- See: Bone Tumor Menu                                                                                           
- Discussion:
    - rare tumor of unknown origin;
    - occurs primarily in young males between 10 - 30 years of age (most common after skeletal maturity);
    - pts present w/ firm, slowly enlarging mass that produces minimal disability;
           - on other occassions the presenting features will be pain, swelling or pathologic fracture;
    - sites of involvement:
           - mandible or tibial diaphysis is involved in 90% of pts;
           - occassionally tumor arises in forearm, hands, or feet;
    - histology:
           - multiple biopsies may be required to have an representative sample;
           - look for a biphasic pattern of glandular epithelial cells which are surrounded by fibrous spindle cells;
           - epithelial cells may be grouped in nests and stain positive for keratin;
   - diff dx:
           - chondromyxoid fibroma
           - nonossifying fibroma
           - fibrous dysplasia
           - osteofibrous dysplasia:
                  - occurs most often in skeletally immature patients;
                  - most often involves the anterior cortex of the tibial diaphysis;
                  - minor anterior bowing of the tibia is frequently seen
                  - extensive histologic evaluation is necessary to distinguish lesions in the spectrum between osteofibrous dysplasia and adamantinoma;
                  - in some patients, this lesion will progress to full blown adamantinoma;
                  - there is some indication that curretage or marginal excision should not be undertaken because of high local recurrance rate;
                  - references:
                          - Osteofibrous dysplasia (ossifying fibroma of long bones): a report of four cases and review of the literature.  
                          - Osteofibrous dysplasia of the tibia and fibula.

- Treatment:

    - because these tumors are slow growing and have a limited potential for local recurrence or metastasis;
    - marginal resection: delayed local recurrence can be expected in 32%;
    - en bloc resection: local recurrence should not occur;
    - mean survival w/ metastatic disease: 12 years;
    - in report by Qureshi AA, et al (2000), authors examined the role of en bloc excision on 70 patients was examined;
           - limb salvage was attempted in 91 percent (sixty-four) of the seventy patients, and the final rate of limb preservation was 84 % (59 of 70);
           - wide operative margins were obtained in 92% (58) of 63 patients;
           - intercalary allograft was used to reconstruct the segmental bone defect in 51% (36) of the 70 patients;
           - reconstruction-related complications occurred in 48% (30) of 62 patients;
           - nonunion and fracture were the most common complications, occurring in 24% (15) and 23% (14) of 62 patients, respectively.
           - Kaplan-Meier analysis demonstrated a rate of local recurrence of 18.6 percent at ten years.
    - XRT: adamantinoma is highly radioresistant
    - chemotherapy has not been shown to be effective;
    - Current trends in the management of adamantinoma of long bones. An international study.

Ten Most Common Bone and Joint Tumors--General Orthopaedics: Adamantinoma of the Appendicular Skeleton--Updated.

The treatment of adamantinoma of the tibia by wide resection and allograft bone transplantation.

Adamantinoma of the tibia masked by fibrous dysplasia. Report of three cases.

Adamantinoma of long bones. A clinicopathological study of fourteen cases with vascular origin suggested.

Adamantinoma of the long bones. A clinicopathological study of thirty-two patients with emphasis on histological subtype, precursor lesion, and biological behavior.


Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Monday, January 5, 2015 1:58 pm