Desmoplastic Small Round Cell Tumour (DSRCT) - A Case Report and Review of Literature

Authors

  • AKM Amirul Morshed Dhaka Medical College & Hospital Dhaka
  • Farhana Afroz Dhaka Medical College & Hospital Dhaka
  • Chowdhury Md. Fuad Galib Dhaka Medical College & Hospital Dhaka
  • Shahnoor Islam Dhaka Medical College & Hospital Dhaka
  • Kaniz Hasina Dhaka Medical College & Hospital Dhaka
  • Ashish Kumar Ghosh Dhaka Medical College & Hospital Dhaka

DOI:

https://doi.org/10.3329/jpsb.v2i2.19552

Keywords:

Childhood, Desmoplastic small round cell tumor, multidrug chemotherapy

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare pathologic entity that most frequently affects the peritoneal cavity and presents in pediatric and adolescent boys. It often presents at an advanced stage and has a generally poor prognosis. Sometimes it may involve liver at diagnosis. We present an unusual case of DSRCT who was present with mass in lower abdomen, pain, hepatomegaly and mild ascites. This tumor is characterized by nests of small undifferentiated cells that show immunohistochemical evidence of epithelial, mesenchymal and neural differentiation. In our patient histologicaly tumor had the characteristic features of DSRCT and were composed of small round cells with hyperchromatic nuclei and scanty cytoplasm. With various difficulties in diagnosis we ultimately reached at diagnosis by open biopsy and immunohistochemistry. Now patient is on multidrug chemotherapy (modified p6 protocol). Diagnosis and management of a rare tumor needs high level of suspicion and in time intervention.

DOI: http://dx.doi.org/10.3329/jpsb.v2i2.19552

Downloads

Download data is not yet available.
Abstract
196
PDF
100

Author Biographies

AKM Amirul Morshed, Dhaka Medical College & Hospital Dhaka

Department of Pediatric Haematology and Oncology

Farhana Afroz, Dhaka Medical College & Hospital Dhaka

Department of Pediatric Haematology and Oncology

Chowdhury Md. Fuad Galib, Dhaka Medical College & Hospital Dhaka

Department of Pediatric Haematology and Oncology

Shahnoor Islam, Dhaka Medical College & Hospital Dhaka

Department of Pediatric Surgery

Kaniz Hasina, Dhaka Medical College & Hospital Dhaka

Department of Pediatric Surgery

Ashish Kumar Ghosh, Dhaka Medical College & Hospital Dhaka

Department of Pediatric Haematology and Oncology

Downloads

Published

2014-07-16

How to Cite

Morshed, A. A., Afroz, F., Galib, C. M. F., Islam, S., Hasina, K., & Ghosh, A. K. (2014). Desmoplastic Small Round Cell Tumour (DSRCT) - A Case Report and Review of Literature. Journal of Paediatric Surgeons of Bangladesh, 2(2), 79–82. https://doi.org/10.3329/jpsb.v2i2.19552

Issue

Section

Case Reports