Primary sclerosing epithelioid XXXX of the sacrum: a case report and review of the literature.

@entity1 @entity5 @entity1492 @entity1463 @entity158 @entity3
@entity1463 epithelioid @entity1492 is a rare @entity5 characterised histologically by a predominant population of epithelioid cells arranged in strands and nests, embedded in a fibrotic and hyalinised stroma. It is a low grade @entity5 with an indolent course. A 48 year old @entity1 presented with a @entity158 her back for six months. Investigation and biopsy revealed features of sclerosing epithelioid @entity1492 involving the left half of the sacrum, left sacro-iliac joint, and posterior part of the left ilium. Preoperative radiotherapy and wide location excision of the @entity5 were followed by metastatic recurrence of the @entity5 in the lung and scalp six years after initial presentation. The @entity5 showed typical histology of sclerosing epithelioid @entity1492 . The radiological features confirmed its primary location in the sacrum. The @entity1 declined chemotherapy and died of disseminated disease eight years after initial presentation. Review of the literature confirms the fact that sclerosing epithelioid @entity1492 , despite its low grade, is a clinicopathologically distinct @entity5 with full malignant potential, the recurrence, @entity3 , and mortality rate being 48%, 60%, and 35%, respectively. @entity1463 epithelioid @entity1492 can occur as a primary @entity5 , the clinical behaviour of which is probably similar to its soft tissue counterpart.
