Osteofibrous dysplasia, osteofibrous Dysplasia-Like adamantinoma, and adamantinoma: A Single-center retrospective analysis

Abstract

Objective: This study aimed to comprehensively investigate and contrast the imaging manifestations, pathological features, surgical interventions, and prognostic outcomes of Osteofibrous Dysplasia(OFD), Osteofibrous Dysplasia-Like Adamantinoma(OFD-AD), and Adamantinoma(AD). By synthesizing disease profiles and exploring their evolutionary relationships, we sought to identify more effective diagnostic and therapeutic strategies for these conditions.

Methods: A retrospective analysis was conducted on patients diagnosed with OFD, OFD-AD, or AD at our institution between 2015 and 2022. The analysis included a detailed comparison of clinical presentations, imaging findings, and pathological characteristics. We also evaluated the impact of different diagnostic and treatment modalities on patient prognosis and explored potential disease evolution and transformation patterns.

Results: Fifty patients were included in this study: 16 with OFD, 27 with OFD-AD, and 7 with AD. The median age of onset was 14 years for OFD, 6 years for OFD-AD, and 33 years for AD. All diagnoses were confirmed through a combination of clinical evaluation, imaging (X-rays and MRI), and pathological examination. Among the patients, 2 (both with OFD-AD) were managed with observation only. Thirty-seven patients underwent intralesional resection (16 OFD, 20 OFD-AD, and 1 AD), and 11 patients had complete resection (5 OFD-AD and 6 AD). After a minimum follow-up of 24 months (range: 24---90 months, median: 56 months), 12 patients experienced tumor recurrence (OFD: 2/16, 12.5 %; OFD-AD: 9/25, 36 %; AD: 1/6, 17 %). One patient had concurrent OFD-AD in the fibula and AD in the tibia. In another case, an OFD-AD recurrence 4 years after surgery was later diagnosed as OFD, and an OFD recurrence 2 years after surgery was reclassified as OFD-AD. No distant metastases were observed in any patient.

Conclusion: OFD, OFD-AD, and AD exhibit similarities in clinical and imaging presentations, and their pathological features may represent different stages of a common lesion's evolution. These diseases have distinct age - related onset patterns and variable recurrence risks. Thus, accurate diagnosis and personalized treatment strategies based on patient characteristics are crucial for effective disease management.

Keywords: Adamantinoma; Differential diagnosis; Osteofibrous Dysplasia; Osteofibrous Dysplasia-Like Adamantinoma; Therapeutic strategy.

Fig. 1

The three different diseases share similar radiological manifestations. A. All three diseases tend to occur in the tibia, causing irregular osteolytic destruction in the middle segment of the tibia, and can simultaneously involve the cortical bone and the medullary cavity. B. All three diseases can also affect both the tibia and fibula simultaneously, leading to bone deformities.

Fig. 2

From pathological view, they all have same osteofibrous background, and varies on distribution of epithelial component. We classified them according to the number of epithelial cells under light microscopy and the distribution of CK+(pan-cytokeratin, broad-spectrum, ready-to-use)clusters in IHC tests.

Fig. 3

The 9-year-old girl diagnosed as OFD in tibia had a recurrent lesion confirmed as OFD-AD 4 years after curettage surgery (Case 1).

Fig. 4

This 4-year-old boy who had a segmental resection of OFD-AD in the fibula (A) had developed a new lesion (B) in the ipsilateral tibia three years later (Case 2).

Fig. 5

This is a 28-year-old female, who had lesions in both tibia and fibula. The pathological test showed completely different features, the final diagnosis was OFD-AD in the fibula and classic AD in the tibia. （Case 3）.