RESUMEN
Bizarre parosteal osteochondromatous proliferation (BPOP) is an unusual lesion mostly affecting the bones of the hand. The mass grows from the bone surface and consists of cartilaginous, osseous and fibrous tissue. The lesion is commonly under/misdiagnosed and confused with other lesions, mostly the osteochondromas. We present a patient with BPOP that initially confused the practitioner and radiologist in their diagnosis. We discuss the clinical, radiologic and histologic characteristics of BPOP of the hand since its first report in 1983 and present its main differential diagnosis. We reviewed 184 cases. Female were affected in 52% and male in 48%. Pro ximal phalanges were most commonly affected, followed by middle phalanges and metacarpals. Pain was reported in 47,9 % of all reported papers. The most common surgical treatment was by excision, and the rate of recurrence was 47.3%.
Asunto(s)
Neoplasias Óseas/fisiopatología , Neoplasias Óseas/cirugía , Cartílago/cirugía , Proliferación Celular , Mano/fisiopatología , Recurrencia Local de Neoplasia/cirugía , Osteocondroma/cirugía , Neoplasias Óseas/diagnóstico , Femenino , Humanos , Masculino , Recurrencia Local de Neoplasia/diagnóstico , Osteocondroma/diagnósticoRESUMEN
Winchester syndrome (WS) is a rare autosomal recessive syndrome resulting in multicentric osteolysis. Only a few cases of WS have been described in the literature worldwide. It has recently been shown to be caused by mutation in the gene encoding matrix metalloproteinase-2 (MMP2). We report a patient affected by WS with a proven mutation of the MMP2 gene and describe the progression of radiological findings over a 23-year period. To our knowledge there is no comparable article concerning the WS in the literature.