Atlas of Genetics and Cytogenetics in Oncology and Haematology (AGCOH) Adamantinoma of long bones is a low-grade, malignant biphasic tumor, characterized by a variety of morphological patterns, most commonly epithelial cells, surrounded by a relatively bland spindle-cell osteo-fibrous component. This paper should be referenced as such : Hazelbag HM, Hogendoorn PCW . Bone: Adamantinoma. Atlas Genet Cytogenet Oncol Haematol. January 2003 .

Adamantinoma - 1o bone tumor with predilection for tibial diaphysis presenting during early adulthood Signs & Symptoms: - painful masses with variable symptom duration ranging from few months to few years Xrays: - well-circumscribed, eccentric, lytic lesions of tibial diaphysis - may be multicystic lesion with surrounding sclerosis - expansion of bone +/- intact cortex but no periosteal reaction Histology: - wide variety of histologic appearances - basaloid pattern composed of groups of cells which peripherally have palisading epithelial cells - hypocellular, poorly organized, fibrous connective tissue between islands of epithelial cells Treatment: - wide excision (limb salvage vs amputation) - 20-25% metastasize to lungs

Radiographically, adamantinomas are sharply circumscribed lesions with lucent filling defects (central or eccentric in location). The tumors are separated from normal bone cortex by a sclerotic zone. Periosteal reaction is generally absent unless the lesion is accompanied by pathologic fracture. Gross pathology shows a well-demarcated lesion with peripheral lobulated contours. Cystic cavities contain either straw-colored fluid or blood, and the lesion may contain spicules of bone. The tumors are usually eccentrically located in the bone, sparing the medullary cavity. Microscopically, adamantinomas consist of epithelial-like cells in tubular or spindle configuration. The cells stain (immunohistochemical) strongly for keratin. The tumors are locally aggressive and metastesize to lung and local lymph nodes in 20% of cases. Treatment consists of wide-block excision of the involved segment of bone. The tumor readily recurs if not completely removed.

Definition adamantinoma of long bones,a rare tumor of limb bones, usually the tibia, that microscopically resembles an ameloblastoma; the histogenesis is uncertain. Description of radiological appearance, Histology and treatment.

Adamantinoma is a rare tumor, and its origin remains controversial. Fischer first described the tumor in 1913, and since then, only approximately 200 cases have been reported. The tumor occurs almost exclusively in the long bones; tumors in the tibia account for more than 80% of cases. The diaphyseal region is the area most commonly affected.

J Clin Pathol. 1982 July; 35(7): 780–786. Adamantinoma of long bones: clinical, pathological and ultrastructural features. A S Pieterse, P S Smith, and J McClure Abstract The clinical, pathological and ultrastructural features of two cases of tibial adamantinoma are described. Both lesions showed identical features with a mixed histological picture exhibiting baseloid, tubular and squamous differentiation and a prominent stromal (mesenchymal) component. By light microscopy there appeared to be an intimate relation between the epithelial and mesenchymal components. By electromicroscopy the cellular constituents of these components could be readily distinguished. In the former the cells contained tonofilaments and desmosomes which were associated with basement membrane material. In the latter (mesenchymal component) none of these features was notes, instead the cells appeared similar to fibroblasts and occasional myofibroblasts. Full text available

Adamantinoma of the long bones is a rare, low-grade malignant neoplasm of unknown histogenesis, which affects mainly the tibia of young adults (Keeney et al., 1989). Sozzi et al. (1990) demonstrated a translocation t(7;13)(q32;q14) in a lung metastasis from an adamantinoma of the tibia in a boy who showed the same translocation constitutionally (in normal fibroblasts and lymphoid cells). The identical translocation was found in his normal father. The breakpoint in chromosome 13 was in the same region as that in retinoblastoma (180200). The level of esterase D was normal in the patient and his parents.

Adamantinoma of tibia: a case of late local recurrence along with lung metastases. Filippou DK, Papadopoulos V, Kiparidou E, Demertzis NT Department of Surgery, Kifissia Oncological Hospital Agii Anargiri, Kaliftaki, Kato Kifissia, Athens, Greece. , Greece Journal of Postgraduate Medicine Year : 2003 | Volume : 49 | Issue : 1 | Page : 75-7 Abstract Adamantinomas of long bones are rare primary low-grade malignant tumours composed of cells with epithelial and fibrous characteristics. Local recurrence, though scarce, occurs 5-15 years after the onset of diagnosis. We report a case of local recurrence of an adamantinoma localised in tibia, along with the presence of two lung metastases, 24 years after diagnosis and surgical therapy of the primary tumour. The local recurrence and the lung metastases were removed surgically. The patient remains free of the disease for 3 years. Full text available

Adamantinoma of tibia (a case report). Journal of Postgradualte Medicine Year : 1985 | Volume : 31 | Issue : 1 | Page : 57-8 Surana SS, Mogra NK, Dube MK, Dhruva AK Adamantinoma of tibia is a rarely encountered neoplasm of dispute histogenesis and variable clinical course. Fischer[3] found the microscopic similarity of the tumor with that of adamantinoma of jaw and labelled it as adamantinoma of tibia. The present case is described because of its rare occurrence. (Full text available)

Sandeep Kanakaraddi, T. M. Ravinath, G. Nagaraj: Adamantinoma Of Tibia With Skeletal Metastasis: An Unusual Presentation. The Internet Journal of Orthopedic Surgery. 2006. Volume 3 Number 2. Abstract Adamantinoma is a rare tumour of long bones whose pathogenesis is unknown. It occurs most commonly in the tibia. Although locally aggressive, recurrences are common after resection with metastasis in 10-20% of cases, most commonly to the lungs and rarely to the lymphnodes. In our case a secondary was detected 4 years after resection of primary tumor.

Adamantinoma is a rare tumor that has a predilection for the tibia. This lesion occurs primarily in young people (age 15 to 35 years) and affects males and females almost equally. In the Mayo Clinic Series, the most common locations for adamantinoma were the tibia (about 80% to 85%) and fibula (5% to 10%). Rare sites include the femur, radius and ulna.

Adamantinoma of Long Bone (extragnathic adamantinoma): Adamantinoma is a rare primary low-grade malignant tumor of bone that is locally aggressive. Adamantinoma of the long bone is not related to adamantinoma or ameloblastoma of the mandible and maxilla, which is derived from Rathke's pouch.

- 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;

Adamantinoma of Long Bone (extragnathic adamantinoma): Adamantinoma is a rare primary low-grade malignant tumor of bone that is locally aggressive. Adamantinoma of the long bone is not related to adamantinoma or ameloblastoma of the mandible and maxilla, which is derived from Rathke's pouch. Diagnostic Radiology/Musculoskeletal Imaging/Tumors Basic/Adamantinoma From Wikibooks, the open-content textbooks collection

Sarcoma Volume 1 (1997), Issue 2, Pages 109-111 Disseminating Adamantinoma of the Tibia Albert N. Van Geel, Hans M. Hazelbag, Rob Slingerland, and Margit I. Vermeulen Full text pdf file available