[Osteochondroma and multiple osteochondromas: recommendations on the diagnostics and follow-up with special consideration to the occurrence of secondary chondrosarcoma]

Abstract

Purpose: Osteochondroma represents the most common form of benign bone tumor. Clinical manifestations include deformity of bone, compression of surrounding tissue and vascular or neurological compromise. Osteochondromas may be solitary (solitary osteochondroma, SO) or multiple (multiple osteochondromas MO). Recurrence after surgery is a known problem especially in MO and malignant transformation is rare but more common in MO than in solitary cases. Reliable recommendations regarding diagnostics and clinical follow-up are currently lacking.

Patients and methods: A comprehensive literature review and a review of own patient files with SO/MO treated between 2000 and 2011 in this hospital were performed. The age of patients at diagnosis, tumor localization, clinical aspects, recurrence and the risk of malignant transformation in secondary (i.e. epiexostotic) chondrosarcoma were analyzed. The follow-up including patients who received surgery ranged between 2 and 127 months for patients with SO and between 2 and 84 months for MO.

Results: A total of 39 patients with SO from this hospital were included in the study. Out of 36 patients who received surgery 3 recurrences were registered after an average time of 62 months. In addition, 11 patients with MO were identified and all received surgery. In 5 out of 11 cases recurrences occurred after an average time of 20.6 months. Secondary chondrosarcomas were not recorded in this series. According to the literature an increased risk of malignant transformation was found for osteochondromas of the axial skeleton, in the proximal aspect of the extremities, as well as for recurrent tumors and for MO. Pain and/or increase in size of lesions after skeletal maturation were the most common clinical signs of transformation. There was a wide time interval between the initial diagnosis and the development of secondary chondrosarcoma. In MO secondary chondrosarcoma has been described before skeletal maturity.

Conclusions: The risk of malignant transformation of SO is generally low. Axial lesions as well as recurrent osteochondromas and MO seem to have an increased risk of malignant transformation. The follow-up, requiring sufficient primary diagnostics, includes regular self-control and can usually be clinically carried out in more peripherally located lesions but in certain cases supplementary X-ray imaging is needed. In cases of anatomical regions which are more difficult to access manually, follow-up examination by magnetic resonance imaging (MRI) is the method of choice. Especially MO patients seem to benefit from long-term follow-up: when the tumor is located in the trunk and in (proximal) long bones MRI or whole-body MRI, respectively, should be performed once a year after skeletal maturity because of the higher risk of malignant transformation in these patients.