Desmoplastic small round cell tumor dsrct is a highly aggressive and rare mesenchymal tumor, around 200450 cases have been so described so far 1,2 despite aggressive therapy, median survival ranges from 17 to 25 month2,8 a 5year survival rate remains around 15%8 with higher survival reported among. It affects mainly children and young adults and has a male predilection. Desmoplastic small round cell tumor genetic and rare. It is usually found in the abdomen belly, but it can also occur in other parts of the body. Desmoplastic small round blue cell tumors dsrcts originate from a cell with multilineage potential. Over 850 patients have been reported in the medical. It primarily affects children and young adults and is more common in males. The pathogenesis or histogenesis of dsrct is uncertain. Desmoplastic smallroundcell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen.
A 14yearold girl presented with intermittent epigastric pain. Desmoplastic small round cell tumor is a rare malignant neoplasm that usually occurs in adolescents and young adult males and typically presents as a large, often multinodular, intraabdominal mass. The 3 and 5year survival rates of patients with dsrct were reported to be 44% and 15%, respectively. Desmoplastic small round cell tumor dsrct is a rare and highly aggressive variety of sarcoma arising typically from abdominal or pelvic peritoneum. Patients typically present with symptoms of abdominal sarcomatosis. Desmoplastic small round cell tumor dsrct is a type of cancer.
Some of the cells showed round pink intracytoplasmic inclusions fig 1b. Desmoplastic small round cell tumor is a tumor composed of small round cells associated with marked desmoplasia and multiphenotypic differentiation. Desmoplastic small round cell peditric ii tumor of the kidney. A molecular hallmark of dsrct is the ewswt1 reciprocal translocation. To the editor we read with great attention the article by tun et al1 describing a case of advanced desmoplastic small round cell tumor dsrct and discussing its optimal management. Shipley sarcoma molecular pathology team, divisions of molecular pathology and cancer therapeutics, the institute of cancer research, london, uk. A case of intraabdominal desmoplastic smallroundcell. Desmoplastic small roundcell tumor is an uncommon, highly aggressive tumor with a predilection for pediatric age groups and young adults. Desmoplastic small round cell tumor of the abdomen and. Desmoplastic small round cell tumor dsrct was first described by gerald and rosai in 19891 as a distinct tumor with specific histologic and immunohistochemical patterns and chromosomal translocation. Desmoplastic small round cell tumor of the parotid gland. It is an aggressive and often misdiagnosed neoplasm of children and young adult. Desmoplastic small round cell tumor dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen. Dsrct belongs to a group of undifferentiated small round cell tumors, which include ewing sarcoma primitive peripheral neuroectodermal tumor pnetaskins tumor and rhabdomyosarcoma.
Ewing sarcoma and dsrct are treated similarly due to similar oncogene activation pathways, and dsrct has been represented in very limited numbers in sarcoma studies. Desmoplastic smallroundcell tumor dsrct is an extremely rare and highly aggressive malignancy that was first described in 1987 by sesterhenn et al. The most common site is the abdominal serosa, although other sites have been described. It usually happens in the tissue that lines the inside of the belly. Desmoplastic small roundcell tumor of the pancreas. Pdf desmoplastic small round cell tumor of the ovary. Dsrct is a rare and highly aggressive tumour that usually occurs in males during adolescence and early adulthood. A 49yearold japanese man noticed a mass in the right parotid gland. Only 850 such patients were reported in the medical literature.
Desmoplastic small round cell tumors occur most often in young men and boys, though they can occur at any age and can also affect women. It is known to involve the formation of fusion protein of ewings sarcoma genes and wilms tumor genes, and it. Desmoplastic small round cell tumor dsrct is an extremely rare, highly aggressive, and malignant neoplasm initially reported by gerald and rosai in 1989. They quickly spread to other structures within the abdomen. The purpose of this study is to evaluate the clinical, pathologic, and multimodality crosssectional imaging features of a cohort of 94 patients with desmoplastic small round cell tumor dsrct.
Light microscopic examination confirmed a malignant small roundcell tumor composed of irregular nests of small round cells set in a desmoplastic stroma. To remove the cells, you might need surgery or a needle can be passed through the skin. Desmoplastic small round cell tumor is a rare aggressive neoplasm, often presenting in young adult males. Desmoplastic small round cell tumor dsrct is a rare aggressive tumor primarily involving serosal surfaces in adolescents and young men. Desmoplastic small round cell tumor dsrct is a rare soft tissue tumor that generally involves the retroperitoneum, pelvis, omentum and mesentery in younger patients. Desmoplastic small round cell tumors dsrct national. Orbital desmoplastic small round cell tumor in an infant amy huang, bs,a and nishita patel, md b auniversity of central florida college of medicine, orlando. Pdf desmoplastic small round cell tumor 20 years after. A high serum ca 125 level may be a specific marker for dsrct, and thus may permit early diagnosis and treatment of this fastgrowing tumor. Dsrct primarily occurs in adolescents and young adults between the ages of 15 and 25 years and has a male predominance. 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.
Desmoplastic small round cell tumor dsrct is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men adults. Orbital desmoplastic small round cell tumor in an infant. Current research is limited by the scarcity of this disease. Diagnosis is based on histological analysis of biopsies which typically show small round blue cells in nests separated by an abundant desmoplastic.
Learn more about how this cancer forms, is treated, and the prognosis. Factors affecting overall survival os were assessed. First described in 1989 2, 3, dsrcts name derives from its distinctive histological findings, which include clusters of undifferentiated, small round blue cells surrounded by abundant desmoplasia. Optimal multimodal treatment for desmoplastic small round. Desmoplastic small round cell tumor dsrct home desmoplastic small round cell tumor dsrct university of north carolina childrens hospital. Establishment and characterization of a novel human. Desmoplastic small round cell tumor dsrct is a rare, biologically aggressive, multifocal primary peritoneal sarcoma that predominantly affects white adolescent boys and young adult men. Primary desmoplastic smallroundcell tumor of the ovary. Imaging of desmoplastic small round cell tumour in adults. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue.
Pdf intraabdominal desmoplastic small round cell tumor. Desmoplastic smallroundcell tumor dsrct is an uncommon and aggressive malignancy with a poor prognosis, which is predominantly diagnosed in adolescent males 1,2. The desmoplastic small round cell tumor dsrct is a relatively rare malignant tumor that typically involves the abdominal andor pelvic peritoneum of children and young adults weiss and goldblum. Abstract desmoplastic small round cell tumor dsrct was proposed as a distinct disease entity by william l gerald and juan rosai in 1991. Primary desmoplastic small round cell tumor of urinary.
Desmoplastic small round cell tumors of the pleura. Diagnosis is based on histologic analysis of biopsy and cytogenetic studies. This cancer happens when abnormal cells form in the soft tissues. The peritoneal cavity is the most common location of the tumor with multiple satellite nodules often noted at presentation. Desmoplastic small round cell tumor of the central nervous system. Desmoplastic small round cell tumor dsrct varied presentation. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Primary desmoplastic small round cell tumor of urinary bladder a22yearoldmanpresentedwithintermittentgrosshematuria and pain in the lower abdomen for 8 days. Desmoplastic small round cell tumor with ovarian involvement. Desmoplastic small round cell tumor of the pleura is a rare malignancy, with only a few cases reported in the scientific literature. It is characterized by a distinct immunohistochemical profile and a recurrent, specific, chromosomal translocation. These aggressive cancers often form as multiple tumors in the tissue peritoneum that lines the inside of the abdomen and pelvis. Over time, numerous approaches have been proposed for the treatment of dsrct, and the best results have been obtained with the. Dsrct is very rare and most commonly occurs in white males between the ages of 10 and 30 years old.
Desmoplastic small round cell tumor dsrct the brett. Adolescent males are primarily affected with a typically abdominal or pelvic mass. The desmoplastic small round cell tumor dsrct is an extremely rare tumor that mainly affects adolescents and mostly involves the abdominal and pelvic peritoneum. Irinotecan and vincristine for the treatment of refractory. Desmoplastic small round cell tumour dsrct belongs to the histological descriptive category of small round blue cell tumours. Desmoplastic small round cell tumors diagnosis and.
Desmoplastic small round cell tumor dsrct is a rare and aggressive mesenchymal malignancy. Then, it was more elaborately described by gerald and rosai in 1989. Desmoplastic small round cell tumors are diagnosed with a procedure to remove a sample of cells for testing. A desmoplastic small round cell tumor dsrct is an extremely rare and highly aggressive cancer that affects adolescents and young adults. Desmoplastic small round cell tumor dsrct is a rare peritoneal surface malignancy. Pdf a new molecular variant of desmoplastic small round. Although we agree with the authors1 on the fact that data are scarce on the clinical management of advanced dsrct, we. Desmoplastic small round cell tumors dsrct are tumors that grow in the abdomen and pelvic area of the body. The tumor usually begins and spreads over the surface of the peritoneum 4, 12, 15, 16. For example, desmin, long considered a marker of rhabdomyosarcoma, is also expressed in a subset of desmoplastic. Desmoplastic small round cell tumor dsrct is an extremely rare, highly aggressive, malignant tumor of undetermined histogenesis. Desmoplastic small round cell tumor is a rare malignant neoplasm. The tumor has a specific genetic abnormality that helps confirm the diagnosis. Desmoplastic small round cell tumor is a rare malignant tumor that has a poor prognosis.
Ultrasound examination revealed a solid tumor about 2 cm in diameter. Primary desmoplastic small round cell tumor of the. Desmoplastic small round cell tumor in transverse colon. Although agent orange has been associated with softtissue sarcoma, an association with desmoplastic small roundcell tumor has not been reported. In rare cases, the tumors can also be found in other sites such as pleural cavity, pelvis, bone, and head and neck region. Desmoplastic small round cell tumor is a rare tumor that was reported by gerald and rosai 1 in 1989, and it is said to be related to primitive neuroectodermal tumor. Desmoplastic small round cell tumors symptoms and causes. Desmoplastic small round cell tumor dsrct is a rare aggressive neoplasm that is typically composed of clusters of small round cells within the desmoplastic stroma.
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