Less than 200 cases are reported in the world literature. Our objective was to evaluate the ct features of desmoplastic small round cell tumor dsrct of the abdomen and pelvis in pediatric and young. Desmoplastic small round cell tumor dsrct is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of. Optimal multimodal treatment for desmoplastic small round cell. Fusion of the ews and wt1 genes in the desmoplastic small round cell tumor.
Desmoplastic small round cell tumors dsrcts are a rare soft tissue sarcoma 850 patients reported in medical literature by 20181 which predominantly occurs in male children and adolescents. Desmoplastic small round cell tumor dsrct is a type of cancer. Pdf desmoplastic small round cell tumor 20 years after. Desmoplastic small round cell tumor dsrct is a re cently recognized clinicopathologic entity that has a pre dilection for adolescent males and usually affects. Desmoplastic small round cell tumor dsrct by fernanda arnaldez, md and david loeb, md, phd also available in chinese, japanese, french, italian and spanish. Pdf intraabdominal desmoplastic small round cell tumor. Pdf desmoplastic small round cell tumor dsrct is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men.
Only a few hundred cases have been reported in worldwide literature. Current research is limited by the scarcity of this disease. It primarily affects children and young adults and is more common in males. Abstract desmoplastic small round cell tumor dsrct is a recently recognized clinicopathologic entity that has a predilection for adolescent males and usually. Desmoplastic small round cell tumor dsrct is an aggressive malignant neoplasm that occurs in adolescents and young adults.
It is usually found in the abdomen belly, but it can also occur in other parts of the body. Intraabdominal desmoplastic small round cell tumor. To the editor we read with great attention the article by tun et al describing a case of advanced desmoplastic small round cell tumor dsrct. Toomer a rare, aggressive cancer that usually affects young males and usually is located in the abdomen. Desmoplastic small round cell tumor dsrct is a rare malignancy most commonly originating in the peritoneum. Desmoplastic smallroundcell tumor dsrct is an uncommon type of malignancy, which primarily occurs in adolescent males and develops. Rosai in 1988 highly malignant mesenchymal neoplasm characterized by. Pdf abstract desmoplastic small round cell tumor dsrct was proposed as a distinct disease entity by william l gerald and juan rosai.
The tumor has a specific genetic abnormality that helps confirm the diagnosis. Ittypicallypresentswith multipleintraabdominaltumors and a distinctive desmoplastic stroma. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. Desmoplastic small round cell tumor genetic and rare. Dsrct is a rare and highly aggressive tumour that usually occurs in males during adolescence and early adulthood. Factors affecting overall survival os were assessed. Desmoplastic small round cell tumor in the abdomen and pelvis. Definition of desmoplastic small round cell tumor nci. Desmoplastic small round cell tumor dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen.
Desmoplastic small round cell tumor dsrct is a rare disease of children, adolescents and young adults, which begins in the abdominal cavity. Pdf desmoplastic small round cell tumor 20 years after its discovery. Desmoplastic small round cell tumor dsrct is a rare peritoneal surface malignancy. Desmoplastic small round cell tumor dsrct is a rare and aggressive mesenchymal malignancy. Background and objectives desmoplastic small round cell tumor dsrct is a rare peritoneal surface malignancy. Desmoplasia definition of desmoplasia by medical dictionary. Desmoplastic small round blue cell tumors dsrcts originate from a cell with multilineage potential. Desmoplastic small round cell tumor of parotid gland. Differential diagnoses include all the small round cells tumors. Pathologic insights of the desmoplastic small round cell. Desmoplastic small round cell tumor dsrct is a rare malignant mesenchymal neoplasm that typically affects young patients and presents as large. Pathologic insights of the desmoplastic small round cell tumor. It is formed by small, round cancer cells surrounded by scarlike tissue and is often found in the tissue peritoneum that lines the inside of the abdomen and pelvis.