- 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;
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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 4 cases and review of the literature. CORR 1992. 278. p 235;
- Osteofibrous dysplasia of the tibia and fibula. J Bone Joint Surg Am 1981;63:367-375.
- 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 AA Qureshi MD et al (J Bone Joint Surg [Am] 82-A: 1122-31, 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;
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 clinicopathologic study of 32 patients with emphasis on histologic subtype, precursor lesion, and biologic behavior.
HM Hazelbag et al. JBJS Vol 76-A. 1994. p 1482-1499.
Adamantinoma of the appendicular skeleton: Updated. Clin Orthop Relat Res 1986;204:215-237.
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