Case Report of International Journal of Case Reports
Intra-Abdominal Desmoplastic Small Round Cell Tumor: A Diagnostic Dilemma In Resource-Constrained Settings – A Case Report & Literature Review
ABIORO Imodoye Ayokunbi
Department of Surgery, University College Hospital, Ibadan, Nigeria
Introduction. Desmoplastic small round-cell tumours (DSRCT) are primarily intra-abdominal soft tissue sarcomas that are extremely rare and aggressive with a rapidly deteriorating clinical course and poor outcome despite therapy. It belongs to the family of small round blue-cell tumours, symptoms of the disease are non-specific and metastasis is often present at diagnosis. Objective. This report presents a typical case of an intra-abdominal DSRCT while highlighting how challenging the diagnosis can be in resource-poor settings and best-practice in the management of its attendant complications. Case presentation. This is a case 10-year-old boy who presented with a two-month history of weight loss and a two-week history of colicky abdominal pain, abdominal distension and vomiting and loss of appetite. He was pale, had left mandibular lymphadenopathy with a distended abdomen and ascites. A palpable mass was occluding ~80% of his rectal lumen. CT scan showed intra-abdominal masses in the rectovesical pouch and hepatic flexure with abdominopelvic lymphadenopathy Patient was worked up for exploratory laparotomy, colostomy placement and tumor biopsy, with the subsequent histology and immunohistochemistry a diagnosis of DSRCT was made. The patient developed moderate anaemia, acute kidney injury, hypertension and anarsarca while on admission; these were successfully managed with blood transfusions, intravenous fluids and drugs subsequently. He had a re-exploration for fecal disimpaction and adhesiolysis due to persistent intestinal obstruction. Patient was discharged 15 days after admissionin a stable clinical state. Conclusion. Desmoplastic round-cell tumour (DSRCT) primarily occurr within the abdomen, it is capable of mimicking the symptomatology of other intra-abdominal malignancies and a high index of suspicion is requisite to make a diagnosis especially in resource-poor settings with swift implementation of treatment modalities necessary to halt disease progress.
Keywords: Desmoplastic small round-cell tumour, aggressive course, complications.
How to cite this article:
ABIORO Imodoye Ayokunbi. Intra-Abdominal Desmoplastic Small Round Cell Tumor: A Diagnostic Dilemma In Resource-Constrained Settings – A Case Report & Literature Review. International Journal of Case Reports, 2021 5:196.
1. Salako O, Habeebu M, Jimoh M, Adeniji A, Adenipekun A. DESMOPLASTIC SMALL ROUND BLUE CELL TUMOUR OF THE ABDOMEN – A CASE REPORT. J West African Coll Surg. 2015;5(4):79-89.
2. Chang F. Desmoplastic Small Round Cell Tumors. Arch Pathol Lab Med. 2006;130:728-732.
3. Gerald WL, Haber DA. The EWS-WT1 gene fusion in desmoplastic small round cell tumor. Semin Cancer Biol. 2005;15(3):197-205. doi:10.1016/j.semcancer.2005.01.005
4. Ferreira EN, Barros BDF, de Souza JE, et al. A genomic case study of desmoplastic small round cell tumor: comprehensive analysis reveals insights into potential therapeutic targets and development of a monitoring tool for a rare and aggressive disease. Hum Genomics. 2016;10(1):36. doi:10.1186/s40246-016-0092-0
5. Lettieri CK, Garcia-filion P, Hingorani P. Incidence and Outcomes of Desmoplastic Small Round Cell Tumor : Results from the Surveillance , Epidemiology , and End Results Database. 2014;2014.
6. Wong HH, Hatcher HM, Benson C, et al. Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature. Clin Sarcoma Res. 2013;3(14):1-9. doi:10.3109/15513818909022347
7. Saab R, Khoury JD, Krasin M, Davidoff AM, Navid F. Desmoplastic Small Round Cell Tumor in Childhood : The St . Jude Children ’ s Research Hospital Experience. 2007;(March 2006):274-279. doi:10.1002/pbc
8. Dufresne A, Cassier P, Couraud L, et al. Desmoplastic small round cell tumor: Current management and recent findings. Sarcoma. 2012;2012. doi:10.1155/2012/714986
9. Zhang S, Zhang Y, Yu Y, Li JIA. Complete response of giant desmoplastic small round cell tumor treated with chemoradiotherapy : A case report. 2016:1069-1072. doi:10.3892/ol.2015.4024
10. Zhang G, Liu G, Zhao D, Cui X, Li G. Desmoplastic Small Round Cell Tumor of the Abdomen and Pelvis: Clinicopathological Characters of 12 Cases. Sci World J. 2014;2014(May 2011):1-7. doi:10.1155/2014/549612
11. Briseño-Hernández AA, Quezada-López DR, Corona-Cobián LE, Castañeda-Chávez A, Duarte-Ojeda AT, Macías-Amezcua MD. Intra-abdominal desmoplastic small round cell tumour. Cirugía y Cir (English Ed. 2015;83(3):243-248. doi:10.1016/j.circen.2015.09.029
12. Nabi S, Saste A, Gulati R. Case Report A Rare Case of Metastatic Desmoplastic Small Round Cell Tumour : Diagnosis and Management. 2015;2015.
13. Ariza M, Casan P, Ariza-prota MA, Pando-sandoval A, Fole-v D. Desmoplastic Small Round Cell Tumor of the Lung : A Case Report and Literature Review. 2015;(September). doi:10.1016/j.rmcr.2015.08.010
14. de Araujo RA, Araujo BJ. Desmoplastic small round cell tumor: report of 2 cases treated with chemotherapy alone or in combination with bevacizumab. Case Rep Oncol. 2014;7:102-108. doi:10.1159/000359997
15. Lee HEEJ, Hyun JINSOO, Jang HOESOO, Sul H, Park SGON. Paraneoplastic secondary hypertension due to a renin ‑ secreting desmoplastic small round cell tumor : A case report. 2014:1986-1992. doi:10.3892/ol.2014.2452
16. Yang S, Wang S, Chai C, Su Y, Fu O. I NTRA – ABDOMINAL D ESMOPLASTIC S MALL R OUND C ELL T UMOR W ITH E LEVATED S ERUM CA 125 : Kaohsiung J Med Sci. 2003;19(10):531-535. doi:10.1016/S1607-551X(09)70503-0
17. Syed A, Wheatley H, Badminton M, McDowell I. Urinary catecholamines and metabolites in the immediate postoperative period following major surgery. J Clin Pathol. 2004;57:548-550. doi:10.1136/jcp.2003.013201
18. Watanabe T, Miyamoto S, Kitagori K, et al. A case of long-term survival of metastatic desmoplastic small round cell tumor treated with multimodal therapy. Oncol Lett. 2012;3(1):30-34. doi:10.3892/ol.2011.457
19. Iyer RS, Schaunaman G, Pruthi S, Finn LS. Imaging of Pediatric Desmoplastic Small-Round-Cell Tumor with Pathologic Correlation. Curr Probl Diagn Radiol. 2013;42(1):26-32. doi:10.1067/j.cpradiol.2012.05.004
20. Wang LL, Perlman EJ, Vujanic GM, et al. Desmoplastic Small Round Cell Tumor of the Kidney in Childhood. Am J Surg Pathol. 2007;31(4):576-584.
21. D’cruze L, Dutta R, Rao S, Varadarajan S, Kuruvilla S. The Role of Immunohistochemistry in the Analysis of the Spectrum of Small Round Cell Tumours at a Tertiary Care Centre. J Clin Diagnostic Res. 2013;7(7):1377-1382. doi:10.7860/JCDR/2013/5127.3132
22. Arnold MA, Schoenfield L, Limketkai BN, Arnold CA. Diagnostic Pitfalls of Differentiating Desmoplastic Small Round Cell Tumor (DSRCT) From Wilms Tumor (WT). Am J Surg Pathol. 2014;38(9):1220-1226.
23. Bulbul A, Fahy BN, Xiu J, et al. Desmoplastic Small Round Blue Cell Tumor : A Review of Treatment and Potential Therapeutic Genomic Alterations. 2017;2017.
24. Lal DR, Su WT, Wolden SL, Loh KC, Modak S, Quaglia MP La. Results of multimodal treatment for desmoplastic small round cell tumors. 2005:251-255. doi:10.1016/j.jpedsurg.2004.09.046
25. Aguilera D, Hayes-jordan A, Anderson P, Woo S, Pearson M, Green H. Case Report Outpatient and Home Chemotherapy with Novel Local Control Strategies in Desmoplastic Small Round Cell Tumor. 2008;2008. doi:10.1155/2008/261589
26. Pinnix CC, Ph D, Fontanilla HP, et al. Whole Abdominopelvic Intensity-Modulated Radiation Therapy for Desmoplastic Small Round Cell Tumor After Surgery. Radiat Oncol Biol. 2012;83(1):317-326. doi:10.1016/j.ijrobp.2011.06.1985