Abstract
Osteofibrous dysplasia (OFD) is benign lesion that affects long bones in children. Adamantinoma (ADA) is a rare malignancy. A new entity known as “differentiated” adamantinoma or OFD-like ADA (OFDLA) has emerged. We examined the clinical outcomes of patients with these conditions.
A single-institution chart review of 59 patients from 1958-2016 was performed. Patient and treatment characteristics were compared across tumor types. Logistic regression was used to assess differences in outcomes including receipt of surgery, local recurrence, and development of metastatic disease.
Forty-three patients were diagnosed with OFD, 17 were treated surgically, and there were 6 local recurrences. Out of the 9 OFDLA patients, 7 were treated surgically, and there were 4 local recurrences. All 5 patients with ADA were treated with wide excision, there was one local recurrence treated with re-excision, and one patient developed metastatic disease after treatment. OFDLA patients were more than four times as likely to undergo surgery compared to OFD patients (OR=4.5, 95%CI=1.1-26.6, p=0.04). There were no differences between OFD and OFDLA patients in surgical outcomes.
OFD and OFDLA patients can be managed conservatively, with surgical intervention reserved for those with radiographically extensive or symptomatic lesions. ADA should continue to be treated with wide local resection.
Level III (retrospective cohort study)