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Answer: Ollier's Disease.

Discussion:

Enchondromas are benign cartilaginous tumors that develop in close proximity to growth plate cartilage.

When multiple enchondromas are present, the disease is termed enchondromatosis or Ollier disease (WHO terminology) - named after the French surgeon Louis Leopold Ollier (1830-1900).

Clinical manifestations typically appear during the first decade of life and are characterized by an asymmetric distribution of cartilage lesions that result in varying degrees of skeletal deformity based on their number, size, and location.

The most common complications associated with the disease include limb-length discrepancy and the potential for malignant transformation to chondrosarcoma.

Fortunately, Ollier disease is very uncommon occuring in isolated patients with an estimated incidence of 1/100,000.

Diagnosis is based on clinical and radiolographic evaluation with histological analysis reserved for evaluation of suspected malignant transformation.

Imaging evaluation of enchondromas typically reveals expansile lytic lesions in the metaphyseal region of tubular bones with internal chondroid matrix.

To date there are no known medical treatments for enchondromatosis with surgery reserved for management of complications (ie disfigurement, fractures, growth abnormalities, and malignant transformation).

References:

Resnick D, Kyriakos M, Greenway GD. Tumors and tumor-like lesions of bone: imaging and pathology of specific lesions. In: Resnick D, editor. Diagnosis of Bone and Joint Disorders. 4th ed. Vol. 4. Philadelphia: WB Saunders Co; 2002. pp. 3833-3848.

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