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Maffucci's Syndrome Involving Hemangioma in the Cervical Spine

Akagi, Shigeo, MD, PhD; Saito, Takanori, MD, PhD; Ogawa, Ryokei, MD, PhD

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Study Design This is a case report of a patient with Maffucci's syndrome, with hemangioma located in the cervical spine causing paraplegia.

Objective To present a rare case of Maffucci's syndrome with hemangioma located in the cervical spine, and to discuss the incidence and treatment of hemangioma located in the central nerve system in Maffucci's syndrome.

Summary of Background Data Maffucci's syndrome is characterized by enchondromatosis and soft tissue hemangioma. Hemangioma usually is located in the subcutaneous tissue and rarely in the mucosa and visceral organs. There have been no reports in the literature on the incidence of hemangioma in the cervical spine occurring with Maffucci's syndrome.

Methods The patient was an 11-year-old girl, who complained of muscle weakness of the upper extremity and gait disturbance. After clinical and radiologic evaluation, the patient was diagnosed as having dumbbell tumor of the cervical spine associated with Maffucci's syndrome.

Results The tumor was successfully excised after selective angiography and embolization of the feeding artery from the ascending cervical artery and costocervical trunk, the result being excellent neurologic recovery for the patient. Histologic study showed the tumor was hemangioma similar to the red-blue tissue tumor in the hand.

Conclusion The possibility of hemangioma being localized in the spinal column, as well as in the subcutaneous tissue, mucosa, and visceral organ, should be considered in cases of Maffucci's syndrome.

From Department of Orthopaedic Surgery, Kansai Medical University, Osaka, Japan.

© Lippincott-Raven Publishers.