Angiosarcomas are rare malignant vascular tumors that comprise only approximately 2% of all soft tissue sarcomas. They are mainly located in the head and neck area and spinal involvement is particularly very uncommon, reported only in occasional case reports or as single cases as part of a clinical series. There is therefore limited data in the literature regarding the optimum treatment strategy. Due to the aggressive nature of these lesions total resection is challenging and the overall survival time is short. In this report the authors report the successful management of a 37-year-old male harbouring spinal angiosarcoma involving the L2 level via en-bloc resection and adjuvant radiotherapy. The patient is disease-free at the 4th year follow-up and the radiological investigations are without the evidence of local recurrence, metastasis or implant failure. En bloc resection of spinal angiosarcomas can significantly improve survival and the surgical treatment should aim for this whenever possible.
[Show abstract][Hide abstract] ABSTRACT: Epithelioid angiosarcoma (EA) of bone is a very uncommon high-grade sarcoma with an unpredictable clinical course, high metastasis rate and poor prognosis. It is extremely rare in the spine. Delayed or missed diagnosis is frequent, and histological and immunohistochemical confirmation of diagnosis is essential. Only few cases of epithelioid angiosarcoma of the spine are described. Recommended treatment is wide en bloc resection with or without adjuvants. We first performed a systematic review and included eight papers on vertebral EA (from 48 titles). In two cases, a total en bloc resection with adjuvant therapy and surgical reconstruction procedure was performed but only one had successful safe margins. After palliative radiotherapy alone, one patient died. Complications are associated to their vascular nature. Second, we present a rare case of EA at the third lumbar vertebra in a 68-year-old male. L3 vertebrectomy and reconstruction was performed through a posterior approach. Histological and immunohistochemical analysis of resected tumor confirmed high-grade EA. Prognosis was extremely poor due to paravertebral extension of this malignant aggressive tumor. The patient did not recover motor deficits. He presented a nosocomial Pseudomonas aeruginosa sepsis originated in urinary tract infection and died 1 month postoperatively. In literature, only few spinal EA are described. Especially in the spine, it represents a diagnostic and surgical challenge. Survival rate of these tumors is unknown but an early diagnosis is essential to allow a successful wide resection.
European Orthopaedics and Traumatology 09/2014; 6(2). DOI:10.1007/s12570-014-0276-9
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