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Desmoplastic small round cell tumor of the abdomen: A case report and literature review of therapeutic options
Department of medical oncology, Hassan II University Hospital, Fez, Morocco
Department of pathology, Hassan II University Hospital, Fez, Morocco
Department of general surgery, Hassan II University Hospital, Fez, Morocco
Department of general surgery, Hassan II University Hospital, Fez, Morocco
Department of radiology, Hassan II University Hospital, Fez, Morocco
Department of pathology, Hassan II University Hospital, Fez, Morocco
Department of medical oncology, Hassan II University Hospital, Fez, Morocco
Department of medical oncology, Hassan II University Hospital, Fez, Morocco
- 1 Department of medical oncology, Hassan II University Hospital, Fez, Morocco
- 2 Department of pathology, Hassan II University Hospital, Fez, Morocco
- 3 Department of general surgery, Hassan II University Hospital, Fez, Morocco
- 4 Department of general surgery, Hassan II University Hospital, Fez, Morocco
- 5 Department of radiology, Hassan II University Hospital, Fez, Morocco
- 6 Department of pathology, Hassan II University Hospital, Fez, Morocco
- 7 Department of medical oncology, Hassan II University Hospital, Fez, Morocco
- 8 Department of medical oncology, Hassan II University Hospital, Fez, Morocco
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Abstract
Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive variety of sarcoma arising typically from abdominal or pelvic peritoneum. Diagnosis and treatment approaches of this entity are complex and require a skilled, experienced, multidisciplinary team. Authors report their experience with a case of an intraabdominal DSRCT arising in a 37-year-old young man in order to discuss the clinico-pathological and radiological behavior of this tumor and underline diagnostic and therapeutic difficulties.
KeywordsDesmoplastic Small Round Cell TumorAbdomenHistologyChemotherapySurgeryOutcome
- Biswas, G., Laskar, S., Banavali, S.D., Gujral, S., Kurkure, P.A., Muckaden, M., Parikh, P.M. and Nair, C.N. (2005) Desmoplastic small round cell tumor: Extra abdominal and abdominal presentations and the results of treatment. Indian Journal Cancer. 42, 78-84. doi:10.4103/0019-509X.16696
- Gerald, W.L. and Rosai, J. (1989) Case 2 desmoplastic small round cell with divergent differentiation. Pediatric Pathology, 9, 177-183. doi:10.3109/15513818909022347
- Gerald, W.L., Rosai, J., Ladanyi, M. (1995) Characterization of genomic breakpoint and chemeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor. Proceedings of the National Academy of Sciences, 92, 1028-1032. doi:10.1073/pnas.92.4.1028
- Kushner, B.H., LaQuaglia, M.P., Wollner, N., Meyers, P.A., Lindsley, K.L. and Ghavimi, F. (1996) Desmoplastic small round cell tumor prolonged progression free survival with aggressive multimodality therapy. Journal of Clinical Oncology, 14, 1526-1531.
- Marina, N.M., Pappo, A.S., Parham, D.M., Cain, A.M., Rao, B.N. and Poquette, C.A. (1999) Chemotherapy dose intensification for pediatric patients with Ewing’s family of tumors and desmoplastic small round blue cell tumors: A feasibility study at St. Jude children’s research hospital. Journal of Clinical Oncology, 17, 180-190.
- Lauridant, G., Philippin, N., Ledem, F., Lemoine, S., Gouerant, C., Guillemet, P., et al. (2010) Optimal treatment with systemic chemotherapy, complete surgical excision and hyperthermic intraperitoneal chemotherapy for a desmplastic small round cell tumor in an adulte male patient. Gastroentérologie Clinique et Biologique, 34, 321-324.
- Kinra, P. and Pujahari, A.K. (2008) Desmoplastic small round cell tumor abdomen. Indian Journal of Pathology and Microbiology, 51, 449-450. doi:10.4103/0377-4929.42526
- Jellouli, M., Mekki, M., Krichene, I., Zakhama, A., Belghith, M., Jouini, R., Sahnoun, L., Rammeh, S., Harbi, A. and Nouri, A. (2003) Intraabdominal desmoplastic small round cell tumor in childhood: Case report and review of the literature. Bulletin de cancer, 12, 1063-1066.
- Kim, J.-W., Park, J.H., Cho, H.J. and Kwon, J.-H. (2009) A case of desmoplastic small round cell tumor diagnosed in a young female patient. Cancer Research and Treatment, 4, 233-236. doi:10.4143/crt.2009.41.4.233
- Farhat, F., Culine, S., L’homme, C., Duvillard, P., Terrier Lacombe, M.J., Michel, G., Soulie, P., Theodore, C., Lotz, J.P. and Droz, J.P. (1995) Tumeurs desmoplastiques à petites cellules rondes de l’adulte, une nouvelle entité. Bulletin de Cancer, 82, 665-673.