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Journal of Paediatric Surgeons of Bangladesh
Case Reports · Vol. 2 No. 2 (2011)

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

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JPSB
Journal of
Paediatric Surgeons of Bangladesh
Published : July 2011
CONTENTSVolume 02Number 2July 2011
Editorial
Laparoscopy in Children, A Brief Outline and Bangladesh Perspective
Md. Jafrul Hannan
42
Original Articles
Outcome of Vesiciureteric Reflux After Primary Fulguration of Posterior Urethral Valves
M. M. Masud Pervez, Kaniz Hasina, Md. Ashraf Ul Huq, Md. Nooruzzaman, Abdul Hanif
46
Post A-H Pyeloplasty Analysis of Renal Function in Unilateral Hydronephrosis Due to Congenital Pelviureteric Junction Obstruction
Nandan Kumar Majumder, Md. Ruhul Amin, Rajib Khastgir, Mizanur Rahman, Md. Abu Bakar Akan
53
Assesment of The Effect of Multimodal Preemptive Analgesia in Children
Md. Nooruzzaman, MM Masud Pervez, Md. Abu Bakar Akan, M Anisuzzaman, Md. Nazrul Islam, AM Shahinoor
56
Early Post-operative Outcome of Primary Repair of Bladder Exstrophy With or Without Osteotomy
S. M. Borhan Uddin, Md. Abu Bakar Akan, Rajib Khastgir, Md. Ruhul Amin
63
Evaluation of Congenital Upper Urinary Tract Anomalies in Hypospadias
Md. Abdullah Al Farooq, Shafiqul Hoque
68
Decreased Morbidity and Mortality from Intestinal Ascariasis: Experience of a Single Center
Md. Akbar Hossain Bhuyian, Md. Abdullah Al Farooq, Md. Minhajuddin Sajid, MA Mushfiqur Rahman, Md. Momtazul Hoque, Khurshid Alam Sarwar, Tanvir Kabir Chowdhury, Mahfuzul Kabir, Tahmina Banu
73
Case Reports
Desmoplastic Small Round Cell Tumour (DSRCT) - A Case Report and Review of Literature
AKM Amirul Morshed, Farhana Afroz, Chowdhury Md. Fuad Galib, Shahnoor Islam, Kaniz Hasina, Ashish Kumar Ghosh
79
Spontaneous Common Bile Duct Perforation on The Background of Pancreatic Lithiasis: A Case Report and The Review of The Literatures
Md. Abdullah Al Farooq, M A Mushfiqur Rahman, Mahfuzul Kabir, Md. Akbar Husain Bhuiyan, Md. Minhajuddin Sajid, Rupam Talukder, Tanvir Kabir Chowdhury, Tahmina Banu
83
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ASSOCIATION OF PAEDIATRIC SURGEONS OF BANGLADESH (APSB)
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Authors

  • AKM Amirul MorshedDepartment of Pediatric Haematology and Oncology, Dhaka Medical College & Hospital, Dhaka
  • Farhana AfrozDepartment of Pediatric Haematology and Oncology, Dhaka Medical College & Hospital, Dhaka
  • Chowdhury Md. Fuad GalibDepartment of Pediatric Haematology and Oncology, Dhaka Medical College & Hospital, Dhaka
  • Shahnoor IslamDepartment of Pediatric Surgery, Dhaka Medical College & Hospital, Dhaka
  • Kaniz HasinaDepartment of Pediatric Surgery, Dhaka Medical College & Hospital, Dhaka
  • Ashish Kumar GhoshDepartment of Pediatric Haematology and Oncology, Dhaka Medical College & Hospital, Dhaka

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.

Journal of Paediatric Surgeons of Bangladesh (2011) Vol. 2 (2): 79–82

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