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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Mankin HJ, Trahan CA, Fondren G, Mankin CJ (2009) Non-ossifying fibroma, fibrous cortical defect and Jaffe-Campanacci syndrome: a biologic and clinical review. Chir Organi Mov 93(1):1–7. doi:10.1007/s12306-009-0016-4. Epub 2009 Apr 29. Review
Mirra JM, Gold RH, Rand F (1982) Disseminated nonossifying fibromas in association with café-au-lait spots (Jaffe-Campanacci syndrome). Clin Orthop Relat Res 168:192–205
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Ruggieri, P. (2014). Multiple Histiocytic Fibromas with Extraskeletal Abnormalities (Jaffe-Campanacci’s Syndrome). In: Picci, P., Manfrini, M., Fabbri, N., Gambarotti, M., Vanel, D. (eds) Atlas of Musculoskeletal Tumors and Tumorlike Lesions. Springer, Cham. https://doi.org/10.1007/978-3-319-01748-8_6
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DOI: https://doi.org/10.1007/978-3-319-01748-8_6
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