Proximal type of epithelioid sarcoma: a rare aggressive tumor presenting simultaneously in spine and pelvis

ABSTRACT

A 40-year-old female presented with bilateral lower limb weakness with bladder and bowel incontinence. MRI study revealed a destructive lesion involving the D7 vertebral body and a large tumor in the gluteal muscles invading the right iliac blade. A histological examination demonstrated a tumor comprising of rounded to ovoid pleomorphic epithelioid cells with marked cytological atypia. Tumor cells expressed CD 34, vimentin and focally pancytokeratin but were negative for CD31, EMA, SMA, WT1 and LCA. A D6-7 laminectomy with posterior decompression was done. Postoperatively, external beam radiotherapy was given. However, the patient deteriorated rapidly with no neurological improvement. Epitheiliod sarcomas and their recently described proximal variant, by virtue of being an exceedingly unusual tumor are often misdiagnosed or diagnosed late beyond the stage of salvage. This report highlights the histopathology and that need to be analyzed to correctly diagnose this entity