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Epithelioid Inflammatory Myofibroblastic Sarcoma with Leukemoid Reaction
Yuan-Yuan Wan1, Cheng-Li Miao2, Shi-Bo Liu2
1Department of Pediatrics, Peking University International Hospital, Beijing, China.
This report details the first case of epithelioid inflammatory myofibroblastic sarcoma (EIMS) with leukemoid reaction and pancreatic infiltration. Early detection and targeted therapies like crizotinib show promise for this rare sarcoma.
Area of Science:
- Oncology
- Pathology
Background:
- Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare, aggressive variant of inflammatory myofibroblastic tumour (IMT).
- Standard treatment protocols for EIMS are not well-established.
Observation:
- A 14-year-old male presented with a large retroperitoneal mass and a significantly elevated white blood cell (WBC) count (85×10^9/L), indicative of a leukemoid reaction.
- The initial presentation included pancreatic infiltration, a previously undocumented feature at disease onset for EIMS.
Findings:
- Histopathology and bone marrow aspiration confirmed EIMS with a concurrent leukemoid reaction.
- Tumour recurrence with hepatic and pulmonary metastasis occurred one month post-surgery, accompanied by a progressive increase in WBC count.
Implications:
- The consistent presence of ALK rearrangement in EIMS suggests targeted therapies may be beneficial.
- Combining surgical resection with ALK inhibitors like crizotinib could potentially improve outcomes for EIMS patients.
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