Sample records for adamantinoma


    Meiske Margaretha


    Full Text Available Adamantinoma tulang panjang merupakan neoplasma tulang yang jarang dengan histogenesis yangmasih menjadi perdebatan dan gejala klinis yang bervariasi. Insiden tumor ini 0,56% dari seluruhkeganasan tulang primer. Kasus ini dibahas karena insidensinya yang sangat jarang. Pasien adalahseorang wanita, usia 16 tahun dengan keluhan timbul benjolan yang terasa nyeri dan membesarperlahan pada tungkai bawah kiri bagian atas selama 2 bulan terakhir. Radiologis memperlihatkanzona berbatas tegas, eksentrik, ekspansil dan lusen pada bagian atas tibia kiri. Setelah pembedahan,spesimen dikirim ke Laboratorium Patologi Anatomi. Tumor berbatas tegas, lobulated, putih abuabu,kenyal, berukuran 7x4,5x2 cm. Mikroskopis, tumor terdiri dari sel-sel epitel tersusun dalamstrukturcorddan pulau dikelilingi oleh stroma fibrouspadat dengan palisadingpada tepinya. Diagnosisadamantinomaditegakkan berdasarkan gambaran klinis, radiologis, dan histopatologi yang khas.

  2. Adamantinoma de ossos longos Adamantinoma of the long bones

    José Donato de Próspero


    Full Text Available OBJETIVOS: Estudar retrospectivamente 18 casos de pacientes com adamantinoma de ossos longos, todos localizados na tíbia; ressaltar a importância da biópsia e a correlação com métodos de imagem para diagnóstico diferencial com osteofibrodisplasia e displasia fibrosa; tecer considerações sobre a natureza do adamantinoma de ossos longos, cujo nome deve-se à analogia histológica com o adamantinoma (ameloblastoma da mandíbula. MÉTODOS: Foram analisados o quadro clínico, imagens e exames anatomopatológicos complementados com imunohistoquímica e a evolução dos pacientes. Todos foram submetidos a tratamento cirúrgico, 17 com "tibialização" da fíbula e os demais com amputação. RESULTADOS: A evolução pós-cirúrgica mostrou-se imprevisível e não relacionada com os aspectos clínicos ou histopatológicos. Dois pacientes evoluíram com metástases pulmonares e morreram. Seis não tiveram recidivas ou metástases e estão clinicamente curados. Os demais, após alta hospitalar não retornaram à consulta. CONCLUSÕES: Trata-se de rara neoplasia constituída por estruturas epiteliais e mesenquimais que devem ser diagnosticadas com precisão, antes de qualquer procedimento. O tratamento é cirúrgico com ressecção do tumor com boa margem oncológica. O comportamento biológico é variável e imprevisível.OBJECTIVE: To make a retrospective study of 18 cases of patients with adamantinoma of the long bone, all of them located in the tibia; to point to the relevance of biopsy and the correlation with imaging methods in order to have a differential diagnosis with osteofibrous dysplasia and fibrous dysplasia; to comment on the nature of long bone adamantinoma, whose name is due to the histological analogy with the adamantinoma (ameloblastoma of the jaw. METHODS: A review was made of the clinical condition, images, and anatomopathological exams supplemented with immunohistochemical essays, and the evolution of the patients. All of them




    Full Text Available Adamantinoma is a ,rare pr i mar y malignant tumor of • the long bones wit h unknown pathogenesis . , So far only a few cases are r eported in t he l ite r ature (2 ,3 ,9 , 11,17 • .Tibia is .t he major s ite o f predi lection , - howevex , the tumor a l so reported occasional ly i n other , l ong bones such as femur , f ibula , humerus , ulna and radius.( l;8 ,12 . . Most of the patients are i n the s e cond and ,t hir d decades of l ife. Rare l y fibr ous dysplasia (5 is a s s oc i a t ed with , adamantinoma of t he long bones . The symptoms a re l ong standing. The rout i ne r adiol ogic finding i s t hat of multiple lucent zones interspers ed with scleroti c bone,us ually wi th one l arge r arefied ' a r ea i n t he mid shaf t . Hist ologi ca lly, islands of epi t helial cells with peripheral l i s adi ng are seen in fibr ous s troma . Histogenesis of this tumor is not yet clear. But majority of the studies believe that of epithelial origin (6,15,16,18.Treatment depends upon the extent of tumor. If it is surgically feasible, resection of the tumor is the treatment of choice, if not, amputation. Here we report one case of adamantinoma of tibia and discuss clinicopathological findings.

  4. Multiple recurrences and late metastasis of adamantinoma in the tibia: a case report.

    Giannoulis, D K; Gantsos, A; Giotis, D; Paschos, N K; Vagionas, A; Arnaoutoglou, C M; Pentheroudakis, G; Xenakis, T A


    Adamantinoma is a rare, low-grade, malignant bone tumour. We report on a 46-year-old woman who had early multiple recurrences of adamantinoma of the right tibia and late metastasis to the lung and ribs 13 years after the first surgical treatment. She underwent multiple complete tumour excisions and eventually below-knee amputation and removal of the left lung and sixth to eighth ribs.

  5. Adamantinoma of the pelvic bone, a difficult diagnosis with fatal outcome

    Binesh F; Abrisham J; Sobhan M; Navabii H; Vahidfar MR; Ashrafi K


    Adamantinoma is a primary low grade malignant bone tumor that is predominantly located in the mid-portion of the tibia. The tumor is of interest for two reasons: first, there still exists considerable dispute as to the origin of the lesion and recent reports reveal that the condition is more malignant than had previously been supposed. Although cases of adamanti-noma located to the axial skeleton have been reported, this is the first case of adamantinoma located to pelvic bone in Iran. Here we present the clinical, radiological & histopathological features of a 19 year-old male with painful lesion located to the right pelvic bone which was morphologically and immunohistochemically diagnosed as adamantinoma. In general, metastasis is seen in 15%–20% of patients. The spread can occur to regional nodes, lung and infrequently to skeleton, liver and brain [1]. Several weeks after surgery, our patient's condition gradually worsened. A CT-scan of abdomen revealed widespread liver metastasis and the patient died due to acute liver failure. This case demonstrates that the mortality rate from adamantinoma is not always low.

  6. 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.

  7. Clonality Assessment in a Case of Multifocal Adamantinoma and a Review of the Literature

    Paul Borbas


    Full Text Available Adamantinoma is a low-grade, malignant biphasic bone tumour predominantly located in the tibia. In up to 50% of all cases this is combined with one or more lesions in the ipsilateral fibula. Whether these lesions represent regional metastases or arise de novo is not yet exactly known. In order to address this question, we extracted DNA from the respective fresh frozen tumour tissues in a case of a young woman with a multifocal adamantinoma of both the tibia and ipsilateral fibula. Afterwards the X inactivation pattern was studied by means of methylation-sensitive polymerase chain reaction and primers that target the polymorphic CGG trinucleotide repeat of FMR1 gene and the polymorphic CAG repeat, on exon 1 of the human androgen receptor gene (AR. The analysis of the AR was homozygous and not informative. Studying the FMR1 gene, we detected a 100% skewing of the X inactivation pattern of both locations and found that the same allele was methylated. Even if the fibula lesion arose de novo there would have been a 50 : 50 chance that the same allele was methylated. As this methylation pattern was found we cannot provide a valid explanation for the origin of the fibula lesion. Analysis of X inactivation patterns in future cases of polyfocal adamantinoma might provide further evidence for one of the two theories.

  8. 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

  9. Adamantinoma-like Ewing family tumor of soft tissue associated with the vagus nerve: a case report and review of the literature.

    Kikuchi, Yoshinao; Kishimoto, Takashi; Ota, Satoshi; Kambe, Michiyo; Yonemori, Yoko; Chazono, Hideaki; Yamasaki, Kazuki; Ochiai, Hidemasa; Hiroshima, Kenzo; Tanaka, Mio; Tanaka, Yukichi; Horie, Hiroshi; Nakatani, Yukio


    Adamantinoma-like Ewing family tumor (EFT) is a rare subset of EFTs showing mixed features of Ewing sarcoma and adamantinoma of the long bones. All currently reported cases of the adamantinoma-like type have been associated with bone. Recently, a unique type of EFT was reported showing complex epithelial differentiation associated with the vagus nerve. Here we describe another unique type of EFT arising in the soft tissue of the neck associated with the vagus nerve. An 11-year-old girl presented to our hospital with a neck tumor on her right side. Surgical resection was performed, and histopathologic examination demonstrated a high-grade malignant neoplasm. The tumor was composed of sheets of small round proliferating cells, basaloid tumor nests with marked squamous differentiation, biphasic growth pattern with epithelioid tumor nests, and spindle cell proliferation. Immunohistochemically, the tumor cells showed diffuse expression of CD99 and FLI-1. In addition, small round cells and basaloid/squamoid components were immunoreactive for AE1/AE3, CAM5.2, cytokeratin 5/6, high-molecular weight keratin, p63, and p40 (ΔNp63). Reverse transcription polymerase chain reaction and direct sequencing analysis revealed that the tumor harbored a t(11;22) translocation, involving EWSR1 and FLI-1, which are characteristic of EFTs. According to these findings, our case has characteristics of both a subset of adamantinoma-like EFT and EFT with complex epithelial differentiation. We suggest that EFT with complex epithelial differentiation is in a common spectrum with the adamantinoma-like type and that adamantinoma-like EFTs can arise in soft tissue, leading to difficulty in differential diagnosis with malignant epithelial tumors.

  10. Ameloblastoma o adamantinoma Ameloblastoma or adamantinoma

    M. C. Verdi


    Full Text Available Se relata el caso de un paciente de 26 años de edad, con deformidad facial, que presenta un tumor gingivoyugal posterior derecho con imágenes oteolíticas en los estudios radiológicos simples y en la resonancia magnética, con histopatología e inmunohistoquímica de ameloblastoma. La terapéutica fue quirúrgica con amplio margen. Motiva la presentación la escasa frecuencia de presentación de este tumor.It is present a ameloblastoma case, tumour of rare case on a patient of 26 years old in who development the process on the maxilla. It is realized a revision of the neoplastic characteristic and it is comment the result of the surgical treatment realized at the patient.

  11. Disseminating Adamantinoma of the Tibia

    Albert N. Van Geel


    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.

  12. Intraosseous glomus tumor of the fibula

    Bahk, W.J. [Catholic Univ. of Korea, Gyunggido (Korea). Dept. of Orthopaedic Surgery; Mirra, J.M. [Orthopaedic Oncology, Orthopaedic Hospital, Los Angeles, CA (United States); Anders, K.H. [Dept. of Pathology, Kaiser Permanente, Woodland Hills, CA (United States)


    Glomus tumor is a rare, benign vascular tumor and intraosseous glomus tumor, which arises primarily within bone, is even rarer. Fewer than 20 cases have been reported in the literature. We present the case of a 34-year-old woman with glomus tumor primarily in the midshaft of the fibula that radiologically mimicked chondromyxoid fibroma, aneurysmal bone cyst or adamantinoma, together with a review of other reported cases. (orig.)

  13. Recently described neoplasms of the sinonasal tract.

    Bishop, Justin A


    Surgical pathology of the sinonasal region (i.e., nasal cavity and the paranasal sinuses) is notoriously difficult, due in part to the remarkable diversity of neoplasms that may be encountered in this area. In addition, a number of neoplasms have been only recently described in the sinonasal tract, further compounding the difficulty for pathologists who are not yet familiar with them. This manuscript will review the clinicopathologic features of some of the recently described sinonasal tumor types: NUT midline carcinoma, HPV-related carcinoma with adenoid cystic-like features, SMARCB1 (INI-1) deficient sinonasal carcinoma, biphenotypic sinonasal sarcoma, and adamantinoma-like Ewing family tumor.

  14. Desmoplastic ameloblastoma

    Nah, Kyung Soo; Cho, Bong Soo [Dept. of Dental Radiology, College of Dentistry, Pusan National University, Pusan (Korea, Republic of)


    The ameloblastoma is a true neoplasm of enamel organ-type tissue which does not undergo differentiation to the point of enamel formation, and the term 'adamantinoma' which implies the formation of hard tissue was replaced by 'ameloblastoma' since these tumor is not associated with the formation of hard tissue (1) Recently experienced a case of ameloblastoma which could not be explained by any of the known bone destruction patter ns of ameloblastoma. Rather the lesions looked like a fibro-osseous lesion. The result of biopsy was quite storage not only to the radiologists but also to the surgeons who operated the lesion. The suspicion could only be explained by finding a report (2) by Japanese who experienced a similar case.

  15. Congenital osteofibrous dysplasia Campanacci: spontaneous postbioptic regression.

    Jobke, Björn; Bohndorf, Klaus; Vieth, Volker; Werner, Mathias


    Osteofibrous dysplasia Campanacci is a rare benign bone tumor most frequently observed in young childhood. The exclusive localization in the tibia is very characteristic. The incidence of congenital primary bone tumors is an absolute rarity. We report a case of a newborn with a histologically proven osteofibrous dysplasia Campanacci at the tibia presenting a regular radiographic follow-up. After a small open biopsy and spontaneous minor fracture, the lesion rapidly remodeled within 1½ months and almost completely regressed with restutio ad integrum. Surgical intervention in this tumor entity at childhood age has been shown to have a high recurrence rate but due to lack of experience with newborns, guidelines do not exist. We analyze the radiologic and histologic differential diagnosis of juvenile adamantinoma and emphasize that congenital peripheral bone tumors should be treated conservatively when malignancy is excluded.

  16. Intercalary Reconstructions with Vascularised Fibula and Allograft after Tumour Resection in the Lower Limb

    Katharina Rabitsch


    Full Text Available Reconstruction with massive bone allograft and autologous vascularised fibula combines the structural strength of the allograft and the advantages of fibula’s intrinsic blood supply. We retrospectively analysed the outcome of twelve patients (4 male, 8 female who received reconstruction with massive bone allograft and autologous vascularised fibula after tumour resection in lower limb. Mean age was 17.8 years (range 11–31 years, with following primaries: Ewing’s sarcoma (n=6, osteosarcoma (n=4, liposarcoma grade 2 (n=1, and adamantinoma (n=1. Mean followup was 38.7 months (median 25.7 months; range 2–88 months. Seven tumours were located in the femur and five in the tibia. The mean length of bone defect was 18.7 cm (range 15–25 cm. None of the grafts had to be removed, but there occurred four fractures, four nonunions, and two infections. Two patients developed donor side complication, in form of flexion deformity of the big toe. The event-free survival rate was 51% at two-year followup and 39% at three- and five-year followup. As the complications were manageable, and full weight bearing was achieved in all cases, we consider the combination of massive bone allograft and autologous vascularised fibula a stable and durable reconstruction method of the diaphysis of the lower limbs.

  17. Ameloblastic fibromas and related tumors in cattle.

    Gardner, D G


    This article concerns rare odontogenic tumors that occur predominantly in the mandibular incisor region of young cattle and which have often in the past been referred to as ameloblastomas, or as the outdated synonym, adamantinoma. Twenty-two examples from the literature and two new ones were studied. Six consisted of epithelial islands which resembled those of ameloblastoma but which were located within a cellular fibrous connective tissue that was the second component of the tumor; these mixed odontogenic tumors therefore represented ameloblastic fibromas, not ameloblastomas. Eight consisted of a combination of ameloblastic fibroma and odontoma and therefore were ameloblastic fibro-odontomas, and one was apparently malignant (ameloblastic fibro-odontosarcoma). Excluding this last lesion, these tumors should respond well to enucleation, like their human counterparts but, to confirm this hypothesis, the margins of future examples should be carefully examined to determine that they are well-demarcated, not invasive. The microscopic features of the remaining 9 tumours could not be evaluated adequately, while another 17 tumors in cattle and water buffalo reported briefly could not be studied to any extent because of insufficient information.

  18. Activating GNAS mutations in parosteal osteosarcoma.

    Carter, Jodi M; Inwards, Carrie Y; Jin, Long; Evers, Barbara; Wenger, Doris E; Oliveira, Andre M; Fritchie, Karen J


    Parosteal osteosarcoma is a surface-based osteosarcoma that often exhibits deceptively bland cytologic features, hindering diagnosis in small biopsies or when correlative radiologic imaging is not readily available. A number of benign and malignant fibro-osseous lesions, including fibrous dysplasia (FD) and low-grade central osteosarcoma, fall within the morphologic differential diagnosis of parosteal osteosarcoma. Somatic mutations in GNAS, encoding the α-subunit of the heterotrimeric G protein complex (Gsα), occur in FD and McCune-Albright syndrome but have not been reported in parosteal osteosarcoma. We evaluated GNAS mutational status in parosteal osteosarcoma and several of its histologic mimics to determine its utility in differentiating these entities. Eleven of 14 (79%) FD cases had GNAS mutations within codon 201 (5 R201C and 6 R201H mutations). GNAS mutations were not detected in any cases of adamantinoma or osteofibrous dysplasia. Direct sequencing of 9 parosteal osteosarcomas, including 3 of low grade and 6 with dedifferentiation, revealed activating GNAS mutations in 5 cases (55%), distributed as 4 R201C-mutated tumors and 1 tumor with an R201H mutation. GNAS codon 227 mutations were not detected in any of the cases. There was no association between GNAS mutational status and patient demographics, histologic dedifferentiation, or clinical outcome. To our knowledge, we report the first series of parosteal osteosarcomas harboring activating GNAS mutations. Our data suggest that GNAS mutational status may have limited utility as an ancillary technique in differentiating benign and malignant fibro-osseous lesions of the bone.

  19. 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

  20. 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