Primary vertebral epithelioid angiossarcoma—a rare case report and systematic literature review

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.