Intra-abdominal desmoplastic small round cell tumor in a 45-year-old man: a case report

Intra-abdominal desmoplastic small round cell tumor is a rare entity with a few reports worldwide. This tumor commonly occurs in children and adolescent and occurrence in adult age group is very rare. Here, we describe an adult male with symptoms of intestinal obstruction due to abdominal mass, located in splenic flexure. After resection, the diagnosis of intra-abdominal desmoplastic small round cell tumor was established by pathologic and immunohistochemical studies. We emphasize that albeit being rare, this tumor should be mentioned as one of the differential diagnoses of widespread intra-abdominal lesions in any age group. A brief review of epidemiology, clinical manifestations as well as pathological and molecular features is also included in the paper.

Tumor desmoplástico de pequenas células: relato de caso e revisão da literatura / Desmoplastic small round cell tumor: a case report and review of the literature

O tumor desmoplástico de pequenas células é uma neoplasia rara e extremamente agressiva que acomete predominantemente jovens do sexo masculino. Dor e distensão abdominal são freqüentes, ascite pode ser observada. Sua histogênese é incerta e a cavidade peritoneal é o local maiscomum de sua ocorrência. Este tumor é caracterizado por apresentar morfologia distinta e diferenciação multifenotípica. Apresentamos, neste relato, o caso de um adolescente admitido no serviçode Medicina Interna e Radiologia do Hospital e Maternidade Marieta Konder Bornhausen, Itajaí, SC, em 2005.

Desmoplastic small round cell tumor is a rare and highly aggressive neoplasm that predominantly occurs in young adult males. Pain and abdominal distention are frequent, ascites could be observed. His histogenesis is uncertain and the most common location is the peritoneal cavity. This tumor is characterized bypresenting a distinct morphology and polyphenotypic differentiation. We present in this report the case of an adolescent admitted in the service of Internal Medicine and Radiology of the Hospital and Maternity Marieta Konder Bornhausen, Itajaí, SC, Brazil,in year 2005.