Primary spinal epitheliod angiosarcoma

Ibáñez, J.A. Contreras; Cueto, P. Muriel; Gómez, Clarisa Villegas

doi:10.1016/s1988-8856(10)70267-4

Cited by 1 publication

(1 citation statement)

References 9 publications

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“…Deshpande et al [6] refer the frequent delay in diagnosis or misdiagnosis. The EA histologically mimics poorly differentiated metastatic carcinoma [6], and frequently, the patient advancing age and multifocal lesions also contributes to an erroneous diagnosis [21,28]. Thus, immunohistochemical confirmation is essential and particularly the positivity for CD31, the most sensitive marker [6,19,27,29] expressed by 90 % of angiosarcomas of bone and less than 1 % of carcinomas [20,28]; CD34 is frequently positive but is less specific [19]; cytokeratin positivity is also higher and expression of endothelium antigens and factor VIII-related antigen have a variable expression [5,[18][19][20].…”

Section: Discussionmentioning

confidence: 99%

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

Oliveira

Rodrigues-Pinto

Costa

et al. 2014

Eur Orthop Traumatol

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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.

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