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Multiple peripheral nerve compressions related to malignantly transformed hereditary multiple exostoses
1Department of Rehabilitation Medicine, Seoul National University College of Medicine, Republic of Korea. rehabdoc@plaza.snu.ac.kr
Muscle & Nerve
|August 3, 2000
Summary
Hereditary multiple exostoses can lead to nerve compression. This case highlights chondrosarcoma transformation from osteochondromas, causing significant neuropathy and requiring surgical intervention.
Area of Science:
- Orthopedics
- Neurology
- Oncology
Background:
- Hereditary multiple exostoses (HME) is a rare autosomal dominant disorder characterized by bone spurs capped with cartilage.
- Lesions typically occur in long bone metaphyses but can affect flat bones.
- Malignant transformation, specifically chondrosarcoma, is a known complication in 1-25% of HME patients.
Observation:
- A 33-year-old male with HME presented with sciatica and multiple peripheral nerve compressions.
- Electrodiagnostic studies revealed severe axon-loss neuropathies affecting sciatic, superior gluteal, and inferior gluteal nerves.
- MRI identified a large sacral mass, suspected to be chondrosarcoma.
Findings:
- Biopsy confirmed chondrosarcoma arising from a chondromatosis within the sacral mass.
- Surgical excision relieved nerve impingement and halted further bone growth.
- This case underscores osteochondroma as a potential cause of compression neuropathy.
Implications:
- Malignant transformation should be suspected in HME patients experiencing focal compression neuropathy.
- Early diagnosis and intervention are crucial for managing complications of HME.
- Further research into the mechanisms of sarcomatous transformation in HME is warranted.