Malignant transformation of cervical ostochondroma in patient with hereditary multiple exostoses (HME): Case report and review of the literature

Abstract

Objectives: Hereditary multiple exosostes (HME) is a disorder characterized by the presence of multiple osteochondromas of the bone. Spinal involvement is rare, represent roughly 3% of cases and any portion of the vertebral body may be affected. Malignant transformation of the lesions is rare, but it is possible and is described in literature.
Methods: We describe an unusual case of malignant transformation of a cervical osteochondromas arising from the articular complex, in a young girl affected by HME. The patient underwent surgery with a complete removal of the lesion, without any signs of recurrence seen at the MRI serial control at 3, 6 12 e 24 months. We analyzed the literature up to 2009 by focusing on the treatment and follow up.
Results: Surgical removal is indicated for symptomatic spinal osteochondormas. The suspicion of malignant transformation is indicated by the sudden growth of the lesion after puberty, the presence of pain and the worsening of the neurological symptoms.
Conclusions: In this case, radical surgery is imperative, given the close correlation with the malignant characteristics of the lesions, it is useful to have an extemporaneous histological examination. In the rare cases of malignant transformation, radical surgery is important for the prognosis and to prevent the progression of the lesions. If the complete removal is not possible, the therapy of choice is adjuvant radiotherapy.