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Malignant extrarenal rhabdoid tumor: a case report
Kanchan Murhekar1, Sneha Suresh2, Shailesh Patidar3
1Department of Onco-Pathology, Cancer Institute (WIA), Adyar, Chennai, India. murhekarkanchan@gmail.com.
Journal of Medical Case Reports
|December 9, 2025
Summary
Malignant extrarenal rhabdoid tumor is a rare, aggressive sarcoma. Accurate diagnosis and early intervention are crucial due to its high recurrence risk despite multimodal therapy.
Area of Science:
- Oncology
- Pathology
- Surgical Oncology
Background:
- Malignant extrarenal rhabdoid tumor (MERRT) is a rare, aggressive soft tissue sarcoma primarily affecting infants.
- Diagnosis is challenging due to overlapping features with other rhabdoid tumors.
- MERRT requires careful differentiation from other neoplasms like epithelioid sarcoma and malignant peripheral nerve sheath tumor.
Purpose of the Study:
- To present a case of malignant extrarenal rhabdoid tumor in a 6-year-old girl.
- To highlight diagnostic challenges and immunohistochemical findings.
- To emphasize the importance of early diagnosis and intervention for aggressive MERRT.
Main Methods:
- Histopathological examination of a gluteal mass.
- Immunohistochemistry (IHC) for epithelial membrane antigen, vimentin, CD99, and INI1.
- Differential diagnosis exclusion using IHC.
- Multimodal treatment including wide local excision, radiation, and chemotherapy.
Main Results:
- Histopathology revealed rhabdoid morphology with cytoplasmic inclusions.
- IHC confirmed MERRT with characteristic marker expression and INI1 loss.
- The patient experienced foot drop and skin necrosis post-treatment.
- Surgical management was required for complications.
Conclusions:
- Accurate diagnosis of MERRT is critical for appropriate management.
- Early intervention is essential due to the aggressive nature and high recurrence risk.
- Multimodal therapy may not prevent disease progression or complications.
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