Bizarre Parosteal Osteochondromatous Proliferation Revisited

Bizarre parosteal osteochondromatous proliferation (BPOP) is a rare benign surface-based bone lesion that primarily occurs in the hands and feet of young and early middle-aged adults. The lesion presents as a firm, usually painless mass of variable duration. Radiographs reveal a well-defined, pedunculated or sessile mass arising from the cortical surface of bone without altering the architecture of the underlying cortex. On magnetic resonance imaging, BPOP shows low to intermediate signal intensity on T1-weighted images and variable signal intensity on T2-weighted images. Marked enhancement is often seen after intravenous contrast administration. Histologically, the lesion is composed of an admixture of cartilage, bone and fibrous tissue. Between the cartilage and bone is a characteristic basophilic stroma, so called "blue bone". Immunohistochemistry does not play a significant role in the diagnosis of BPOP. Cytogenetic studies have demonstrated recurrent chromosomal abnormalities, including t(1;17)(q32-42;q21-23), inv(7)(q21.2-22q31.3-32) and inv(6)(p25q15). Most recently, gene rearrangements of collagen type I alpha 1 chain (COL1A1) or collagen type I alpha 2 chain (COL1A2) have been identified in a significant subset of cases. Surgical resection is the treatment of choice for symptomatic BPOP, but its local recurrence rate is relatively high. This review provides an updated overview of the clinicopathological, radiological, cytogenetic and molecular genetic features of BPOP and discusses the differential diagnosis of this uncommon lesion.