Nonoperative Management of Multiple Hand Enchondromas in Ollier Disease With Progressive Ossification
Ollier disease, or multiple enchondromatosis, has a distinct hallmark of disease variability and requires individualized patient treatment. This is a case report of an 18-year-old woman with Ollier disease isolated to her left hand that was treated nonoperatively for 8 years and followed with serial radiographs. Prognosis of Ollier disease limited to the bones of the hands is believed to be very good: only 12 cases of transformation to chondrosarcoma have been reported in the literature. However, a recent large retrospective multi-institutional study estimated a 15% lifetime risk of developing a malignancy at these locations.
Histologic analysis of biopsied or surgically treated lesions contributes to the differentiation between benign hand enchondromas and chondrosarcoma. Pathologic findings must be correlated with clinical and radiographic findings because hand enchondromas contain cytologic features of chondrosarcoma.12 In a series of 55 patients with chondrosarcoma, Liu and colleagues8 reported no cases from the hand. Verdegaal and colleagues7 reported a total of 13 chondrosarcomas in the metacarpals and hand phalanges in 97 group I and III patients. Five of these lesions were grade 1, 2 were grade 2, 1 was grade 3, and 5 lesions were unknown.
For patients with multiple enchondromatosis limited to the hands, prognosis is relatively good with respect to risk of secondary chondrosarcoma transformation, metastasis of secondary chondrosarcoma, and death. Verdegaal and colleagues7 reported the rate of secondary transformation in the hand to be 15%. Patil and colleagues13 reported no distant metastases in 23 patients with hand chondrosarcoma at mean follow-up of 8.5 years (range, 2-19 years), although none of their patients had Ollier disease. Verdegaal and colleagues7 reported 7 of the 8 deaths in their study were related to development of pulmonary metastases; however, none originated from chondrosarcomas in the hand. Additionally, there were no disease-related deaths in 29 group I patients. Herget and colleagues,14 in summarizing the literature, postulated that the overall survival rate of patients with secondary chondrosarcoma at 5 years is approximately 90%.
In our case, the patient, who had 3 enchondromas isolated to the left hand, can be categorized in group I. Thus, this case highlights the natural history of a patient with hand enchondromas and demonstrates that enchondromatosis of the short tubular bones of the hands can mature and ossify.
