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.
Multiple Histiocytic Fibromas with Extraskeletal Abnormalities (Jaffe-Campanacci’s Syndrome)
RUGGIERI, PIETRO
2014
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.
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