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Adamantinoma


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- 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.

Adamantinoma