Metastatic Desmoplastic Small Round Cell Tumor - A Case Report and Review of the Literature

Yesmin Tanjin Jahan(1), Zohora Jameela Khan(2), Zannat Ara(3), S. M. Rezanur Rahman(4), Mehnaz Akter(5), Md. Tanvir Ahammed(6), Rezwana Refat(7)
(1) Dhaka Medical College, Dhaka, Bangladesh
(2) Dhaka Medical College, Dhaka, Bangladesh
(3) Dhaka Medical College, Dhaka, Bangladesh
(4) Dhaka Medical College, Dhaka, Bangladesh
(5) Dhaka Medical College, Dhaka, Bangladesh
(6) Dhaka Medical College, Dhaka, Bangladesh
(7) Dhaka Medical College, Dhaka, Bangladesh

Abstract

Desmoplastic small round cell tumour is a rare malignancy most often seen in the abdomen or pelvis of young men. Unfortunately, this disease is usually metastatic at diagnosis and has dismal outcomes. Since 1991, when it was first described as a distinct clinical entity by Gerald WL and Rosai J some 200 cases were reported. All cases are characterized by the chromosomal translocation t (11;22) (p13; q12), which results in a formation of EWSR1-WT1 fusion gene. The diagnosis of IDSRCT is often made with core-needle tissue biopsy specimens of laparoscopy or laparotomy. Immunohistochemical analyses have shown the co-expression of epithelial, neuronal, myogenic, and mesenchymal differentiation markers. Multimodal therapy is usually indicated with chemotherapy, surgery & radiotherapy. We report a rare and unusual case of an extensive abdominopelvic desmoplastic small round cell tumour with liver & lung metastases, in an adolescent male patient from Bangladesh who presented with abdominal mass, pain & hepatomegaly. With various difficulties in diagnosis, we ultimately reached at diagnosis by open biopsy and immunohistochemistry as desmoplastic small round cell tutor. Now patient is on multidrug chemotherapy (modified p6) protocol.

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References

Gerald WL, Rosai J. Desmoplastic small cell tumour with divergent differentiation. Pediatr Pathol. 1989; 9:177–183.

Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J. Intra-abdominal desmoplastic small round-cell tumour. Report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol. 1991; 15:499–513.

Lae ME, Roche PC, Jin L, Lloyd RV, Nascimento AG. Desmoplastic small round cell tumour: aclinicopathologic, immunohistochemical, and molecular study of 32 tumours. Am J Surg Pathol. 2002; 26: 823-35.

Morshed AA, Afroz F, Galib C, Islam S, Hasina K, Ghosh AK. Desmoplastic small round cell tumour (DSRCT) - A case report and review of literature. Journal of Paediatric Surgeons of Bangladesh. 2011; 2 (2): 79-82.

Ladanyi M, Gerald W. Fusion of the EWS and WT1 genes in the desmoplastic small round cell tumour. Cancer Research. 1994; 54 (11): 2837–2840.

Bent MA, Padilla BE, Goldsby RE, DuBois SG. Clinical Characteristics and Outcomes of Pediatric Patients with Desmoplastic Small Round Cell Tumor. Rare tumours. 2016; 8(1): 6145.

Chang F. Desmoplastic Small Round Cell Tumors: Cytologic, Histologic and immunohistochemical Features. Arch Pathol Lab Med. 2006; 130: 728-732.

Xiang L, Jing Y, Shibao F, Xiaoming X, Jie Z. Desmoplastic small round cell tumour: a case report and review of the literature. World J. Surg. Oncol. 2014; 12: 9.

Kis B, Regan KN, Agoston A, Javery O, Jaganathan J, Ramaiya NH. Imaging of desmoplastic small round cell tumours in adults. Br J Radiol. 2012; 85(1010):187-192.

Zhang G, Zhu Y, Gan H, Ye D. Testicular desmoplastic small round cell tumour: a case report and review of the literature. World J Surg Oncol. 2014; 12:227.

W. L. Gerald, M. Ladanyi, E. De Alava et al. Clinical, pathologic, and molecular spectrumof tumours associated with t(11;22)(p13;q12): desmoplastic small round-cell tumour and

its variants. Journal of Clinical Oncology. 1998;16(9):3028–3036.

Bent MA, Padilla BE, Goldsby RE, DuBois SG. Clinical Characteristics and Outcomes of

Pediatric Patients with Desmoplastic Small Round Cell Tumor. Rare tumours. 2016; 8(1):6145.

Ofori E, et al. Desmoplastic small round cell tumour: a rare case of extraluminal bowel obstruction and review of the literature. J Gastrointest Cancer. 2019; 50(3):560–3.

Butt SU, Bull JMH, Scott A. Desmoplastic small round-cell tumour in a young indigenous Australian man: a case report. J Glob Oncol. 2017; 3(1):79–81.

Ertoy Baydar D, et al. Desmoplastic small round cell tumour of the kidney: a case report. Diagn Pathol. 2020; 15(1):95.

Vuji? G, et al. Desmoplastic small round cell tumour of the ovary: a case report with a new modality of treatment and review of the literature. Rev Bras Ginecol Obstet. 2020; 42(5):297–302.

Liping Cao, Jun Ni, Risheng Que, Zhengrong Wu, and Zhenya Song. Desmoplastic Small Round Cell Tumor: A Clinical, Pathological, and Immunohistochemical Study of 18 Chinese Cases. Int J Surg Pathol. 2008; 16: 257-62.

Thway K, Noujaim J, Zaidi S, Miah AB, Benson C Messiou C, et al. Desmoplastic Small Round Cell Tumor: Pathology, Genetics, and Potential Therapeutic Strategies. Int. J. Surg. Pathol. 2016; 24: 672–684.

Chang F. Desmoplastic Small Round Cell Tumors: Cytologic, Histologic, and immunohistochemical Features. Arch Pathol Lab Med. 2006; 130:728-732.

Parham DM. Small Round Cell Tumors. In: Miettinen M, editor. Modern Soft Tissue Pathology: Tumors and Non-neoplastic conditions, New York, Cambridge University Press; 2010: 896-929.

Klijanienko J, Colin P, Couturier J, et al. Fine-Needle Aspiration in Desmoplastic Small Round Cell Tumor. A report of 10 New Tumors in 8 Patients with Clinicopathological and Molecular Correlations with Review of the Literature. Cancer Cytopathol. 2014; 122(5):386-393.

Miwa S, Kitamura S, Shirai T, et al. Desmoplastic small round cell tumour successfully treated with caffeine-assisted chemotherapy: a case report and review of the literature. Anticancer Res. 2010; 30(9):3769-3774.

Stuart-Buttle CE, Smart CJ, Pritchard S, Martin D, Welch IM. Desmoplastic Small Round Cell Tumour: A Review of Literature and Treatment Options. Surg Oncol 2008; 17(2):107–12. doi: 10.1016/j.suronc.2007.11.005

Saab R, Khoury JD, Krasin M, Davidoff AM, Navid F. Desmoplastic small round cell tumour in childhood: the St. Jude Children’s Research Hospital experience. Pediatr Blood Cancer. 2007; 49: 274-9.

Schwarz RE, Gerald WL, Kushner BH, et al. Desmoplastic small round cell tumours: Prognostic indicators and results of surgical management. Ann Surg Oncol. 1998; 5:416 – 22.

Kushner BH, LaQuaglia MP, Wollner N, et al. Desmoplastic small round-cell tumour: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol. 1996; 14:1526–31. [PubMed] [Google Scholar]

Lal DR, Su WT, Wolden SL, et al. Results of multimodal treatment for desmoplastic small round cell tumours. J Pediatr Surg. 2005; 40:251–5.

Authors

Yesmin Tanjin Jahan
yesmin.048586@gmail.com (Primary Contact)
Zohora Jameela Khan
Zannat Ara
S. M. Rezanur Rahman
Mehnaz Akter
Md. Tanvir Ahammed
Rezwana Refat
Author Biographies

Yesmin Tanjin Jahan, Dhaka Medical College, Dhaka

Assistant Professor, Department of Paediatric Haematology & Oncology, Dhaka Medical College Hospital, Dhaka

Zohora Jameela Khan, Dhaka Medical College, Dhaka

Professor of Paediatric Haematology & Oncology, Dhaka Medical College Hospital, Dhaka

Zannat Ara, Dhaka Medical College, Dhaka

Assistant Professor of Paediatric Haematology & Oncology, Dhaka Medical College Hospital, Dhaka

S. M. Rezanur Rahman, Dhaka Medical College, Dhaka

Assistant Professor of Paediatric Haematology & Oncology, Dhaka Medical College Hospital, Dhaka

Mehnaz Akter, Dhaka Medical College, Dhaka

Assistant Professor of Paediatric Haematology & Oncology, Dhaka Medical College Hospital, Dhaka

Md. Tanvir Ahammed, Dhaka Medical College, Dhaka

Assistant Professor of Paediatric Haematology & Oncology, Dhaka Medical College Hospital, Dhaka

Rezwana Refat, Dhaka Medical College, Dhaka

Registrar, Department of Paediatric Haematology & Oncology, Dhaka Medical College Hospital, Dhaka

1.
Jahan YT, Khan ZJ, Ara Z, Rahman SMR, Akter M, Ahammed MT, et al. Metastatic Desmoplastic Small Round Cell Tumor - A Case Report and Review of the Literature. Haematol J Bangladesh [Internet]. 2023 Dec. 31 [cited 2024 Apr. 13];7(2):89-94. Available from: https://journal.hematologybd.org/index.php/haematoljbd/article/view/105

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How to Cite

1.
Jahan YT, Khan ZJ, Ara Z, Rahman SMR, Akter M, Ahammed MT, et al. Metastatic Desmoplastic Small Round Cell Tumor - A Case Report and Review of the Literature. Haematol J Bangladesh [Internet]. 2023 Dec. 31 [cited 2024 Apr. 13];7(2):89-94. Available from: https://journal.hematologybd.org/index.php/haematoljbd/article/view/105
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