Adamantinoma-like Ewing Sarcoma: A Report of Two Rare Cases in the Pancreatic Tail and Spine

Abstract

Adamantinoma-like Ewing sarcoma (ALES), an extremely rare variant of Ewings sarcoma occurs primarily in the head and neck sites. ALES is characterized by basaloid cytomorphology, immunohistochemically expresses keratins, p63, p40, CD99, and harbors t (11; 22) EWSR1::FLI1 translocation on fluorescence in situ hybridization (FISH). The current case report discusses two cases of ALES, one in the pancreatic tail of a 64-year-old male and the second in the lumbar spine of an 18 yr old male. The IHC for both cases showed similar picture, confirming ALES through Fluorescence in situ hybridization (FISH), which indicated EWSR1 gene rearrangement. ALES being relatively rare and aggressive, diagnosis and the treatment poses significant challenges. Diagnosis relies on morphological, immunohistochemical, and molecular findings. The diagnostic findings helped in establishing a diagnosis of sarcoma which could have easily been misdiagnosed as a carcinoma,thus aiding appropriate selection of therapeutic strategies. Due to its rarity, further research is necessary to improve diagnostic accuracy and treatment outcomes.