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先天性血管外皮细胞瘤/婴儿肌纤维瘤病:根治性手术与保守的“观察等待”方法对比

Congenital hemangiopericytoma/infantile myofibromatosis: radical surgery versus a conservative "wait and see" approach.

作者信息

Toren A, Perlman M, Polak-Charcon S, Avigad I, Katz M, Kuint Y, Rechavi G

机构信息

Pediatric Hemato/Oncology Department, Chaim Sheba Medical Center, Tel-Hashomer, Israel.

出版信息

Pediatr Hematol Oncol. 1997 Jul-Aug;14(4):387-93. doi: 10.3109/08880019709041599.

Abstract

Infantile/congenital hemangiopericytoma, although sharing many similar histological features with adult hemangiopericytoma, has a much better prognosis. Nevertheless, most cases described in the literature were pursued by radical surgery with or without adjuvant chemotherapy. We describe a neonate who presented with a huge mass in the right gluteus, 6 x 5 x 4 cm, and a small ventral abdominal mass. The masses were confirmed on biopsy according to light microscopy, immunohistochemistry, and electron microscopy as congenital hemangiopericytoma. They shrank spontaneously within 2 weeks and vanished within 2 months. We present a hypothesis that masses appearing in the neonatal period with this histology and with no life-endangering pressure on vital organs should routinely be dealt with conservatively.

摘要

婴儿期/先天性血管外皮细胞瘤虽然与成人血管外皮细胞瘤有许多相似的组织学特征,但其预后要好得多。然而,文献中描述的大多数病例都采用了根治性手术,辅以或不辅以化疗。我们报告一例新生儿,其右侧臀部有一个6×5×4厘米的巨大肿块,腹部腹侧还有一个小肿块。根据光镜、免疫组化和电镜检查,活检确诊这些肿块为先天性血管外皮细胞瘤。它们在2周内自行缩小,并在2个月内消失。我们提出一个假说,即新生儿期出现的具有这种组织学特征且对重要器官没有危及生命压力的肿块,应常规采取保守治疗。

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