Table of Contents    
Case Report
 
Embryonal rhabdomyosarcoma of liver in a 16-year-old boy: A rare case report
Rajamahendran Rajendran1, Amudhan A.2, Prabhakaran R.2, Kannan D.3 Chandramohan S.M.4
1Post Graduate in Surgical Gastroenterology, Institute of Surgical Gastroenterology, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India.
2Assistant Professor in Surgical Gastroenterology, Institute of Surgical Gastroenterology, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India.
3Professor of Surgical Gastroenterology, Institute of Surgical Gastroenterology, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India.
4Head of Department of Surgcial Gastroenterology, Institute of Surgical Gastroenterology, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India.

Article ID: 100021IJHPDRR2014
doi:10.5348/ijhpd-2014-22-CR-10

Address correspondence to:
Professor D. Kannan
No. 245, Tower 2, 4th Floor, Rajiv Gandhi Government General Hospital
Chennai-600003, Tamil Nadu
India
Phone: 919787387183
Email: minnalraja@gmail.com

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How to cite this article:
R Rajamahendran, Amudhan A, Prabhakaran R, Kannan D, Chandramohan SM. Embryonal rhabdomyosarcoma of liver in a 16-year-old boy: A rare case report. Int J Hepatobiliary Pancreat Dis 2014;4:52–56.


Abstract
Introduction: Primitive mesenchymal tumors represent about 9–15% of all hepatic tumors in children. Only about 150 cases have been reported in literature so far. Herein, we report a case of ruptured embryonal rhabdomyosarcoma.
Case Report: A 16-year-old boy, with weight of 26 kg, presented to us with sudden onset of abdominal pain and fever. Clinical examination revealed a tender hepatomegaly about 6 cm below right costal margin. Contrast-enhanced computed tomography scan of abdomen revealed 15x11x9 cm heterogenous multi-septated loculated mass lesion involving right lobe of liver involving segments 5, 6, 7, 8. Diagnostic laparoscopy showed a tumor occupying the entire right lobe of liver with no other deposits. The tumor capsule was seen ruptured on the right lateral side for which we did emergency right hepatectomy. Weight of the specimen was 2.8 kg. Histopathology revealed the lesion as embryonal rhabdomyosarcoma with co-existing mesenchymal hamartoma. Immunohistochemistry showed Vimentin and Desmin positive. Patient was on carboplatin and Ifosfamide chemotherapy. Patient expired on 30th postoperative day due to acute respiratory distress.
Conclusion: Improved survival can be expected only if we detect the disease early. This case report will enlighten this type of rare tumor in young children and an early referral to the specialist.

Keywords: Primary embryonal rhabdomyosarcoma, Heterogenous mass in liver, Ruptured capsule, Right Hepatectomy, Liver


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Author Contributions
Rajamahendran Rajendran – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Amudhan A. – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Prabhakaran R. – Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published
Kannan D. – Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published
Chandramohan S.M. – Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2014 Rajamahendran Rajendran et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.