Sample records for adamantinoma

  1. Disseminating Adamantinoma of the Tibia

    Margit I. Vermeulen


    Full Text Available Patient. This report describes a patient with a primary long bone adamantinoma. The lesion was initially wrongly diagnosed as fibrous dysplasia and the patient was treated by curettage. At second local recurrence, the tumour had progressed from an osteofibrous dysplasia-like to a full-blown classic adamantinoma, with metastatic potential to the lungs 19 years after the initial treatment. Lung metastasectomy by sternotomy was carried out twice in a period of over 312 years. The patient is currently alive without evidence of other metastatic disease.

  2. Adamantinoma: A clinicopathological review and update

    Ranjan Prabhat


    Full Text Available Abstract Adamantinoma is a primary low-grade, malignant bone tumor that is predominantly located in the mid-portion of the tibia. The etiology of the tumor is still a matter of debate. The initial symptoms of adamantinoma are often indolent and nonspecific and depend on location and extent of the disease. Histologically, classic adamantinoma is a biphasic tumor characterized by epithelial and osteofibrous components that may be intermingled with each other in various proportions and differentiating patterns. To assure the histological diagnosis, pathologists should employ immunohistochemistry for demonstrating the sometimes sparse epithelial cell nests when the radiological features are suggestive of adamantinoma. There is paucity of compiled data over adamantinoma in the literature, hence authors tried to make a comprehensive review which must be of use to beginners and trained pathologists. Our objective is to further define the clinicoradiologic features and pathologic spectra of adamantinoma.

  3. Case report 506: Adamantinoma of the soft tissues of leg

    Bertoni, F.; Bacchini, P.; Zucchi, V.; Mapelli, S.


    A 12-year-old boy with a longstanding soft tissue lesion of the leg in the pretibial and perifibular areas is presented. A synovial biphasic or monophasic sarcoma was considered but this lesion has a well-organized pattern and a more malignant cellular pattern than is demonstrated in adamantinoma of the soft tissues or in bone. The adamantinoma is a malignant tumor, as demonstrated by the prompt recurrence after intralesional excision in this case. (orig./GDG).

  4. Adamantinoma in tibia. A case report and review

    Adamantinoma of the long bones is an uncommon tumor usually located in the tibial diaphysis. We present the case of a 47-year-old man who presented with a pathological tibial fracture which was studied by plain radiography, computed tomography and ultrasound followed by needle biopsy. We review the literature concerning tumors of this type, their most relevant features and the differential diagnosis. (Author) 13 refs

  5. Adamantinoma in childhood: report of six cases and review of the literature

    Adamantinoma is a rare slow-growing malignant bone tumour. To describe the imaging appearances of six childhood cases and review the published literature. 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. 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. 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. (orig.)

  6. Caso raro de pneumotórax: adamantinoma metastático A rare case of pneumothorax: metastatic adamantinoma

    Roberto Gonçalves


    Full Text Available Descrevem-se dois casos de metástases pulmonares de adamantinoma de ossos longos, o qual é uma neoplasia óssea de baixo grau que raramente metastatiza. Nos dois casos a apresentação clínica das metástases se deu por pneumotórax espontâneo secundário a escavação tumoral, fenômeno descrito em apenas três dos trabalhos consultados na literatura. São descritos os achados clínicos, radiológicos e anatomopatológicos, bem como os procedimentos adotados nos dois casos.Here, we describe two cases of lung metastasis of adamantinoma of long bones, a low-grade bone neoplasm that rarely metastasizes. In both cases, the clinical presentation of the metastases was characterized by spontaneous pneumothorax secondary to tumor cavitation, a phenomenon described in only three of the studies reviewed in the literature. Clinical, radiological, and anatomopathological findings, as well as the procedures adopted in the two cases, are described.

  7. The Complimentary Role of Methoxy-Isobutyl-Isonitrile and Hand-Held Gamma Probe in Adamantinoma.

    Maharaj, Masha; Korowlay, Nisaar; Ellmann, Prof


    Adamantinoma is a rare locally aggressive osteolytic tumor that is found 90% of the time in the diaphysis of the tibia with the remaining lesions found in the fibula and long tubular bones. A case of adamantinoma of the tibia is presented. The added value of nuclear medicine investigations in the workup of this patient is described. A three-phase whole body (99m)Tc-methylene diphosphonate bone and a whole body (99m)Tc-methoxy-isobutyl-isonitrile scans were complimentary in the demarcation of viable bone tumor and the assessment of the remainder of the bone and soft tissue to exclude other sites. Intra-operative assistance with a hand-held gamma probe, guided the biopsy of the most metabolically active tumor tissue. Histology revealed a biphasic tumor composed of epithelial and fibrous components, in keeping with an adamantinoma. PMID:26912979

  8. Osteofibrous dysplasia, osteofibrous dysplasia-like adamantinoma and adamantinoma: correlation of radiological imaging features with surgical histology and assessment of the use of radiology in contributing to needle biopsy diagnosis

    Khanna, Monica; Saifuddin, Asif [Royal National Orthopaedic Hospital NHS Trust, Department of Clinical Radiology, Stanmore, Middlesex (United Kingdom); Delaney, David; Tirabosco, Roberto [Royal National Orthopaedic Hospital NHS Trust, Department of Histopathology, Stanmore, Middlesex (United Kingdom)


    The aim of this study was to correlate the imaging features with surgical histology for tibial osteofibrous dysplasia (OFD), osteofibrous dysplasia-like adamantinoma (OFD/LA) and classical adamantinoma and to determine the additional role of imaging in suggesting a correct diagnosis in cases of needle biopsy misdiagnosis. This is a retrospective audit of 24 patients presenting over a 9-year period to a specialist orthopaedic oncology unit. Radiographic and axial magnetic resonance imaging (MRI) characteristics were recorded for each patient. The needle biopsy diagnosis and resection specimen histological diagnoses were retrospectively reviewed and compared with the imaging findings. The 24 cases comprised five OFD, 11 OFD/LA and eight adamantinoma based on surgical resection histology. The mean length of OFD was 6.1 cm (range 2-8.5 cm), for OFD/LA was 6.5 cm (range 2-13 cm) and for adamantinoma was 13.2 cm (range 6.5-26 cm). Seven of eight adamantinomas had moth-eaten margins compared to five of 11 OFD/LA and two of five OFDs. Three of eight adamantinomas demonstrated cortical destruction, with seven of eight cases completely involving the marrow cavity. In comparison, only one of 11 OFD/LA cases and one of five OFD cases demonstrated cortical destruction, and complete marrow involvement was rare. Four of 19 cases had a different needle biopsy result compared to the final histology, three cases being upgraded from an OFD/LA or OFD to classical adamantinoma. The radiological features of these three cases were more in keeping with a diagnosis of adamantinoma. A diagnosis of classical adamantinoma is suggested by an extensive lesion with moth-eaten margins and complete involvement of the medullary cavity on axial MR imaging. Misdiagnosis on needle biopsy may occur in up to one fifth of cases, and radiological features can assist in making the correct diagnosis. (orig.)

  9. Cytological diagnosis of adamantinoma of long bone in a 78-year-old man

    Mimi Gangopadhyay


    Full Text Available A clinicohistopathological study of a rare case of adamantinoma of long bone in a 78-year-old patient is presented. The cytological features when evaluated in conjunction with clinical and radiologic features are sufficiently diagnostic. The primary knowledge of its existence and knowledge of its cytological features are important for a correct preoperative cytological diagnosis.

  10. Prognosis of Adamantinoma of Long Bones: A Long-Term Follow-Up Study

    Khodamorad Jamshidi


    Full Text Available Background Adamantinoma of long tubular bones is a rare primary malignant bone tumor. According to the literature, different prognosis and recurrence rates have been reported. Objectives The purpose of this case series study was to evaluate the clinical behavior and prognostic features of adamantinoma of long bones. Patients and Methods In this study, 13 histologically proven cases of adamantinoma of long bones which were treated in our hospital during March 1977 to June 2015 were evaluated. The mean follow-up period was 72 ± 44.4 months. The male:female ratio was 10:3, aged between 13 and 63 years at the time of diagnosis (mean = 24.8 ± 13.1 years. There was a period between the onset of disease and definite diagnosis (mean: 17.5 ± 14.7 months. In two cases according to the imaging and clinical symptoms the lesion seemed to be osteofibrous dysplasia, but during the follow-up, the diagnosis was changed to adamantinoma when open biopsy and pathologic assessment were performed. In 10 cases the tumor was located in the tibial diaphysis, in two cases the location was the distal shaft of the femur, and in one case the location was proximal of the humerus. All the cases underwent wide resection and intercalary or osteoarticular allograft reconstruction as the main surgery after primary incisional biopsy. Results In eight cases, at least one local recurrence happened in an average 33.4-month period after the wide resection. In four of these patients rather than one local recurrence was occurred, but in one patient despite pulmonary metastasis the patient underwent pulmonary lobectomy and was alive at the end of the study. Allograft-related complications happened in five cases (two infections, one osteoarthritis of ankle, and two allograft fractures. For five patients ultimately recurrence and complication lead to amputation. Six patients died because of this disease, in all of which pulmonary metastasis occurred. The five-year survival rate in this

  11. Adamantinoma-like Ewing family tumors of the head and neck: a pitfall in the differential diagnosis of basaloid and myoepithelial carcinomas.

    Bishop, Justin A; Alaggio, Rita; Zhang, Lei; Seethala, Raja R; Antonescu, Cristina R


    Ewing sarcoma family tumors (EFTs) of the head and neck are rare and may be difficult to diagnose, as they display significant histologic overlap with other more common undifferentiated small blue round cell malignancies. Occasionally, EFTs may exhibit overt epithelial differentiation in the form of diffuse cytokeratin immunoexpression or squamous pearls, resembling the so-called adamantinoma-like EFTs and being challenging to distinguish from bona fide carcinomas. Furthermore, the presence of EWSR1 gene rearrangement correlated with strong keratin expression may suggest a myoepithelial carcinoma. Herein, we analyze a series of 7 adamantinoma-like EFTs of the head and neck, most of them being initially misdiagnosed as carcinomas because of their anatomic location and strong cytokeratin immunoexpression, and subsequently reclassified as EFT by molecular techniques. The tumors arose in the sinonasal tract (n=2), parotid gland (n=2), thyroid gland (n=2), and orbit (n=1), in patients ranging in age from 7 to 56 years (mean, 31 y). Microscopically, they departed from the typical EFT morphology by growing as nests with peripheral nuclear palisading and prominent interlobular fibrosis, imparting a distinctly basaloid appearance. Moreover, 2 cases exhibited overt keratinization in the form of squamous pearls, and 1 sinonasal tumor demonstrated areas of intraepithelial growth. All cases were positive for CD99, pancytokeratin, and p40. A subset of cases showed synaptophysin, S100 protein, and/or p16 reactivity, further confounding the diagnosis. Fluorescence in situ hybridization assays showed EWSR1 and FLI1 rearrangements in all cases. Our results reinforce that a subset of head and neck EFTs may show strong cytokeratin expression or focal keratinization, and are therefore histologically indistinguishable from more common true epithelial neoplasms. Thus, CD99 should be included in the immunopanel of a round cell malignancy regardless of strong cytokeratin expression or

  12. Report on a case with adamantinoma of long bone%罕见长骨造釉细胞瘤1例报告

    徐辉铭; 林维和; 陈泽智; 王斌


    目的 总结分析临床罕见的骨肿瘤的临床表现及治疗特点.方法 经X线初诊,病理切片检查并根据具体的临床情况采用相应的方法治疗.结果 经病理确诊为低度恶性造釉细胞瘤,采用骨瘤段切下经煮沸40min后原位植入,人工骨材料填充、钢板内固定,抗炎治疗, 14d拆线,切口愈合而出院,随防4年,患肢骨愈合良好,未见肿瘤复发.结论 采用瘤段切除煮沸后原位植入是治疗骨造釉细胞瘤的一种较好的方法.

  13. Radiological diagnostics of skeletal tumors

    The book contains contributions concerning the following topics: 1. introduction and fundamentals: WHO classification of bone tumors, imaging diagnostics and their function; localization, typical clinical and radiological criteria, TNM classification and status classification, invasive tumor diagnostics; 2. specific tumor diagnostics: chondrogenic bone tumors, osseous tumors, connective tissue bony tumors, osteoclastoma, osteomyelogenic bone tumors, vascular bone tumors, neurogenic bone tumors, chordoma; adamantinoma of the long tubular bone; tumor-like lesions, bony metastases, bone granulomas, differential diagnostics: tumor-like lesions

  14. Radiological diagnostics of skeletal tumors; Radiologische Diagnostik von Knochentumoren

    Uhl, M. [Universitaetsklinikum Freiburg (Germany). Kinderradiologie; Herget, G.W. [Universitaetsklinikum Freiburg (Germany). Dept. Orthopaedie und Traumatologie


    The book contains contributions concerning the following topics: 1. introduction and fundamentals: WHO classification of bone tumors, imaging diagnostics and their function; localization, typical clinical and radiological criteria, TNM classification and status classification, invasive tumor diagnostics; 2. specific tumor diagnostics: chondrogenic bone tumors, osseous tumors, connective tissue bony tumors, osteoclastoma, osteomyelogenic bone tumors, vascular bone tumors, neurogenic bone tumors, chordoma; adamantinoma of the long tubular bone; tumor-like lesions, bony metastases, bone granulomas, differential diagnostics: tumor-like lesions.

  15. Diagnosis and treatment of sarcomas and related tumors


    The Cancergram focuses on clinical aspects of sarcomas involving soft tissue and bone, and also includes abstracts on related malignant and benign tumors. Soft tissues are considered as all non-epithelial extra-skeletal tissues of the body, with the exception of the reticuloendothelial system, the neuroglia, and visceral and parenchymal organs. Included, therefore, are sarcomas of the vascular system, fatty tissue, muscle tissues, connective tissues, and synovial tissues. Bone tumors included are osteosarcoma, chondrosarcoma, adamantinoma, chrondroblastoma, Ewing's sarcoma, and other benign and malignant disorders of the bone. This Cancergram excludes disorders of the bone marrow, which are the subjects of separate Cancergrams (see series CT03 for leukemias, and series CT12 for multiple myeloma). The scope includes diagnosis and staging, supportive care, evaluation, and therapy. Selected abstracts concerning epidemiology, etiology and other pre-clinical studies will also be included where they have direct clinical relevance.

  16. Topography of the inferior alveolar nerve in relation to cystic processes of the mandible in dental MRI

    Purpose: Cystic processes are changing the course of the inferior alveolar nerve in the mandible. This study evaluates the possibility of demonstrating the relationship between space-occupying processes and the course of the neurovascular bundle. Materials and Methods: Thirteen patients with cystic processes in the mandible (9 keratocystic lesions, 1 eosinophilic granuloma, 1 plasmocytoma, 2 adamantinomas) were examined by MRI (1.5-T magnet, 8-cm surface coil, PD-gradient-echo-sequences in sagittal and coronal orientation, without enhancement) and the results retrospectively evaluated. Results: The entire course of the nerve could be delineated in all patients. In six patients with minor cystic processes, the nerve was identified in both sagittal and coronal orientation. In seven patients with major cystic lesions, only parts of the nerve were detected in either image orientation, but the nerve could be visualized in its entire length by evaluating coronal and sagittal images side by side. Conclusion: It is possible to delineate the inferior alveolar nerve in its entirety along pathologic mandibular lesions. For large cystic lesions, this requires the evaluation of both coronal and sagittal sections of multidirectional MRI. (orig.)

  17. Clinicopathological features of five unusual cases of intraosseous myoepithelial carcinomas, mimicking conventional primary bone tumours, including EWSR1 rearrangement in one case.

    Rekhi, Bharat; Joshi, Sujit; Panchwagh, Yogesh; Gulia, Ashish; Borges, Anita; Bajpai, Jyoti; Jambehekar, Nirmala A; Pant, Vinita; Mandholkar, Mahesh; Byregowda, Suman; Puri, Ajay


    Primary intraosseous myoepithelial tumours, including carcinomas are rare tumours. The concept of histopathological spectrum of these tumours is evolving. We describe clinicopathological and immunohistochemical features of five myoepithelial carcinomas, including molecular cytogenetic results in one case. There were five male patients within age-range of 8-40 years (median = 26). Four tumours occurred in the long bones, including two tumours, each, in the femur and fibula, respectively, while a single tumour occurred in the proximal phalanges. Tumour size (n = 3 cases) varied from 5.6 to 8.6 cm. On radiological imaging, most tumours appeared as expansile, lytic and destructive lesions. Two tumours appeared as sclerotic lesions. Two cases were referred with diagnoses of chondrosarcomas and a single case was referred with two different diagnoses, including an adamantinoma and an osteosarcoma. Histopathological examination in all these cases showed multinodular tumours comprising mostly polygonal cells, exhibiting moderate nuclear atypia and interspersed mitotic figures within a stroma containing variable amount of myxoid, chondroid, hyalinised and osteoid-like material. Three tumours revealed prominent squamous differentiation. By immunohistochemistry, tumour cells were positive for EMA (5/5), pan CK (AE1/AE3) (3/3), CK5/6 (4/4), CK MNF116 (1/1), S100 protein (5/5) and GFAP (3/5). The first tumour revealed EWSR1 rearrangement. The first patient, 10 months after tumour resection and a simultaneous lung metastatectomy, is free-of-disease (FOD). The second patient, 11 months after tumour resection is FOD. The third and fourth patients underwent wide resections and are on follow-up. The fifth patient underwent resections, including a lung metastatectomy. Primary intraosseous myoepithelial carcinomas are rare and mimic conventional primary bone tumours. Some primary intraosseous myoepithelial carcinomas display EWSR1 rearrangement. Squamous differentiation may be

  18. Tumores congénitos do sistema nervoso II - Craniofaringiomas

    Horácio M. Canelas


    Full Text Available Os craniofaringiomas, embora raros entre os tumores intracranianos, representam o tipo mais freqüente de neoplasia congênita do sistema nervoso. Os autores referem a constituição e a ulterior destruição parcial do ducto hipofisário, de cujos restos êsses tumores derivam. Expõem as classificações dos craniofaringiomas sob os pontos de vista macroscópico (císticos, semicísticos e sólidos, histopatológico (cistos epiteliais mucosos, adamantinomas e epiteliomas planocelulares e topográfico (selares, supra-diafragmáticos e intracrânio-selares. A propósito da incidência, ressalta a maior freqüência nas duas primeiras décadas, embora os craniofaringiomas não sejam exclusivos dos jovens. Predominam ligeiramente no sexo masculino. A sintomatologia é estudada de acôrdo com a situação supradiafragmá-tica (manifestações predominantemente hipotálamo-quiasmáticas ou selar (distúrbios das funções gônado e somatotrófica da hipófise. As alterações radiológicas são de grande valor para o diagnóstico, particularmente as que se revelam no craniograma (calcificações e erosões selares, pneumencefalografia, pneumo e iodoventriculografia, e arteriografia cerebral. No tocante ao tratamento dos craniofaringiomas, os autores referem as controvérsias existentes sôbre os resultados da radioterapia. O tratamento cirúrgico comporta técnicas variadas, desde a punção transesfenoidal (hoje quase abandonada, até a craniotomia frontal ou frontotemporal. O problema da excisão radical é discutido, salientando-se as dificuldades na sua consecução, dadas as aderências do tumor com as vias ópticas na região quiasmática, as artérias regionais e o hipotálamo. A elevada mortalidade operatória é atribuida principalmente à manipulação do hipotálamo e ao colapso hipofisário. É estudado com minúcias o emprêgo do ACTH ou cor-tisona associado à cirurgia. São de prever bons resultados do tratamento com r