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婴儿肌纤维瘤病:两例报告。

Infantile myofibromatosis: report of two cases.

作者信息

Sonoda T, Itami S, Seguchi S, Kurata S, Takayasu S

机构信息

Department of Dermatology, Oita Medical University, Japan.

出版信息

J Dermatol. 1994 Jul;21(7):508-13. doi: 10.1111/j.1346-8138.1994.tb01784.x.

Abstract

Two cases of solitary type infantile myofibromatosis (IM) were presented. Case 1 was a 6-month-old male infant with a firm, dark red, fixed tumor on his right knee. A computerized tomographic scan revealed that the tumor was attached to the underlying muscle. Case 2 was a 1-month-old male infant with a tumor on his right knee, similar to that in case 1. In neither case was there any clinical evidence of visceral involvement. Histological and immunohistochemical findings were similar. The lesions appeared histologically as non-encapsulated nodules composed of whorled fascicles of spindle-shaped cells and a vascular element with a hemangiopericytoma-like appearance. The tumor cells were positively stained with PTAH. They were positive for alpha-smooth muscle actin and vimentin, but negative for desmin. These findings support the myofibroblastic nature of IM. In case 2, the tumor regressed spontaneously at the age of 12 months. Unlike the multicentric form, spontaneous regression of the solitary form of IM has not previously been reported.

摘要

本文报告了两例孤立型婴儿肌纤维瘤病(IM)。病例1为一名6个月大的男婴,右膝有一个质地坚硬、暗红色、固定的肿瘤。计算机断层扫描显示肿瘤附着于深层肌肉。病例2为一名1个月大的男婴,右膝有一个与病例1相似的肿瘤。两例均无内脏受累的临床证据。组织学和免疫组化结果相似。病变在组织学上表现为非包膜性结节,由梭形细胞的漩涡状束和具有血管外皮细胞瘤样外观的血管成分组成。肿瘤细胞经磷钨酸苏木精(PTAH)染色呈阳性。它们对α-平滑肌肌动蛋白和波形蛋白呈阳性,但对结蛋白呈阴性。这些发现支持IM的肌成纤维细胞性质。在病例2中,肿瘤在12个月大时自发消退。与多中心型不同,孤立型IM的自发消退此前尚未见报道。

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