Share This Article:

Desmoplastic small round cell tumor of the abdomen: A case report and literature review of therapeutic options

Abstract Full-Text HTML XML Download Download as PDF (Size:852KB) PP. 207-211
DOI: 10.4236/health.2012.44031    4,235 Downloads   6,815 Views   Citations


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.

Conflicts of Interest

The authors declare no conflicts of interest.

Cite this paper

Benhammane, H. , Chbani, L. , Ousadden, A. , Mouquit, O. , Tizniti, S. , Amarti, A. , Mellas, N. and Mesbahi, O. (2012) Desmoplastic small round cell tumor of the abdomen: A case report and literature review of therapeutic options. Health, 4, 207-211. doi: 10.4236/health.2012.44031.


[1] 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
[2] 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
[3] 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
[4] 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.
[5] 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.
[6] 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.
[7] 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
[8] 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.
[9] 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
[10] 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.
[11] Pickhardt, P.J., Fisher, A.J., Balfe, D.M., Dehner, L.P. and Huettner, P.C. (1999). Desmoplastic small round cell tumor of the abdomen: Radiologichistopathologic correlation. Radiology, 210, 633-638.
[12] Stuart-Buttlea, C.E., Smarta, C.J., Pritchardb, S., Martinc, D. and Welch, I.M. (2008) Desmoplastic small round cell tumour: A review of literature and treatment options. Surgical Oncology, 17, 107-112 doi:10.1016/j.suronc.2007.11.005
[13] Chang, F. (2005) Desmoplastic small round cell tumours: Cytologic, histologic, and immunochemical features. Archives of Pathology and Laboratory Medicine, 5, 728-732.
[14] Ordonez, N.G. (1998) Desmoplastic small round cell tumor: A histopathologic study of 39 cases with emphasis on unusual histological patterns. American Journal of Surgical Pathology, 22, 1303-1313. doi:10.1097/00000478-199811000-00001
[15] Lal, D.R., Su, W.T., Loh, K.C. and La Quaglia, M.P. (2005) Results of multimodal treatment for desmoplastic small round cell tumour. Journal of Paediatric Surgery, 40, 251-255. doi:10.1016/j.jpedsurg.2004.09.046
[16] Livaditi, E., Mavridis, G. and Soutis, M., (2006) Diffuse intraabdominal desmoplastic small round cell tumor: A ten-year experience. European Journal of Paediatric Surgery, 16, 423-437. doi:10.1055/s-2006-924736
[17] Gil, A., Paortilla, A., Brun, E., et al. (2004) Clinical perspective on desmoplastic small round cell tumour. Oncology, 67, 231-242. doi:10.1159/000081323
[18] Mrabti, H., Kaikani, W., Ahbeddou, N., Abahssain, H., El Khannoussi, B., Amrani, M. and Errihani, H. (2011) Metastatic desmoplastic small round cell tumor controlled by an anthracycline-based regimen: Review of the role of chemotherapy. Journal of Gastrointestinal Cancer, 43, 103-109. doi:10.1007/s12029-011-9260-6
[19] Hayes-Jordan, A., Green, H., Fitzgerald, N., Xiao, L.C. and Anderson, P. (2010) Novel treatment for desmoplastic small round cell tumor: Hyperthermic intraperitoneal perfusion. Journal of Pediatric Surgery, 45, 1000-1006. doi:10.1016/j.jpedsurg.2010.02.034
[20] Chao, J., Budd, G.T., Chu, P., Frankel, P., Garcia, D., Junqueira, M., Loera, S., Somlo, G., Sato, J. and Chow, W.A. (2010) Phase II clinical trial of imatinib mesylate in therapy of KIT and/or PDGFRalpha-expressing Ewing sarcoma family of tumors and desmoplastic small round cell tumors. Anticancer Research, 30, 547-552.

comments powered by Disqus

Copyright © 2018 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.