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先天性面部浸润性脂肪瘤病:临床病理评估与治疗

Congenital infiltrating lipomatosis of the face: clinicopathologic evaluation and treatment.

作者信息

Slavin S A, Baker D C, McCarthy J G, Mufarrij A

出版信息

Plast Reconstr Surg. 1983 Aug;72(2):158-64. doi: 10.1097/00006534-198308000-00006.

Abstract

Congenital lipomatosis of the face is characterized by collections of nonencapsulated, mature lipocytes which infiltrate local tissues and tend to recur after surgery. These lesions represent a distinct clinicopathologic entity that has not been previously reported in this location in children. Three children with congenital lipomatosis of the face were treated at the Institute of Reconstructive Plastic Surgery over a 2- to 14-year follow-up period. In each instance, pathologic evaluation by light and electron microscopy revealed similar lesions sharing the following morphologic criteria: (1) nonencapsulated tumors containing mature fat cells, (2) infiltration of adjacent muscle and soft tissue, (3) absence of malignant characteristics, (4) absence of lipoblasts, (5) presence of fibrous elements in conjunction with increased numbers of nerve bundles and vessels, and (6) hypertrophy of subjacent bone. All three lesions recurred after numerous excisions, some of which were extensive. All were benign by histologic examination and remained so for as long as 14 years. Surgical treatment improved the aesthetic appearance of each child despite evidence of tumor persistence. Although these tumors are benign, we recommend an early aggressive surgical approach to control the infiltrative nature of their growth and to improve facial appearance.

摘要

先天性面部脂肪瘤病的特征是存在非包膜性的成熟脂肪细胞聚集,这些细胞浸润局部组织,术后易于复发。这些病变代表一种独特的临床病理实体,此前在儿童的该部位尚未见报道。在重建整形外科研究所,对3例先天性面部脂肪瘤病患儿进行了2至14年的随访治疗。在每例病例中,通过光镜和电镜进行的病理评估显示出相似的病变,具有以下形态学标准:(1)含有成熟脂肪细胞的非包膜性肿瘤;(2)浸润相邻肌肉和软组织;(3)无恶性特征;(4)无脂肪母细胞;(5)存在纤维成分,同时神经束和血管数量增加;(6)下方骨肥大。所有3个病变在多次切除后均复发,其中一些切除范围广泛。经组织学检查,所有病变均为良性,且长达14年一直保持良性。尽管有肿瘤持续存在的证据,但手术治疗改善了每个患儿的美观。虽然这些肿瘤是良性的,但我们建议早期采取积极的手术方法,以控制其生长的浸润性并改善面部外观。

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