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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.
How common: no registry figure; under 1 percent of primary bone tumours in the older literature the review cites (Orthopedics 2007).
Treatment: wide en bloc resection with reconstruction, or amputation where resection is impossible; no role for chemotherapy or radiotherapy; lifelong follow-up because recurrence and lung metastasis can occur decades later (Journal of Surgical Oncology 2018; Orthopedics 2007).
Very rare: a single institution saw 46 histologically confirmed cases between 1939 and 2012, 31 of them in the tibia, mean age 24 (Journal of Surgical Oncology 2018). No registry figure exists.
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
Wide en bloc resection with reconstruction; no chemotherapy or radiotherapy; decades of follow-up for late recurrence and lung metastasis.
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Query for this cancer: (TITLE:"Adamantinoma of bone" OR ABSTRACT:"Adamantinoma of bone" OR TITLE:"Adamantinoma of the long bones" OR ABSTRACT:"Adamantinoma of the long bones" OR TITLE:"Adamantinoma of the tibia" OR ABSTRACT:"Adamantinoma of the tibia" OR TITLE:"Osteofibrous dysplasia-like adamantinoma" OR ABSTRACT:"Osteofibrous dysplasia-like adamantinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Adamantinoma of bone, not a curated reading list.
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