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Published on: January 31, 2016
Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report
Ritu Garg1, Sumaid Kaul1, Deepshikha Arora1
1Department of Histopathology, Apollo Hospital, New Delhi, India.
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare tumor in children post-transplant. This case highlights the first EIMS diagnosis in a liver transplant recipient, who achieved remission without chemotherapy.
Area of Science:
- Oncology
- Pediatric Pathology
- Transplant Surgery
Background:
- Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of inflammatory myofibroblastic tumor (IMT).
- EIMS typically affects children and young adults, with post-transplant occurrence being exceptionally rare.
- Factors like immunosuppression and viral infections are implicated in post-transplant IMT etiology.
Observation:
- A 2-year-old female, 8 months post-liver transplant for PFIC, developed multiple omental and mesenteric tumor nodules.
- Initial biopsy suggested a mesenchymal neoplasm; debulking was performed.
- Final histology confirmed EIMS in the resected tumor.
Findings:
- The patient achieved remission 7 months post-surgery without systemic chemotherapy.
- This represents the first reported case of EIMS in a pediatric liver transplant recipient.
- Only five cases of IMT post-solid organ transplant have been previously documented.
Implications:
- This case expands the understanding of EIMS in the context of solid organ transplantation.
- It suggests EIMS may occur in post-transplant settings, even in young children.
- Further research is needed to elucidate the etiology and metastatic potential of EIMS in transplant patients.
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