Abstract
Desmoplastic small round cell tumor (DSRCT) is an uncommon entity characterized by sarcomatous abdomino-pelvic mass arising from serosal surface of peritoneum. Till date, only a few cases of DSRCT has been reported in literature. Here we presented a case of small intestinal DSRCT in a young male presented with peri-umbilical pain of 3-months duration. Patient underwent exploratory laparotomy with resection of large ileal mass. Histopathology of surgical specimen revealed desmoplastic small round cell tumor. Patient was planned for adjuvant chemotherapy with VAC regimen. But after receiving 2-courses, patient condition deteriorated and patient was succumbed to death. This case report demonstrated the aggressiveness of DSRCT.
Keywords: Adjuvant chemotherapy, desmoplastic small round cell tumor, exploratory laparotomy, VAC regimen.
References
- Saleh, Daniyah et al. "Desmoplastic Small Round Cell Tumor of Pancreatic Origin in a Young Child: A Case Report and Review of Literature." The American journal of case reports vol. 21 e922762. 13 Jul. 2020, doi:10.12659/AJCR.922762
- Hatanaka, Kanako C et al. "Desmoplastic small round cell tumor of the parotid gland-report of a rare case and a review of the literature." Diagnostic pathology vol. 14,1 43. 18 May. 2019, doi:10.1186/s13000-019-0825-1
- He, Xue-Rui et al. "Primary desmoplastic small round cell tumor in the left orbit: a case report and literature review." International ophthalmology vol. 39,2 (2019): 471-475. doi:10.1007/s10792-018-0829-y
- Abu-Zaid, Ahmed, et al. "Desmoplastic small round cell tumor of stomach." Case Reports in Gastrointestinal Medicine 2013 (2013).
- Huang, Jian Ping et al. "Desmoplastic small round cell tumor in transverse colon: report of a rare case." International surgery vol. 100,5 (2015): 809-13. doi:10.9738/INTSURG-D-14-00134.1
- Gerald, W L, and J Rosai. "Case 2. Desmoplastic small cell tumor with divergent differentiation." Pediatric pathology vol. 9,2 (1989): 177-83. doi:10.3109/15513818909022347
- Salako, O et al. "DESMOPLASTIC SMALL ROUND BLUE CELL TUMOUR OF THE ABDOMEN - A CASE REPORT." Journal of the West African College of Surgeons vol. 5,4 (2015): 79-89.
- Wong, Han Hsi et al. "Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature." Clinical sarcoma research vol. 3,1 14. 26 Nov. 2013, doi:10.1186/2045-3329-3-14
- Subbiah, Vivek et al. "Multimodality Treatment of Desmoplastic Small Round Cell Tumor: Chemotherapy and Complete Cytoreductive Surgery Improve Patient Survival." Clinical cancer research : an official journal of the American Association for Cancer Research vol. 24,19 (2018): 4865-4873. doi:10.1158/1078-0432.CCR-18-0202
- Hayes-Jordan, Andrea, et al. "Continuous hyperthermic peritoneal perfusion for desmoplastic small round cell tumor." Journal of pediatric surgery 42.8 (2007): e29-e32.
- Kushner, B H et al. "Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy." Journal of clinical oncology : official journal of the American Society of Clinical Oncology vol. 14,5 (1996): 1526-31. doi:10.1200/JCO.1996.14.5.1526
- Goodman, Karyn A et al. "Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor." International journal of radiation oncology, biology, physics vol. 54,1 (2002): 170-6. doi:10.1016/s0360-3016(02)02871-7
- Pinnix, Chelsea C et al. "Whole abdominopelvic intensity-modulated radiation therapy for desmoplastic small round cell tumor after surgery." International journal of radiation oncology, biology, physics vol. 83,1 (2012): 317-26. doi:10.1016/j.ijrobp.2011.06.1985
- Bexelius, Tomas S et al. "Mini-Review on Targeted Treatment of Desmoplastic Small Round Cell Tumor." Frontiers in oncology vol. 10 518. 21 Apr. 2020, doi:10.3389/fonc.2020.00518
- Shi, Chunyu et al. "Effective treatment of apatinib in desmoplastic small round cell tumor: a case report and literature review." BMC cancer vol. 18,1 338. 27 Mar. 2018, doi:10.1186/s12885-018-4135-x
- Hayes-Jordan, Andrea et al. "Management of desmoplastic small round cell tumor." Seminars in pediatric surgery vol. 25,5 (2016): 299-304. doi:10.1053/j.sempedsurg.2016.09.005
- Subbiah, Vivek, Ravi Murthy, and Pete M. Anderson. "[90Y] yttrium microspheres radioembolotherapy in desmoplastic small round cell tumor hepatic metastases." Journal of Clinical Oncology 29.11 (2011): e292.
- Lal, Dave R et al. "Results of multimodal treatment for desmoplastic small round cell tumors." Journal of pediatric surgery vol. 40,1 (2005): 251-5. doi:10.1016/j.jpedsurg.2004.09.046
- Biswal, Biswa Mohan et al. "Intra-abdominal desmoplastic small round cell tumor: Presentation of four cases and review of the literature." Indian journal of medical and paediatric oncology : official journal of Indian Society of Medical & Paediatric Oncology vol. 31,1 (2010): 24-7. doi:10.4103/0971-5851.68849
- Gil, Alberto et al. "Clinical perspective on desmoplastic small round-cell tumor." Oncology vol. 67,3-4 (2004): 231-42. doi:10.1159/000081323
Corresponding Author
Dr Diptajit Paul
Pt BD Sharma PGIMS, Rohtak, Haryana India 124001
DOI: https://dx.doi.org/10.18535/jmscr/v10i7.06
