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Journal of Case Reports
Metastasized Intraabdominal Desmoplastic Small Round Cell Tumor
Mariana Silva Costa1, Sara Pinto Lourenço1, Marta Filomena Guimarães1,2,3, Mário Caetano Nora1,2
1Department of General Surgery, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, Portugal; 2Endocrine, Cardiovascular & Metabolic Research, Unit for Multidisciplinary Research in Biomedicine (UMIB), University of Porto, Porto, Portugal; 3Department of Anatomy, Institute of Biomedical Sciences Abel Salazar (ICBAS), University of Porto, Porto, Portugal.
Corresponding Author:
Dr. Mariana da Silva Costa
Email: mariana87costa@gmail.com
Received: 07-AUG-2019 Accepted: 28-NOV-2019 Published Online: 30-DEC-2019
DOI: http://dx.doi.org/10.17659/01.2019.0071
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.
Keywords : Biopsy, Cytogenetic Analysis, Liver Neoplasm, Pain, Weight Loss.
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