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4 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Adamantinoma is a very rare, slow-growing bone cancer of young adults that almost always affects the shin bone, made of epithelial cells inside fibrous bone, and listed with the bone sarcomas in the WHO classification. It is cured by cutting it out with a margin; chemotherapy and radiotherapy do not work, and a minority spread to the lungs many years later.
Adamantinoma is a low-grade malignant biphasic bone tumour of epithelial cells in an osteofibrous stroma, classified with the bone tumours of uncertain differentiation in the WHO soft tissue and bone classification; the classic form is distinguished from osteofibrous dysplasia-like (differentiated) adamantinoma, which occurs in children and rarely metastasises (Orthopedics 2007). In 46 consecutive patients followed for a mean of 16 years, the tibia was the commonest site (31), patients presented with pain and swelling at a mean age of 24, 37 had limb-salvage surgery, 39 percent needed reoperation, and ten-year disease-specific and recurrence-free survival were 92 and 72 percent, with three recurrences more than 15 years after treatment (Journal of Surgical Oncology 2018).
How it differs from its parent: the sarcoma page covers the mesenchymal cancers of bone and soft tissue; adamantinoma is epithelial in lineage, indolent, radiologically distinctive (a multilocular lytic lesion of the anterior tibial cortex) and insensitive to the chemotherapy used for osteosarcoma and Ewing sarcoma.
| Setting | Approach | Guideline |
|---|---|---|
| All cases | Wide en bloc resection with reconstruction; no chemotherapy or radiotherapy; decades of follow-up for late recurrence and lung metastasis. | not mapped |