Metastasized Intraabdominal Desmoplastic Small Round Cell Tumor

Journal of Case Reports

article

Mariana Silva Costa¹  Sara Pinto Lourenço¹  Marta Filomena Guimarães¹  Mário Caetano Nora¹ 

[1] Department of General Surgery, Centro Hospitalar de Entre o Douro e Vouga;Endocrine, Cardiovascular & Metabolic Research, Unit for Multidisciplinary Research in Biomedicine ,(UMIB), University of Porto;Department of Anatomy, Institute of Biomedical Sciences Abel Salazar ,(ICBAS), University of Porto

关键词: Biopsy;    Cytogenetic Analysis;    Liver Neoplasm;    Pain;    Weight Loss.;   

DOI  :  10.17659/01.2019.0071

学科分类：生理学

来源: Journal of Case Reports

【 摘 要 】

Background: The desmoplastic small round cell tumor is a rare disease with poor prognosis. It affects predominantly young males with preferential involvement of the abdominal cavity. Case Report: A 33 year old male presented with right hypochondrium pain, associated with anorexia and weight loss for last 2 months. The imaging studies identified an abdominal mass, without hollow organ association, and multiple liver metastases. The anatomopathological study of the tissue obtained with the percutaneous abdominal mass biopsy confirmed the presence of desmoplastic small round and blue tumor cells. The immunohistochemical and cytogenetic study confirmed the diagnosis. It was proposed for systemic chemotherapy as primary treatment. Conclusion: We intend to emphasize that although this tumor is rare, it should be included in the differential diagnosis of intra-abdominal solid lesions. It also includes a review of the epidemiology, clinical manifestations, as well molecular and pathologic features.

【 授权许可】

Unknown