BLUE ROUND SMALL CELL TUMOR: A SURGICAL UPDATE OF DSRCT WITH REVIEW OF LITERATURE ‘A GRIM AFFAIR’


Blue Round Small Cell tumor: A Surgical Update of DSRCT with review of literature ‘A Grim Affair’


Dr.UwaisRiazUlHasan3 M.Med, Dr.Khathija Hasan M.Med, Dr.Farooq Ahmed Qureshi MS, Dr. Victor Effiong Obong M.B.B.Ch, MWACS, Dr.Abdul Aziz Al Nami M.B.B.S, Dr.Ali Ibrahim AlShaqaqiq M.B.B.S, Dr.Mohammad AbdulMajeed Alghadeer M.B.B.S Dr. Marwan Ahmad AlRayhan M.B.B.S, Dr. Mohammed Ali AlJummah M.B.B.S, Dr.Baqer Ali Aldheen M.B.B.S, Dr. Ali Hussain AlRufayi MD, Dr.ShehlaRiazUlHasan Phd.
Dr. Moath AbdulAziz AlMasoud2 M.D, Dr. Noura Al Dossary1

Department of General Surgery, Al Omran General Hospital, Al Hassa, Kingdom of Saudi Arabia. Hospital Director1,Head of department2, Assoc Consultant General Surgeon3


Desmoplastic small round cell tumor (DSRCT) is a tumor derived from the Greek desmos referring to knot and plasis to formation an uncommon soft tissue malignant tumor, mesenchymal in origin and aggressive with a prelidiction for males and advanced at presentation. It was first described as a distinct clinical entity by Gerald WL and Rosai J (7). There are fewer than 200 reported to date. Depending on the primary site of location the Clinical manifestations vary. As most arise from the abdomen and pelvis they remain asymptomatic till they attain a huge size. Other reported sites are the skull, thorax, and paratesticular region (10,13). We report the case of a 19 yr old male who had non specific abdominal discomfort with asthenia for a period of six months and was referred to us for evaluation of left supraclavicular nodes. The prognosis of Desmoplastic small round cell tumor (DSRCT) is poor with few surviving less than two years.


Keywords: Blue round, Desmoplastic small round cell tumor, Radiology, Multimodal therapy.

Free Full-text PDF


How to cite this article:

UwaisRiazUlHasan, Khathija Hasan, Farooq Ahmed Qureshi, Victor Effiong Obong, MWACS, Abdul Aziz Al Nami, Ali Ibrahim AlShaqaqiq, Mohammad AbdulMajeed Alghadeer, Marwan Ahmad AlRayhan, Mohammed Ali AlJummah, Baqer Ali Aldheen, Ali Hussain AlRufayi,ShehlaRiazUlHasan,Moath AbdulAziz AlMasoud, Noura Al Dossary. Blue Round Small Cell tumor: A Surgical Update of DSRCT with review of literature ‘A Grim Affair’. American Journal of Surgical Research and Reviews, 2021; 4:31. DOI:10.28933/ajsrr-2021-06-2706


References

1. A. Hayes-Jordan and P. M. Anderson, “The diagnosis and management of desmoplastic small round cell tumor: a review,” Current Opinion in Oncology, vol. 23, no. 4, pp. 385–389, 2011.
2. B. H. Kushner, M. P. LaQuaglia, N. Wollner et al. “Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy,” Journal of Clinical Oncology, vol. 14, no. 5, pp.1526–1531,1996.
3. Chang F. Desmoplastic Small Round Cell Tumors: Cytologic, Histologic,and immunohistochemical Features. Arch Pathol Lab Med. 2006;130:728-732.
4. D. R. Lal, W. T. Su, S. L. Wolden, K. C. Loh, S. Modak, and M. P. La Quaglia, “Results of multimodal treatment for desmoplastic small round cell tumors,” Journal of Pediatric Surgery, vol. 40, no. 1, pp. 251–255, 2005.
5. Dufresne A, Cassier P, Couraud L, et al. Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings. Clin Sarcoma Res.2013;3:14.
6. Gerald WL, Ladanyi M, De Alava E, et al. Clinical, pathologic and molecular spectrum of tumors associated with t[11;22][p13;q12]: desmoplastic small round-cell tumor and its variants. J Clin Oncol. 1998;16[9]:3028-3036.
7. Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J. Intraabdominal desmoplastic small round-cell tumor: report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol. 1991;15:499–513W.L.
8. J. Liu, M. M. Nau, J. C. Yeh, C. J. Allegra, E. Chu, et al “Molecular heterogeneity and function of EWS-WT1fusion transcripts in desmoplastic small round cell tumors”
9. Kretschmar CS, Colbach C, Bhan I, Crombleholme TM. et al Desmoplastic small cell tumor: A report of three cases and a review of the literature. J Pediatr Hematol Oncol. 1996;18:293–8.
10. O.W. Cummings, T.M. Ulbright, R.H. Young, A.P. Dei Tos, et al. Desmoplastic small round cell tumors of the paratesticular region. A report of six cases, Am. J. Surg. Pathol. 21 [1997] 219e225.
11. R. E. Schwarz, W. L. Gerald, B. H. Kushner, D. G. Coit, et al M. F. Brennan, “Desmoplastic small round cell tumors: prognostic indicators and results of surgical management,” Annals of Surgical Oncology, vol. 5, no. 5, pp,416–422, 1998.
12. Tison V, Cerasoli S, Morigi F, et al Intracranial desmoplastic small-cell tumor: report of a case. Am J Surg Pathol. 1996;20:112–117.
13. V. Parkash, W.L. Gerald, A. Parma, M. Miettinen, et al, Desmoplastic small round cell tumor of the pleura, Am. J. Surg. Pathol. 19 [1995].
14. Xiang L, Jing Y, Shibao F, Xiaoming X, Jie Z. et al, Desmoplastic small round cell tumor: a case report and review of the literature. World Journal of Surgical Oncology. 2014;12:9.
15. 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[1]:14.
16. Z.M. Wang, W.B. Xiao, S.S. Zheng, et al. Desmoplastic small round cell tumor of the lung: case report, Chin. Med. J. Engl. 120 [2007] 2327e2328.
17. Richard Bellah, Lisa Suzuki Bordalo, Eric Brecher et al.Desmoplastic small round cell tumors in the abdomen and pelvis : A report of the CT findings in Eleven Children and Young Adults. Am Journal of Roentgenology. 2005;184:1910-1914. 10.2214/ajr.184.6.01841910.
18. Choi JK, van Hoeven K, Brooks JJ, et al. Desmoplastic small round cell tumor presenting in pleural fluid and accompanied by desmin-positive mesothelial cells. Acta Cytol. 1995;39:377–378.
19. Roganovich J, Bisogno G, Cecchetto G, et al. Paratesticular desmoplastic small round cell tumor: case report and review of the literature. J Surg Oncol. 1999;71:269–272.
20. Takhtani D, Saleeb SF, Teplick SK et al. General case of the day. Desmoplastic small roundcelltumorof the abdomen with scrotal metastases. Radiographics. 1999;19:252–254.
21. Ordóñez NG. Desmoplastic small round cell tumor: I: a histopathologic study of 39 cases with emphasis on unusual histological patterns. Am J Surg Pathol. 1998;22:1303–1313.
22. Yoshizawa J, Maie M, Eto T, et al. A case of intra-abdominal desmoplastic small-round-cell tumor with elevated serum CA125. Pediatr Surg Int. 2002;18:238–240.
23. Mazuryk M, Paterson AH, Temple W, et al. Benefit of aggressive multimodality therapy with autologous stem cell support for intra-abdominal desmoplastic small round cell tumor. Bone Marrow Transplant. 1998;21:961–963.
24. Thway K., Noujaim J., Zaidi S., et al. Desmoplastic Small Round Cell Tumor: Pathology, Genetics, and Potential Therapeutic Strategies. Int. J. Surg. Pathol. 2016;24:672–684. doi: 10.1177/1066896916668637.
25. Sawyer J.R., Tryka A.F., Lewis J.M. et al. A Novel Reciprocal Chromosome Translocation t[11;22][p13;q12] in an Intraabdominal Desmoplastic Small Round-Cell Tumor. Am. J. Surg. Pathol. 1992;16:411–416. doi: 10.1097/00000478-199204000-00010.
26. Rodriguez E., Sreekantaiah C., Gerald W. et al., A Recurring Translocation, t[11;22][p13;q11.2], Characterizes Intra-Abdominal Desmoplastic Small Round-Cell Tumors. Cancer Genet Cytogent. 1993;21:17–21. doi: 10.1016/0165-4608[93]90105-U.
27. Gedminas J.M., Chasse M.H., McBrairty M., et al. Desmoplastic small round cell tumor is dependent on the EWS-WT1 transcription factor. Oncogenesis. 2020;9:1–8. doi: 10.1038/s41389-020-0224-1.
28. Scharnhorst V., van der Eb A.J., Jochemsen A.G et al. WT1 proteins: Functions in growth and differentiation. Gene. 2001;273:141–161. doi: 10.1016/S0378-1119[01]00593-5.
29. Lal D.R., Su W.T., Wolden S.L., Loh K.C., Modak S. et al. Results of multimodal treatment for desmoplastic small round cell tumors. J.PediatrSurg. 2005;40:251, 255. doi: 10.1016/j.jpedsurg.2004.09.046.
30. Kis B., O’Regan K.N., Agoston A., Javery O., et al. Imaging of desmoplastic small round cell tumour in adults. Br. J. Radiol. 2012;85:187–192. doi: 10.1259/bjr/57186741.
31. Hollingsworth R.E., Jansen K. et al. Turning the corner on therapeutic cancer vaccines. NPJ Vaccines. 2019;8:7. doi: 10.1038/s41541-019-0103-y.
32. Andrea A Hayes-Jordan , Brian A Coakley , Holly L Green et al. Desmoplastic Small Round Cell Tumor Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: Phase 2 Trial , DOI: 10.1245/s10434-018-6333-9 PMCID: PMC5842144.
33. Mallone, S.; De Angelis, R.; Van Der Zwan, J.M.; Trama, A.; Siesling, S.; Gatta, G.; Capocaccia, R. Methodological aspects of estimating rare cancer prevalence in Europe: The experience of the RARECARE project. Cancer Epidemiol. 2013, 37, 850–856.


Terms of Use/Privacy Policy/ Disclaimer/ Other Policies:
You agree that by using our site, you have read, understood, and agreed to be bound by all of our terms of use/privacy policy/ disclaimer/ other policies (click here for details).



This work and its PDF file(s) are licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.