Osteofibrous dysplasia

Osteofibrous dysplasia is a rare, benign non-neoplastic condition with no known cause. It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula,^[1] and coined the term. In 1942, however, Jaffe and Lichtenstein described the same condition under the name "nonosteogenic fibroma",^[2] so the condition is often known as Jaffe-Campanacci Syndrome. The syndrome's exact nature is under debate, with some authors suggesting that it may be a manifestation of neurofibromatosis.^[3]

Presentation

The tibia is the most commonly involved bone, accounting for 85% of cases.^[4] It is usually painless, although there may be localized pain or fracture, and presents as a localized firm swelling of the tibia in children less than two decades old (median age for males 10, females 13^[5]). Several authors have related this non-neoplastic lesion to Adamantinoma, a tumor involving subcutaneous long bones, stating the common cause to be fibrovascular defect. However, the latter is distinguished from an osteofibrous dysplasia by the presence of soft tissue extension, intramedullary extension, periosteal reaction and presence of hyperchromic epithelial cells under the microscope.

Osteofibrous dysplasia may also be mistaken for fibrous dysplasia of bone, although osteofibrous dysplasia is more likely to show an immunohistochemical reaction to osteonectin, neurofibromin, and S-100 protein.^[5]

Treatment

Osteofibrous dysplasia is treated with marginal resection with or without bone grafting, depending on the size of the lesion and the extent of bony involvement. However, due to the high rate of recurrence in skeletally immature individuals, this procedure is usually postponed till skeletal maturity.

References

1. ^ Campanacci M (August 1976). "Osteofibrous dysplasia of long bones a new clinical entity". Ital J Orthop Traumatol 2 (2): 221–37. PMID 1024109. 
2. ^ Jaffe HL, Lichtenstein L (March 1942). "Non-osteogenic fibroma of bone". Am. J. Pathol. 18 (2): 205–221. PMC 2032933. PMID 19970624. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2032933. 
3. ^ Colby RS, Saul RA (November 2003). "Is Jaffe-Campanacci syndrome just a manifestation of neurofibromatosis type 1?". Am. J. Med. Genet. A 123A (1): 60–3. doi:10.1002/ajmg.a.20490. PMID 14556247. 
4. ^ Robert Mervyn Letts, Osteofibrous Dysplasia, http://emedicine.medscape.com/article/1256595-overview 
5. ^ ^{a b} Steven P. Meyers (2008). MRI of bone and soft tissue tumors and tumorlike lesions. Thieme. http://books.google.com/books?id=V-7y1nNatmoC&pg=PA654. 

External links

• E-medicine overview
• humpath