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一例侵袭性骨假肌源性血管内皮瘤伴病理性骨折及快速进展性肺转移疾病:病例报告及文献复习

An aggressive case of pseudomyogenic haemangioendothelioma of bone with pathological fracture and rapidly progressive pulmonary metastatic disease: case report and review of the literature.

作者信息

Shah Asad Rabbani, Fernando Malee, Musson Rachel, Kotnis Nikhil

机构信息

Department of Medical Imaging and Medical Physics, Sheffield Teaching Hospitals, Sheffield, UK.

出版信息

Skeletal Radiol. 2015 Sep;44(9):1381-6. doi: 10.1007/s00256-015-2168-7. Epub 2015 May 17.

Abstract

Pseudomyogenic haemangioendothelioma (PMH) is a rare recently described vascular tumour typically presenting with soft tissue disease in distal extremities of young adults. Multi-focal and multi-layered involvement is commonly recognised. The majority of cases described so far have shown an indolent clinical course and distant metastatic spread is rare. We report a case of PMH in an 82-year-old male diagnosed following a pathological fracture of the distal tibia. Further bone lesions were identified in the fibula, patella and distal femur. The patient was found to have multiple nodules suspicious for pulmonary metastases on a CT scan at the time of diagnosis that showed significant progression at a follow-up scan 4 weeks later. To our knowledge, this is the first reported case of PMH presenting with a pathological fracture. The rapid progression of bone and distant metastatic disease in this case is highly unusual given the typically indolent clinical course reported in the literature to date.

摘要

假肌源性血管内皮瘤(PMH)是一种最近才被描述的罕见血管肿瘤,典型表现为年轻成年人远端肢体的软组织疾病。多灶性和多层受累较为常见。迄今为止描述的大多数病例临床过程较为惰性,远处转移很少见。我们报告一例82岁男性的PMH,该病例是在胫骨远端病理性骨折后确诊的。在腓骨、髌骨和股骨远端发现了更多骨病变。诊断时CT扫描发现患者有多个可疑肺转移结节,4周后的随访扫描显示有明显进展。据我们所知,这是首例报道的以病理性骨折为表现的PMH病例。鉴于迄今为止文献报道的典型惰性临床过程,该病例中骨和远处转移疾病的快速进展非常罕见。

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