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手部软组织血管平滑肌瘤作为血友病性假肿瘤的一种罕见鉴别诊断。

Soft-tissue angioleiomyoma of the hand as a rare differential diagnosis of haemophilic pseudotumour.

作者信息

Gassel F, Kraft C N, Wallny T, Hess L, Schmitt O

机构信息

Department of Orthopaedic Surgery, University of Bonn, Bonn, Germany.

出版信息

Haemophilia. 2001 Sep;7(5):528-31. doi: 10.1046/j.1365-2516.2001.00542.x.

Abstract

The benign vascular tumours known as angioleiomyomas, which originate from smooth muscle cells of arterial or venous walls are rare, particularly in the region of the hand. Frequently, the diagnosis is only made after surgical extirpation and histological assessment. The occurrence of an angioleiomyoma in a haemophiliac has not yet been reported, to our knowledge. A 62-year-old patient with a haemophilia B complained of increasing pain in the vicinity of the soft tissue covering the carpo-metacarpophalangeal joint of his left palm. In the T1-weighted magnetic resonance images a hypointense well-demarcated mass was found, showing a homogeneous enhancement after intravenous application of contrast medium. T2-weighted images showed a hyperintense signal. Based on clinical and radiographic findings, the tumour was initially thought to be a haemophilic pseudotumour arising from a prior local haematoma. Intraoperatively, the mass was found to be solid, and histological assessment diagnosed an angioleiomyoma, without signs of malignant transformation. Descriptions of the clinical symptoms of angioleiomyoma in an extremity vary considerably in the literature. Although rare, the vascular leiomyoma should therefore be contemplated in the differential diagnosis in patients with a solid nodular lesion of unclear aetiology in arms or legs. Especially in a haemophiliac patient, the growth can easily be mistaken for a pseudotumour because of its clinical and radiological similarities.

摘要

血管平滑肌瘤是一种良性血管肿瘤,起源于动脉或静脉壁的平滑肌细胞,较为罕见,尤其是在手部区域。通常,只有在手术切除和组织学评估后才能做出诊断。据我们所知,血友病患者发生血管平滑肌瘤的情况尚未见报道。一名62岁的乙型血友病患者主诉其左手掌腕掌指关节覆盖的软组织附近疼痛加剧。在T1加权磁共振图像中发现一个边界清晰的低信号肿块,静脉注射造影剂后显示均匀强化。T2加权图像显示高信号。根据临床和影像学表现,该肿瘤最初被认为是由先前局部血肿引起的血友病性假肿瘤。术中发现肿块为实性,组织学评估诊断为血管平滑肌瘤,无恶性转化迹象。文献中关于肢体血管平滑肌瘤临床症状的描述差异很大。因此,对于手臂或腿部病因不明的实性结节性病变患者,在鉴别诊断时应考虑血管平滑肌瘤,尽管其罕见。特别是在血友病患者中,由于其临床和影像学表现相似,其生长很容易被误诊为假肿瘤。

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