A case of myelopathy because of enchondromas from Maffucci syndrome with successful surgical treatment.

BACKGROUND CONTEXT: Maffucci syndrome is a rare disorder comprising multiple enchondromas associated with multiple hemangiomas. Less than 200 cases have been reported in the literature. Most reported cases comprised lesions of the long bone metaphyses, hands, and feet. No previous case of myelopathy due to enchondroma from Maffucci syndrome has been reported. PURPOSE: To highlight an interesting and rare presentation of thoracic myelopathy because of enchondromas from Maffucci syndrome. STUDY DESIGN: This is a case report of a single patient presenting with myelopathy in whom enchondromas from Maffucci syndrome were removed from the spinal canal with the return of normal function. METHODS: Clinical examination, magnetic resonance imaging, surgical resection, and histologic analysis. RESULTS: The patient experiencing myelopathy symptoms underwent a surgical resection of the offending lesions that resulted in return to normal function. CONCLUSIONS: Maffucci syndrome is a rare condition; however, in affected patients the possibility of structural spinal abnormalities causing cord compression must be considered, as discrete surgical resection can result in a good outcome.

Lumbar hemangioma masking a plasma cell tumor--case report and review of the literature.

BACKGROUND CONTEXT: Vertebral hemangiomata are ubiquitous bone tumors. Often multiple, they are generally benign in nature and slow growing. They typically have a predictable radiographic appearance. Occasionally, hemangiomata may behave in a more aggressive manner, causing pathologic fracture or even symptoms/signs of nerve compression. In such cases, one must be careful not to assume that an atypical hemangioma is responsible. Coexisting, more malignant processes may be present and sometimes may be radiographically undetectable in the setting of acute fracture. This was the case in our patient. STUDY DESIGN: Case report/university spine surgery center. METHODS: The patient underwent a corpectomy of her affected vertebra with conversion to a total spondylectomy when intraoperative frozen section was consistent with plasma cell neoplasm. A reconstruction with vertebral body replacement and fusion through anterior and posterior approaches was completed. Subsequently, the literature was reviewed for other cases of atypical hemangiomata to investigate the incidence of coexistent lesions. RESULTS: This patient presented with pain secondary to an unstable pathologic vertebral body fracture. Surgery to stabilize her spine was elected. Intraoperative recognition of abnormal-appearing tissue led to the diagnosis of a plasma cell neoplasm that was not seen on imaging. Coexistent in the same vertebra was hemangiomatous tissue that was visible on preoperative imaging. CONCLUSIONS: There are rare reports of aggressively behaving hemangiomata that mainly have occurred in the thoracic spine. There have been no reports of the coexistence of a hemangioma and a plasma cell tumor in the same vertebral level in the setting of acute compression fracture.