Primary epithelioid sarcoma of the oesophagus

Epithelioid sarcoma is a rare soft tissue tumour presenting two main variants: the 'classical' distal type and the more recently described proximal type. The latter is distinguished from the former by occurrence in elderly patients, more axial and deep location, prominent atypical and pleomorphic appearance and eventually aggressive clinical behaviour with poor outcome. To date, only few perivisceral epithelioid sarcomas have been described, and they are mainly related to the colon and bladder in pelvic and perineal sites. We report a hitherto undescribed epithelioid sarcoma of the distal oesophagus and discuss the relevance of molecular cytogenetics.