(1)
2nd Orthopaedic and Traumatologic Clinic, Istituto Ortopedico Rizzoli, Bologna, Italy
Abstract
It is a very rare condition possibly linked to neurofibromatosis. Multiple large histiocytic fibromas extend to the long bones of one or both lower limbs or to the four limbs with prevalence in one side of the body and even including the pelvis. Café au lait spots are usually seen. Occasionally, mental retardation, hypogonadism or cryptorchidism, ocular and cardiovascular anomalies, and other skin alterations suggestive of neurofibromatosis are associated.
It is a very rare condition possibly linked to neurofibromatosis. Multiple large histiocytic fibromas extend to the long bones of one or both lower limbs or to the four limbs with prevalence in one side of the body and even including the pelvis. Café au lait spots are usually seen. Occasionally, mental retardation, hypogonadism or cryptorchidism, ocular and cardiovascular anomalies, and other skin alterations suggestive of neurofibromatosis are associated.
More frequently symptomatic, as compared to the usual histiocytic fibroma, they cause slight expansion of the bone, stress or pathologic fractures, and sometimes deformity or limb length discrepancy.
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