Demsoplastic Small Round Cell Tumor: a Diagnostic and Therapeutic Dilemma
Moeinadin Safavi1, Jahanbanoo Shahryari1, Mohammad Mehdi Moeini Aghtaei2
1Dept. of Pathology, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran; Molecular Pathology and Cytogenetic Unit, Pathology Department, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
Abstract:
Desmoplastic small round cell tumor (DSCRT) is a rare variant of sarcoma with a highly aggressive behavior. It usually affects abdominal cavity and has a male predominance. Its correct diagnosis and treatment is sophisticated and requires an experienced multidisciplinary team. Hereby we present a 25 yr old man from Kerman Province in 2013 with abdominal mass and ascites who underwent sonography guided percutaneous needle biopsy which was misleading and inconclusive for diagnosis. Thus an open biopsy was fulfilled which revealed solid nests of small round cells with hyperchromatic nuclei and clear cytoplasm surrounded by a desmoplastic stroma suggestive for DSCRT. The diagnosis was confirmed by positive immunohitochemical reaction for cytokeratin, desmin and neuron specific enolase (NSE).Ultimately the patient underwent chemotherapy on the basis of P6 protocol without surgical debulking. Diagnosis and treatment of DSCRT could be a dilemma due to its rarity, various clinicopathologic mimickers and lack of a consensus about its management.
