PubMed · 15444436
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DIOUDONNAT. [Adamantinomas].. https://pubmed.ncbi.nlm.nih.gov/15444436/
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Recent studies have advocated the genotypic and phenotypic delineation of a novel Ewing's sarcoma histologic variant showing epithelial features defined as "adamantinoma-like Ewing's sarcoma". We described an 18-year-old girl with a primary small round-cell sarcoma of the right tibia showing polyphenotypic differentiation with epithelioid features. The neoplastic cells had mainly round or oval nuclei with fine chromatin with a portion of epithelial arrangements. The immunohistochemical analysis showed the epithelial markers of cytokeratin 5/6/18, AE1/AE3, and cytokeratin high molecular weight were stained especially in the foci with epithelioid features, as well as MIC2, S100, and NSE. The diagnosis of the lesion was confirmed as Ewing's sarcoma by the presence of the EWS-FLI1 fusion transcript, and could be defined as the so-called "adamantinoma-like Ewing's sarcoma". After wide excision and high-dose chemotherapy with peripheral blood stem cell transfusion, the patient has been well and continuously event-free for 3 years since the initial diagnosis.
BACKGROUND: Adamantinoma is a rare slow-growing malignant bone tumour. OBJECTIVE: To describe the imaging appearances of six childhood cases and review the published literature. MATERIALS AND METHODS: The database of the Working Group on Paediatric Oncology, Academic Medical Centre/Emma Children's Hospital Amsterdam, was searched for cases of adamantinoma. Additionally a literature study was performed to identify cases of adamantinoma in childhood. RESULTS: We identified six local cases of adamantinoma of the long bones, two boys (age 3 and 8 years) and four girls (mean age 8.8 years, range 3.0-14.0 years). The location of the tumour was the tibia in five and the tibia and fibula in one patient. In two patients initially a different diagnosis was made, which led to a delay in appropriate treatment. None of the children showed pulmonary metastases and all underwent total gross resection. On follow-up (mean 6.1 years, range 1.6-12.0 years) all children remained disease-free. Besides a discussion of our six patients, imaging features, histopathology, surgical approach and a literature review of childhood adamantinomas is presented. CONCLUSION: Although the incidence of adamantinoma is low, it is important to recognize this rare bone tumour, since in the early stages of the disease adequate treatment will result in an excellent prognosis.
BACKGROUND: Adamantinoma is a rare tumor of long bones that can be mistaken for a carcinoma, and numerous other lesions such as myoepithelial tumor, malignant mixed tumor of bone, fibrous dysplasia/osteofibrous dysplasia, and vascular tumors. METHODS: The histology and clinical features of 12 cases accrued from the surgical pathology files over a period of 23 years were analyzed. RESULTS: Of the 12 cases, 4 were men and 8 women, with a mean age of 30 years. Radiologically the tumor presented as a lytic, eccentric, intracortical lesion in the tibia. The histology revealed epithelial islands with basaloid, spindle, and squamous features on a background of fibrotic stroma. Three patients came back with local recurrences, and one of them developed pulmonary metastasis. DISCUSSION: In this article, an attempt is made to study the morphology, biology, and the differential diagnoses of adamantinoma of long bones.