|
Year |
2002 |
|---|---|
|
Question Number |
62 |
|
Category |
Pediatric Orthopaedics |
Question
What lesion typically found in an infant is believed to be a precursor to adult adamantinoma?
1- osteofibrous dysplasia
2- desmoplastic fibroma
3- nonossifying fibroma
4- congenital pseudoarthrosis of the tibia
5- multiple enchondromatosis (Ollier’s disease)
Figures
Answer (click for answer)
Preferred response: 1.
Adamantinoma is a malignant bone tumor most commonly seen in the tibia of adults 30-50 years of age Treatment is wide excision. Osteofibrous dysplasia is a benign bone tumor usually found in the anterior aspect of the tibia in a child 1-5 years of age. Treatment is observation. There has been a reported association between osteofibrous dysplasia and adamantinoma. In the referenced article they state "…osteofibrous dysplasia is either the benign counterpart of a neoplastic process that produces an adamantinoma or that it is the residual of a spontaneously regressing adamantinoma." They reviewed the radiographs and histological samples of 32 patients who underwent biopsy or resection of lesions originally diagnosed as ossifying fibroma, fibrous dysplasia, osteofibrous dysplasia and adamantinoma. What they found was that the initial diagnosis was different than the diagnosis they made after further review in 19 patients. Six of these patients ended up with a diagnosis of adamantinoma that wasn’t originally determined. Their point was that osteofibrous dysplasia should be observed very closely because it may already be or turn into an adamantinoma. Desmoplastic fibroma is a benign bone tumor that is locally aggressive and it is treated with wide excision. Nonossifying fibroma is a benign bone tumor usually found in the posterior aspect of the distal femur in patients up to 20 years of age. Treatment is curettage and bone grafting. Congenital pseudarthrosis of the tibia is a nonunion of the tibia at birth that causes tibial bowing. It is associated with neurofibromatosis. Ollier’s disease is a benign process of patients age 10-30 years in which multiple enchondromas are seen. There is potential for malignant transformation to chondrosarcoma.
Reference: Springfield DS, Rosenberg AE, Mankin HJ, Mindell ER: Relationship between osteofibrous dysplasia and adamantinoma. Clin Orthop 1994;309:234-244.