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Association of Paediatric Surgeons of Bangladesh
Journal of Paediatric Surgeons of Bangladesh
Case Reports · Vol. 7 No. 2 (2016)

Renal primitive neuroectodermal tumor (RPNET) in children

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JPSB
Journal of
Paediatric Surgeons of Bangladesh
Published : July 2016
CONTENTSVolume 07Number 2July 2016
Editorial
Subspeciality or super speciality in paediatric surgery – is it essential or luxury?
Md Tosaddeque Hossain Siddiqui
27
Review Articles
Tumor lysis syndrome in paediatric cancer patients
AKM Amirul Morshed, Shahnoor Islam
55
Original Articles
Interrelationship between food intake & nutritional anaemia among the children studied in a tertiary care hospital in Dhaka city
Md Delwar Hossain, Golam Mowlah, Monira Razzak
29
Nonligation and ligation of hernial sac technique during orchiopexy for palpable undescended testis in children: A comparative study
Md Mahbubul Alam, Partha Sarathy Majumder, Syed Abdul Adil, Md Abdul Aziz, Ashrarur Rahman
35
Serum lead level in children with acute lymphoblastic leukemia
AKM Amirul Morshed, Tasnim Ahmed, Tasrina Rabia Choudhury, Shahnoor Islam
41
Evaluation of the efficacy and safety of intralesional 5- fluorouracil in keloid
Masuda Khatun, M M Abu Sayed
46
The optimal biliary reconstruction for pancreaticobiliary maljunction and congenital biliary dilatation: Clinical comparison between hepaticojejunostomy and hepaticoduodenostomy
Akio Kubota, Yasuyuki Mitani
50
Case Reports
Fetus in fetu with jaundice- A rare presentation of a rare disease
Sifat Zereen Khan, Md Ashraf Ul Huq, Tahmina Hossain, Mohammed Moinul Huque, Syed Md Adil, Sahadeb Kumar Das, H M Zafar Sharif, S M Shafiqul Alam
61
Renal primitive neuroectodermal tumor (RPNET) in children
K M Didarul Islam, Umme Habiba Dilshad Munmun, Shoheli Alam, Noor Mahammad, Md Ruhul Amin, Sanjay Kumar Sah, Shirish Silwal
66
The Official Journal of the
ASSOCIATION OF PAEDIATRIC SURGEONS OF BANGLADESH (APSB)
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Authors

  • K M Didarul IslamAssociate Professor, Department of Paediatric Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • Umme Habiba Dilshad MunmunDepartment of Paediatric Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • Shoheli AlamAssistant Professor, Department of Paediatric Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • Noor MahammadDepartment of Paediatric Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • Md Ruhul AminProf. and Chairman, Department of Paediatric Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • Sanjay Kumar SahDepartment of Paediatric Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • Shirish SilwalDepartment of Paediatric Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Keywords

Renal PNET IHC

Abstract

Renal primitive neuroectodermal tumor (rPNET) as a member of Ewing’s sarcoma family is extremely rare and usually occurs in children and young adults. Most literature about rPNET was isolated case reports. Here we described a case of renal PNET in a 7-year-old girl who presented at our Paediatric Surgery Department of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh with fever, left flank pain and mass Ultrasonogram (USG) and Computer tomography (CT) scan revealed a huge mass in the left kidney. The patient underwent left radical nephrectomy with neoadjuvent chemotherapy on April,2016. Histopathology and immunohistochemistry (IHC) confirmed the diagnosis of PNET. She did not receive adjuvant chemotherapy, as advised. She had a local tumor recurrence 3 months after the surgery and continued to deteriorate but not developed distant metastasis. She again received 2-cycle of chemotherapy but size did not decrease. So re-do surgery was performed on 10.01.2017 and send to paediatric haemato-oncology department of BSMMU for further management. rPNET is rare and presents aggressive clinical behavior and worse prognosis. We expect that further awareness and study of this rare tumor can be had by presenting our case.

Journal of Paediatric Surgeons of Bangladesh (2016) Vol. 7 (2): 66–71

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