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梭形细胞血管瘤病的特征性表现,常被误诊为静脉畸形:11例回顾性研究

Characteristic appearance of spindle cell hemangiomatosis, often misdiagnosed as venous malformation: A retrospective study of 11 cases.

作者信息

Liu Hongyuan, Rao Yamin, Gu Hao, Yang Xi, Hu Li, Sun Yi, Chen Hui, Lin Xiaoxi

机构信息

Departments of, Department of, Plastic and Reconstructive Surgery, School of Medicine, Shanghai Ninth People's Hospital, Shanghai Jiaotong University, Shanghai, China.

Department of, Pathology, School of Medicine, Shanghai Ninth People's Hospital, Shanghai Jiaotong University, Shanghai, China.

出版信息

J Dermatol. 2020 Dec;47(12):1424-1431. doi: 10.1111/1346-8138.15571. Epub 2020 Sep 29.

DOI:10.1111/1346-8138.15571
PMID:32991744
Abstract

Spindle cell hemangiomatosis is a benign multifocal vascular proliferation that mostly occurs in the distal extremities. It is a relatively rare disease and causes difficulties in clinical diagnosis and differential diagnosis of venous malformation. We retrospectively assessed the medical history, and clinical features, imaging features, pathological features and follow up of 11 patients diagnosed pathologically with spindle cell hemangiomatosis after surgery. There are two types of clinical appearances in spindle cell hemangiomatosis in the distal extremities: bleb-like nodules and varix-like nodules. Bleb-like nodules are mostly superficially located in the palm or interphalangeal joints with obvious hemorrhage in lesions and mainly composed of cavernous spaces rather than spindle cells. Varix-like nodules are located in the back of the hands or arms with normal skin color and mainly composed of solid areas of accumulated spindle cells. Surgery is the standard therapy for spindle cell hemangiomatosis, while sclerotherapy is invalid. Despite the tendency to develop new lesions, there are no residual lesions at the surgical site during follow up. Bleb-like nodules in the palm and interphalangeal joints are a typical clinical appearance in spindle cell hemangiomatosis which can help make clinical and a differential diagnosis of venous malformation. Surgery is the standard therapy for spindle cell hemangiomatosis with no residual lesions remaining at the surgical sites.

摘要

梭形细胞血管瘤病是一种良性多灶性血管增生性疾病,主要发生于四肢远端。它是一种相对罕见的疾病,在临床诊断及与静脉畸形的鉴别诊断方面存在困难。我们回顾性评估了11例经手术病理诊断为梭形细胞血管瘤病患者的病史、临床特征、影像学特征、病理特征及随访情况。四肢远端的梭形细胞血管瘤病有两种临床表现:水疱样结节和静脉曲张样结节。水疱样结节多浅表位于手掌或指间关节,病变内有明显出血,主要由海绵状腔隙而非梭形细胞构成。静脉曲张样结节位于手背或手臂,皮肤颜色正常,主要由聚集的梭形细胞实性区域构成。手术是梭形细胞血管瘤病的标准治疗方法,而硬化治疗无效。尽管有出现新病变的倾向,但随访期间手术部位无残留病变。手掌和指间关节处的水疱样结节是梭形细胞血管瘤病的典型临床表现,有助于对静脉畸形进行临床及鉴别诊断。手术是梭形细胞血管瘤病的标准治疗方法,手术部位无残留病变。

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