Case report
Year :
2024 |
Month :
July
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Volume :
13 |
Issue :
3 |
Page :
PC08 - PC12 |
Full Version
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Undifferentiated Embryonal Sarcoma of Liver- A Rare Case Entity
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Gayathri B Rajaraman, K Chandramouleeswari, M Dougul Regis, J Nivedita 1. Postgraduate Student, Department of Pathology, Institute of Pathology, Madras Medical College, Chennai, Tamil Nadu, India.
2. Professor and Head, Institute of Child Health and Hospital for Children, Madras Medical College, Chennai, Tamil Nadu, India.
3. Assistant Professor, Institute of Child Health and Hospital for Children, Madras Medical College, Chennai, Tamil Nadu, India.
4. Assistant Professor, Institute of Child Health and Hospital for Children, Madras Medical College, Chennai, Tamil Nadu, India.
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Correspondence
Address :
Gayathri B Rajaraman, K Chandramouleeswari, M Dougul Regis, J Nivedita, M Dougul Regis,
Assistant Professor, Department of Pathology, A block, No.15, Institute of Child
Health and Hospital for Children, Halls Road,Tamil Salai, EGMORE, Chennai-600008, Tamil Nadu, India.
E-mail: dougulregis@gmail.com
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| ABSTRACT | | : Undifferentiated Embryonal Sarcoma of the Liver (UESL) is a rare hepatic malignant mesenchymal neoplasm in the paediatric population, typically affecting children between 6-10 years of age without sex predilection. It accounts for 9-15% of paediatric liver malignancies, with an incidence of 0.6-1.2 cases per one million patients. Here, the authors present a case of an 11-year-old male, who presented with right upper quadrant pain, loss of appetite for the past one month, and high-grade fever for the past three days. On examination, a firm mass in the right hypochondrium and epigastrium, about 4 cm below the right costal margin, was noted, moving with respiration. Blood work showed normal values and normal alpha-fetoprotein levels. An ultrasonogram revealed a cystic lesion, while a contrast-enhanced Computed Tomography (CT) scan showed a large hepatic space-occupying lesion with cystic and solid architecture, with the following differential diagnosis as, embryonal sarcoma and hepatoblastoma. The patient underwent surgery for a hepatic hydatid cyst, and the procedure performed was percutaneous aspiration, irrigation and respiration. Gross examination revealed multiple grey-brown and grey-white fragments with a variegated appearance. Microscopic analysis showed a malignant neoplasm composed of spindle cells, stellate cells, multinucleated giant cells in a myxoid stroma, with many atypical mitoses present. Periodic Acid-Schiff (PAS) positive eosinophilic hyaline globules were observed in the tumour cell cytoplasm, along with extensive areas of haemorrhage and necrosis. The final diagnosis of embryonal sarcoma was made after a panel of Immunohistochemistry (IHC) markers. The present case is presented here due to its rarity and diagnostic challenge, arising from the lack of a characteristic clinical presentation, serological markers and inconclusive radiological findings. |
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Keywords
: Hepatic hydatid cyst, Malignant mesenchymal neoplasm, Tumour cell cytoplasm |
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DOI and Others
: DOI: 10.7860/NJLM/2024/69371.2859
Date of Submission: Dec 31, 2023
Date of Peer Review: Feb 01, 2024
Date of Acceptance: Mar 14, 2024
Date of Publishing: Jul 01, 2024
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Dec 31, 2023
• Manual Googling: Mar 06, 2024
• iThenticate Software: Mar 13, 2024 (20%)
ETYMOLOGY: Author Origin
EMENDATIONS: 7 |
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