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Pediatric Chondromyxoid Fibroma-Like Osteosarcoma
Matthew Stark1, Stephen D Heinrich2, Raju Sivashanmugam3
1a Department of Pathology , Children's Hospital New Orleans , New Orleans , LA , USA.
Fetal and Pediatric Pathology
|December 10, 2016
Summary
Chondromyxoid fibroma-like osteosarcoma (CMF-OS), a rare low-grade bone cancer, can be mistaken for benign tumors. This case highlights its aggressive features and positive response to chemotherapy.
Area of Science:
- Orthopedic Oncology
- Pediatric Oncology
- Skeletal Radiology
Background:
- Chondromyxoid fibroma-like osteosarcoma (CMF-OS) is a rare, low-grade malignant bone tumor.
- CMF-OS is frequently misdiagnosed as a benign lesion on initial biopsy.
- Previous literature describes only five cases of CMF-OS.
Observation:
- A 13-year-old male presented with an intramedullary CMF-OS in the proximal right tibia.
- The tumor exhibited lytic properties, cortical destruction, and soft tissue extension.
- Radiological and pathological findings confirmed malignant new bone formation and bony destruction.
Findings:
- The diagnosis was confirmed by malignant new bone formation, increased mitotic figures, and lamellar bone permeation.
- No metastasis were present at the time of diagnosis.
- The tumor demonstrated a significant response to standard chemotherapy, achieving >95% necrosis.
Implications:
- This case expands the understanding of CMF-OS presentation and behavior.
- Accurate diagnosis requires careful correlation of imaging and histopathology.
- CMF-OS shows potential for good response to established osteosarcoma treatment protocols.
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