Desmoplastic small round cell tumor a case report of a rare intra-abdominal tumor of the young adult

ILIAS EL AZHARI

doi:10.58372/2835-6276.1147

Authors

ILIAS EL AZHARI Department of General Surgery, Faculty of Medicine and Pharmacy, Ibn Rochd University Hospital Center, Hassan II University, Casablanca, Morocco

DOI:

https://doi.org/10.58372/2835-6276.1147

Keywords:

Desmoplastic small round cell tumor

Abstract

Desmoplastic small round cell tumor is a rare and highly aggressive mesenchymal tumor described as a distinct clinico-pathological entity in 1989 by Gerald . These tumors occur mainly in the peritoneal cavity,although other primary sites, such as testicular, ovarian, thoracic, pulmonary, intracranial and head and neck regions have been reported.

Clinical symptoms are non-specific. Diagnosis is based on histological analysis combined with immunohistochemical study.

Histological study typically shows small round blue cells in nests separated by abundant desmoplastic stroma associated with a single chromosomal translocation t(11:22) (p 13; q 12) involving the EWSR1 and WT1 genes.

The prognosis is particularly poor, with median survival ranging from 17 to 25 months. Management of this tumor remains difficult, and current regimens do not achieve a significant cure rate despite the use of aggressive treatments such as polychemotherapy.

References

DUFRESNE, Armelle, CASSIER, Philippe, COURAUD, Laure, et al. Desmoplastic small round cell tumor: current management and recent findings. Sarcoma, 2012, vol. 2012.

Lae ME, Roche PC, Jin L, Lloyd RV, Nascimento AG. Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors. Am J Surg Pathol. 2002 Jul et 121311, 26(7):823-35. doi: 10.1097/00000478-200207000-00001. PMID:.

Bakrin N, Glehen O, Gilly FN. Pathologie tumorale du mésentère et du péritoine. EMC - Gastro-entérologie 2015 et 9-039-A-20], 10(1):1-11 [Article.

MORANI, A. C., BATHALA, T. K., SURABHI, V. R., et al. Desmoplastic small round cell tumor: imaging pattern of disease at presentation. American Journal of Roentgenology, 2019, vol. 212, no 3, p. W45-W54.

Mar, Coulibalya B and al Tumeur desmoplastique à petites cellules rondes: deux cas d'atteinte abdominopelvienne diffuse. Gastroenterol Clin Biol. 2008 et 10.1016/j.gcb.2008.01.023, 32(3):278-81.doi:.

THEJUS JAYAKRISHNAN, RYAN MOLL, ARIEL SANDHU, ANGELA SANGUINO, GURVEEN KAUR and SHIFENG MAO Anticancer Research August 2021, 41 (8) 3859-3866 et https://doi.org/10.21873/anticanres.15179, DOI:.

Scheer, M, Vokuhl, C, Blank, B, et al. Desmoplastic small round cell tumors: Multimodality treatment and new risk factors. Cancer Med. 2019 et https://doi.org/10.1002/cam4.1940, 8: 527–542.

Honoré, C., Amroun, K., Vilcot, L. et al. Abdominal Desmoplastic Small Round Cell Tumor: Multimodal Treatment Combining Chemotherapy, Surgery, and Radiotherapy is the Best Option.Ann Surg Oncol 22,1073–1079 (2015).https://doi.org/10.1245/s10434-014-4123-6.

Hayes-Jordan A, Green H, Fitzgerald N, Xiao L, Anderson P. Novel Treatment for Desmoplastic Small Round Cell Tumor: Hyperthermic Intraperitoneal Perfusion. J Pediatr Surg (2010) 45(5):1000–6. doi: 10.1016/j.jpedsurg.2010.02.034.

WEI, Guixia, SHU, Xinyao, ZHOU, Yuwen, et al. Intra-abdominal desmoplastic small round cell tumor: current treatment options and perspectives. Frontiers in Oncology, 2021, vol. 11, p. 705760.

Christina K. Lettieri et al, "Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database", Journal of Cancer Epidemiology, vol. 2014, https://doi.org/10.1155/2014/680126.