/15513818909022334.

Abstract

Leiomyoblastomas are rare smooth muscle tumors occurring most commonly in the gastrointestinal tract of adults. We report the unusual occurrence of a gastric leiomyoblastoma in a child who presented with refractory iron deficiency anemia. The tumor had a superficial erosion which was apparently the source of intermittent hemorrhage resulting in anemia. Epithelioid cells, spindled cells, and cells arranged in a perithelial pattern were seen by microscopy, characteristic of a leiomyoblastoma. Immunohistochemical staining demonstrated muscle actin, vimentin, desmin, and alpha-1-antichymotrypsin, and electron microscopy showed focal densities alternating with thin filaments. All of these features help to differentiate the tumor from an inflammatory pseudotumor. Leiomyoblastomas are associated with paragangliomas and pulmonary chondromas in children, but these were not present in this child. The prognosis of leiomyoblastomas may be related to tumor size, mitotic rate, and cellular characteristics.

摘要

平滑肌瘤是一种罕见的平滑肌肿瘤,最常见于成人胃肠道。我们报告了一例发生在儿童的胃平滑肌瘤,该患儿表现为难治性缺铁性贫血。肿瘤有一处浅表糜烂,显然是导致贫血的间歇性出血的来源。显微镜下可见上皮样细胞、梭形细胞以及呈血管周样排列的细胞,这是平滑肌瘤的特征。免疫组化染色显示肌动蛋白、波形蛋白、结蛋白和α-1-抗糜蛋白酶阳性,电子显微镜显示局灶性致密物质与细丝交替出现。所有这些特征有助于将该肿瘤与炎性假瘤区分开来。儿童平滑肌瘤与副神经节瘤和肺软骨瘤有关,但该患儿未出现这些情况。平滑肌瘤的预后可能与肿瘤大小、有丝分裂率和细胞特征有关。

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