Desmoplastic Small Round Cell Tumors- Case series of a Rare Tumor and Review of Literature
Desmoplastic small round cell tumor (DSRCT) is an uncommon and aggressive malignancy with male predominance. It commonly occurs in the abdominal cavity and is usually diagnosed with help of histopathology. It shows a characteristic EWSR1-WT1 gene fusion in most cases. We hereby report a series of 3...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
West Asia Organization for Cancer Prevention
2023-07-01
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| Series: | Asian Pacific Journal of Cancer Care |
| Subjects: | |
| Online Access: | http://www.waocp.com/journal/index.php/apjcc/article/view/1022 |
| _version_ | 1797342238915690496 |
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| author | Vallathol D. Harindran Sajna Vengasseri Moideenkutty Neeliyath Thazha Kuni Thanseer Parameswaran Prashanth Narayanankutty Warrier |
| author_facet | Vallathol D. Harindran Sajna Vengasseri Moideenkutty Neeliyath Thazha Kuni Thanseer Parameswaran Prashanth Narayanankutty Warrier |
| author_sort | Vallathol D. Harindran |
| collection | DOAJ |
| description | Desmoplastic small round cell tumor (DSRCT) is an uncommon and aggressive malignancy with male predominance. It commonly occurs in the abdominal cavity and is usually diagnosed with help of histopathology. It shows a characteristic EWSR1-WT1 gene fusion in most cases. We hereby report a series of 3 cases of DSRCT that we encountered at our institute. All of them presented in an advanced stage as quoted in the previously available literature. The ideal treatment should include a multidisciplinary team. The chemotherapy regimen used is usually Ewing’s sarcoma protocol of multiagent chemotherapy. Surgical approaches which include cytoreduction and intra-peritoneal hyperthermic chemotherapy are the procedure of choice but rarely feasible. Newer clinical trials of targeted therapy are in progress for improving the treatment and thereby the prognosis of DSRCT. |
| first_indexed | 2024-03-08T10:30:25Z |
| format | Article |
| id | doaj.art-5ae64f995a194620b176ffd01d06e3cd |
| institution | Directory Open Access Journal |
| issn | 2588-3682 |
| language | English |
| last_indexed | 2024-03-08T10:30:25Z |
| publishDate | 2023-07-01 |
| publisher | West Asia Organization for Cancer Prevention |
| record_format | Article |
| series | Asian Pacific Journal of Cancer Care |
| spelling | doaj.art-5ae64f995a194620b176ffd01d06e3cd2024-01-27T06:53:42ZengWest Asia Organization for Cancer PreventionAsian Pacific Journal of Cancer Care2588-36822023-07-018362763110.31557/apjcc.2023.8.3.627-6311022Desmoplastic Small Round Cell Tumors- Case series of a Rare Tumor and Review of LiteratureVallathol D. Harindran0Sajna Vengasseri Moideenkutty1Neeliyath Thazha Kuni Thanseer2Parameswaran Prashanth3Narayanankutty Warrier4Department of Medical Oncology, MVR Cancer Centre and Research Institute, Calicut, Kerala, India.Department of Medical Oncology, MVR Cancer Centre and Research Institute, Calicut, Kerala, India.Department of Medical Oncology, MVR Cancer Centre and Research Institute, Calicut, Kerala, India.Department of Medical Oncology, MVR Cancer Centre and Research Institute, Calicut, Kerala, India.Department of Medical Oncology, MVR Cancer Centre and Research Institute, Calicut, Kerala, India.Desmoplastic small round cell tumor (DSRCT) is an uncommon and aggressive malignancy with male predominance. It commonly occurs in the abdominal cavity and is usually diagnosed with help of histopathology. It shows a characteristic EWSR1-WT1 gene fusion in most cases. We hereby report a series of 3 cases of DSRCT that we encountered at our institute. All of them presented in an advanced stage as quoted in the previously available literature. The ideal treatment should include a multidisciplinary team. The chemotherapy regimen used is usually Ewing’s sarcoma protocol of multiagent chemotherapy. Surgical approaches which include cytoreduction and intra-peritoneal hyperthermic chemotherapy are the procedure of choice but rarely feasible. Newer clinical trials of targeted therapy are in progress for improving the treatment and thereby the prognosis of DSRCT.http://www.waocp.com/journal/index.php/apjcc/article/view/1022dsrct; aggressive; chemotherapy; advanced; multidisciplinary |
| spellingShingle | Vallathol D. Harindran Sajna Vengasseri Moideenkutty Neeliyath Thazha Kuni Thanseer Parameswaran Prashanth Narayanankutty Warrier Desmoplastic Small Round Cell Tumors- Case series of a Rare Tumor and Review of Literature Asian Pacific Journal of Cancer Care dsrct; aggressive; chemotherapy; advanced; multidisciplinary |
| title | Desmoplastic Small Round Cell Tumors- Case series of a Rare Tumor and Review of Literature |
| title_full | Desmoplastic Small Round Cell Tumors- Case series of a Rare Tumor and Review of Literature |
| title_fullStr | Desmoplastic Small Round Cell Tumors- Case series of a Rare Tumor and Review of Literature |
| title_full_unstemmed | Desmoplastic Small Round Cell Tumors- Case series of a Rare Tumor and Review of Literature |
| title_short | Desmoplastic Small Round Cell Tumors- Case series of a Rare Tumor and Review of Literature |
| title_sort | desmoplastic small round cell tumors case series of a rare tumor and review of literature |
| topic | dsrct; aggressive; chemotherapy; advanced; multidisciplinary |
| url | http://www.waocp.com/journal/index.php/apjcc/article/view/1022 |
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