Key result
Pelvic bone epithelioid angiosarcoma progresses rapidly to fatal metastases within four months.
Why the study?
Primitive bone epithelioid angiosarcoma is an extremely rare and aggressive vascular tumour with poor short-term prognosis, requiring detailed diagnostic and therapeutic evaluation.
Case Report (n=1)
No
Pelvic multifocal primitive bone epithelioid angiosarcoma is an extremely rare and aggressive tumor with a rapidly fatal prognosis.
Multifocal pelvic bone epithelioid angiosarcoma in a young adult warrants histologic vigilance for aggressive osteolytic lesions; leaves optimal therapy undefined.
Epithelioid angiosarcoma is a rare vascular tumour with dark prognosis at short term. The primitive bone localization is exceptional and sometimes presents itself under a unifocal form. We report a clinical case of pelvic multifocal primitive bone epithelioid angiosarcoma (iliac crest and hip) diagnosed on a young male adult (45 years old). The radiological investigations put in evidence geographic osteolytic lesions (type IC) and, diagnostic was given thanks to the histological examination of the surgical biopsy pieces. The evolution was marked by the appearance of pulmonary and lymph node metastases with a death occurring in less than 4 months. Extremely rare tumour, bone epithelioid angiosarcoma is a variant of angiosarcoma characterised by an aggressive nature whose evolution is always fatal. Through this observation we report our experience on the diagnostic approach; and using a literature review we discuss the therapeutic modalities of this tumour with very bad prognosis.
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Koné et al. (2017) conducted a case report in Pelvic multifocal primitive bone epithelioid angiosarcoma (n=1). Surgical biopsy and provisional spacer filling was evaluated on Clinical outcome. A 45-year-old male diagnosed with pelvic multifocal primitive bone epithelioid angiosarcoma experienced rapid disease progression and died from pulmonary and ganglionic metastases within 4 months.
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