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Head pleomorphic sarcoma showing murine double minute 2 amplification without a well-differentiated liposarcoma
Mitsuko Akaihata1, Ikuko Takahashi1, Yuko Kakuda2
1Division of Pediatrics, Shizuoka Cancer Center, Shizuoka, Japan.
Background:
Murine double minute 2 (MDM2) is an oncogene that inhibits p53, leading to decreased apoptosis. Sarcomas showing MDM2 amplification are rare among pediatric patients.
Case:
A 14-year-old boy presented with pleomorphic sarcoma of the head showing MDM2 amplification without a well-differentiated liposarcoma component. Although chemotherapy was initially performed to reduce the tumor size before surgery, the tumor did not shrink. The patient underwent complete surgical resection. Microscopic examination revealed a positive surgical margin; thus, postoperative proton-beam radiotherapy was performed. 3 years after the therapy, no sign of recurrence was observed.
Conclusion:
Macroscopic surgical resection combined with adjuvant postoperative radiotherapy was effective against MDM2-amplified pleomorphic sarcoma refractory to neoadjuvant chemotherapy in a pediatric patient.
