An unusual case of adamantinoma of long bone

Adamantinoma of the long bones is an exceedingly rare and slow-growing tumor that affects the diaphysis of long bones, particularly the tibia. Based on the pattern of the epithelial cell component and the presence or absence of the osteofibrous dysplasia-like element, several histological variants have been described, such as (i) tubular (the most frequent), (ii) basaloid, (iii) squamous, (iv) spindle variant, (v) osteofibrous dysplasia -like variant, and (vi) Ewing's sarcoma - like adamantinoma (the least frequent). The diagnosis may be challenging since this tumor may be mistakenly interpreted as carcinoma, myoepithelial tumor, osteofibrous dysplasia, and vascular tumor. We report the case of a 41-year-old male who presented with swelling over the right leg associated with pain. The X-ray showed a lytic lesion of the right-sided tibia. The diagnosis of adamantinoma was made based on the clinico-radiological, histomorphology, and immunohistochemical findings. Histologically, classic adamantinoma is a biphasic tumor characterized by epithelial and osteofibrous components in varying proportions and differentiating patterns. The diagnosis can be confirmed by immunohistochemistry for demonstrating sparse epithelial cell nests when the radiological features are strongly consistent with adamantinoma. This case is highlighted because the epithelial component can lead to a misdiagnosis, particularly when the clinico-radiological features are overlooked. Adamantinoma of long bones has the potential for local recurrence and may metastasize to the lungs, lymph nodes, or other bones. The prognosis is good if early intervention is taken.

Complications of Allograft Reconstruction following Wide Resection of Malignant Bone Tumors in Long Bones / 대한정형외과학회잡지

PURPOSE: We evaluated the results of allograft reconstruction following wide resection of malignant bone tumors in long bone, retrospectively. MATERIALS AND METHODS: Seven patients were included. The mean age was 44 years old. Male was 4 cases, and female was 3 cases. Mean follow-up period was 38 months. The mean Musculoskeletal Tumor Society (MSTS) score at final follow-up was evaluated. Postoperative complications were evaluated via periodic radiologic follow-up. Oncologic results were analyzed at final follow-up. RESULTS: The primary malignancies occurred at femur in 5 cases, humerus in 1 case and tibia in 1 case. Pathologic diagnoses were osteosarcoma in 4 cases, multiple myeloma in 2 cases and adamantinoma in 1 case. Mean length of allograft was 165 mm. Fixations of allograft were intramedullary nailing with additional plate in 4 cases, intramedullary nailing in 2 cases, and screw fixation in 1 case. Mean time to union was 14.5 weeks. Mean MSTS score at final follow-up was 20 (67%). Postoperative complications were nonunion in 3 cases, implant failure in 1 case, and infection in 1 case. Oncologic outcomes were continuous disease free in 5 cases and alive with disease in 2 cases at final follow-up. Autologous bone graft and hemi-cortical onlay graft were performed in 2 cases of nonunion. CONCLUSION: Allograft reconstruction following wide resection of malignant bone tumors in long bone was effective surgical option. However, the possibility of nonunion between host bone and allograft should be considered.

Adamantinoma de la tibia / Adamantinome du tibia / Adamantinoma of the tibia

El adamantinoma es un tumor maligno de origen epitelial con una frecuencia de aparición escasa. Con este artículo se pretende describir la conducta y evolución de un paciente portador de adamantinoma de la tibia asociado a infección pulmonar y ósea por Mycobacterium avium. Se presenta un paciente masculino de 43 años de edad con el diagnóstico de adamantinoma, localizado en el tercio medio-inferior de la tibia derecha. Los hallazgos clínicos, imaginológicos e histopatológicos permiten llegar al diagnóstico de un adamantinoma del esqueleto apendicular. Se realizó la exéresis de la tumoración y relleno con cemento quirúrgico. Doce meses después del tratamiento aparecieron signos clínicos y radiográficos de recidiva tumoral y fue necesaria la amputación infratuberositaria de la pierna derecha. Además, se observaron lesiones fibroquísticas en el vértice del pulmón izquierdo relacionadas con Mycobacterium avium. Luego de un seguimiento por dos años, se observaron múltiples nódulos metastásicos en ambos campos pulmonares(AU)

Adamantinoma is an epithelial-origin malignant tumor with uncommon frequency of appearance. This article aims to describe the behavior and evolution of a patient with adamantinoma of the tibia associated with pulmonary and bone infection by Mycobacterium avium. We present a 43 years old male patient with the diagnosis of adamantinoma. This adamantinoma was located in the middle-lower third of the right tibia. The clinical, imaging and histopathological findings allowed to reach the diagnosis of adamantinoma of the appendicular skeleton. The excision of the tumor and filling with surgical cement was performed. Twelve months after the treatment, clinical and radiographic signs showed tumor recurrence. The infratuberosal amputation of the right leg was necessary. In addition, fibrocystic lesions were observed at the apex of the left lung related to Mycobacterium avium. After a two-year follow up, multiple metastatic nodules were observed in both lung fields(AU)

L'adamantinome est une tumeur maligne assez rare d'origine épithéliale. Le but de cet article est de décrire le comportement et l'évolution d'un patient atteint d'un adamantinome au niveau du tibia, associé à une infection pulmonaire et osseuse par Mycobacterium avium. Le cas d'un patient âgé de 43 ans, diagnostiqué d'un adamantinome localisé dans le tiers moyen inférieur du tibia droit, est présenté. Les observations cliniques, histopathologiques et d'imagerie ont permis de confirmer le diagnostic d'un adamantinome du squelette appendiculaire. On a effectué l'exérèse de la tumeur et le remplissage à ciment chirurgical. Douze mois après ce traitement, des signes cliniques et radiographiques d'une récidive tumorale ont apparu, et une amputation infratubérositaire de la jambe droite a été nécessaire. On a aussi trouvé des lésions fibrokystiques sur l'apex du poumon gauche, associées à Mycobacterium avium. Après un suivi de deux ans, on a rencontré plusieurs nodules métastatiques dans tous les deux côtés de la région pulmonaire(AU)

Adamantinoma de tibia: manejo quirúrgico con aloinjerto intercalar. A propósito de un caso / Adamantinoma of tibia: Surgical management with intercalary allograft. Report of a case

El adamantinoma es un tumor maligno bifásico que comprende el 0,4% de todos los tumores óseos primarios y el 23% comienza con fracturas patológicas asociadas a traumatismos ocurridos meses o años antes. Presentamos el caso de una paciente de veinte años de edad quien comienza con fractura patológica de la tibia derecha por un trauma de baja energía, con estudios radiológicos e histopatológicos que confirmaron la presencia de un adamantinoma. Fue manejado quirúrgicamente mediante resección amplia del segmento tumoral y reconstrucción con aloinjerto intercalar de tibia fijado con un clavo endomedular de estabilidad multidireccional sustentado con una placa antirrotatoria proximal. Los resultados radiológicos y clínicos fueron satisfactorios.

The adamantinoma is a biphasic malignant tumor comprising 0.4% of all primary bone tumors and 23% debuts with pathological fractures associated with trauma that occurred months or years earlier. We report the case is presented of a twenty-year-old woman that debuted with a pathological fracture of the right tibia with a low-energy trauma. The radiological and histopathological studies confirmed the presence of an adamantinoma. This was treated surgically by wide resection of the tumor and reconstructed with an intercalary allograft bone segment that was fixed with a tibial intramedullary multidirectional antirotatory stability nail sustained further by a proximal plate. The radiological and clinical results were satisfactory.

Adamantinoma en pediatría: Presentación de caso / Pediatric adamantinoma: Case report

El adamantinoma es un tumor primario de los huesos largos, que afecta principalmente la diálisis de la tibia, y es extremadamente raro en pediatría. Se presenta a partir de la segunda década de vida, con un ligero predominio en el sexo masculino. Se trata de un tumor de bajo grado, con alta agresividad a nivel local y bajo índice de metástasis y recurrencia una vez resecado en forma completa. Su diagnóstico resulta difícil, no solo por tratarse de una patología poco frecuente en pediatría, sino también por la dificultad para el diagnóstico diferencial con otras lesiones benignas. Presentamos el caso de un paciente de 15 años, con una tumoración indolora de larga evolución en la tibia distal, cuyo diagnóstico fue confirmado histológicamente con la pieza de amputación, ya que los estudios complementarios y las dos biopsias iniciales no fueron concluyentes. Aunque la mayoría de la bibliografía publicada consta de reportes de casos, y muy pocos en pacientes pediátricos, es de común acuerdo la dificultad para arribar al diagnóstico de adamantinoma.

Adamantinoma is a primary tumor of long bones, which affects mainly the shaft of the tibia, and is extremely rare in pediatrics. It frequently presents during the second decade of life, with a slight predominance in males. It is a low grade tumor, with local aggressiveness and low rate of metastasis and recurrence once it is completely removed. Its diagnosis is difficult, not only because it is a rare disease in children, but also because of the difficulty in the differential diagnosis with other benign lesions. We report the case of a 15-year-old patient with a painless swelling of the distal tibia, whose diagnosis was confirmed with the piece of amputation, as imaging features and both initial biopsies were not enough to achieve diagnosis. Though most of the literature consists of case reports, and very few in pediatric patients, they all agree on the difficulty in achieving the diagnosis of adamantinoma.

Resección tumoral ósea en bloque y sustitución del defecto óseo creado en pacientes del hospital Teodoro Maldonado Carbo: análisis 1993 – 2003 / Osseous tumoral block resection and substitution of osseous defect created in patients at Teodoro Maldonado Carbo hospital: analysis 1993-2003

Tipo de estudio: retrospectivo, descriptivo y observacional. Objetivo general: determinar si la técnica de resección tumoral ósea en bloque es una buena alternativa como parte del tratamiento multidisciplinario para la cirugía de salvataje de miembros. Objetivos específicos: conocer cuáles son los tipos más frecuentes de tumores óseos sometidos a resección tumoral en bloque según clasificación histológica de la OMS. Establecer la relación entre el tipo histológico del tumor según clasificación de la OMS con el sexo de los pacientes. Determinar de acuerdo a la clasificación de Enneking en que estadiaje es más frecuente la resección en bloque. Precisar la viabilidad y funcionalidad de los miembros post-resección en bloque, con o sin el uso de agentes sustitutos del defecto óseo. Resultados: de los 37 pacientes sometidos a resección en bloque, 23 eran hombres y 14 mujeres. Según la clasificación de la OMS, los tumores malignos predominaron en la muestra con 20 casos (54), el TCG u osteoclastoma fue el tipo de tumor más encontrado con un 43,2 (16 casos). Según la estadificación de Enneking los tumores en estadío IIB fueron los de mayor incidencia con 7 casos (18,9), las endoprótesis no convencionales predominaron con 15 casos. Para el análisis de los 28 pacientes que pasaron a la segunda fase de estudio, fue necesaria la evaluación de los pacientes por medio de la escala de funcionalidad para miembros inferiores de la clínica Mayo, encontrándose los siguientes resultados: de los 28 pacientes 2 (7), presentaban dolor moderado; 13(46), podían caminar más de 10 cuadras; 20, no necesitaban ningún medio de apoyo para movilizarse; 21 (75), podían movilizarse en un vehículo fácilmente; 24(86), no presentaban ninguna dificultad en el cuidado de sus pies, ninguno presentaba claudicación en la marcha, 15 pacientes podían subir escaleras normalmente. De la suma de 80 puntos, la media de los resultados en esta investigación fue de 72,5 puntos y se reportaron.

Study type: retrospective, descriptive, and observational. General objective: to determine if the technique of osseous tumoral block resection is a good alternative as part of the multidisciplinary treatment for the member salvation surgery. Specific objectives: to get to know which are the most frequent types of osseous tumours subjected to osseous tumoral block resection according to the histological classification of the WHO. To establish the relationship between the histological types of tumour according to the WHO’s classification against the patient’s sex. To determine, according to Enneking stratification, in which stage the block resection is most frequent. To define the viability and functionality of the members after the block resection with or without the use of substitutes for the osseous defect. Results: out of the 37 patients subjected to block resection, 23 were men and 14 women. According to WHO’s classification, malignant tumours were predominant in the sample with 20 cases (54), “GCM” or osteoclastoma was the most found type of tumour with a 43.2 (16 cases). According to Enneking stratification the tumours in stage IIB were majority with 7 cases (18.9), unconventional endoprosthesis predominated with 15 cases. For the analysis of the 28 patients who passed to the second phase of the study, the patients’ evaluation at the Mayo Clinic was necessary through functionality scale for the lower limbs, the following results were found: out of the 28 patients 2 (7) presented moderate pain; 13 (46), were able to walk more than 10 blocks; 20, didn’t need any support to move; 21 (75), were able to move easily in a vehicle ; 24 (86), didn’t present any difficulty in the care of their feet, none presented claudication, 15 patients were able to climb stairs normally. In the addition of 80 points, the ratio of the results in this research.

Adamantinoma de ossos longos / Adamantinoma of the long bones

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 were submitted to surgical treatment, 17 with "tibialization" of the fibula and the others with amputation. RESULTS: The post-surgical evolution showed to be unpredictable and not related to clinical or histopathological aspects. Two patients had an evolution with lung metastasis and died. Six did not present recurrent disease or metastases, and are clinically cured. The others, after being released from hospital, did not return for consultation. CONCLUSIONS: This is a rare neoplasia made of epithelial and mesenchymal structures that must be accurately diagnosed before any procedure is attempted. Treatment is surgical, with tumor resection with a good oncologic margin. The biological behavior is varied and unpredictable.

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

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.

Adamantinoma de la tibia con componente de quiste óseo aneurismático secundario / Adamantinoma of the tibia component of secondary aneurysmal bone cyst

El adamantinoma de los huesos largos es uno de los tumores malignos menos frecuentes, representando únicamente el 1%. Su localización en la tibia es la más común, y el tratamiento indicado es la resección en bloque y salvamento de extremidad. En algunos reportes de la literatura se describe con poca frecuencia un componente óseo aneurismático. En este artículo reportamos el caso de un paciente masculino quien presentó un adamantinoma de la tibia proximal con un gran componente de quiste óseo aneurismático que enmascaró su patología de base, y retrasó el diagnóstico y su tratamiento. Presentamos el tratamiento realizado, su evolución y las complicaciones presentadas.

Differentiated adamantinoma: a case report and review of literature.

Adamantinoma of the long bones are uncommon, slow growing tumors with a predilection for the tibia. A special rare variant known as the osteofibrous dysplasia-like adamantinoma or differentiated adamantinoma is thought to be a precursor of the classic adamantinoma. We report a case in a 24 year old female who presented with pain and lytic lesion of the left tibia. Microscopy showed a tumor composed predominantly of osteofibrous dysplasia-like areas with focal cytokeratin positive epithelial islands. This case is highlighted because of its rarity, its association with good prognosis and possible misdiagnosis as osteofibrous dysplasia.

Treatment of Osteofibrous Dysplasia and Associated Lesions

PURPOSE: To report long term treatment outcomes of osteofibrous dysplasia and association with adamantinoma. PATIENTS AND METHODS: From January 1984 to July 2001, 14 patients with osteofibrous dysplasia were followed for an average of 108 months (78 to 260 months). Our patient group consisted of 6 men and 8 women, with a mean age of 13.9 years (2 to 65 years). We reviewed the clinical and pathological features of all 14 patients. RESULTS: Thirteen patients had a lesion in the tibia, while one patient had lesions in both the tibia and the fibula. Initial treatments were observation after biopsy (6 patients), curettage with or without a bone graft (3 patients), resection followed by a free vascularized fibular bone graft (4 patients), or resection and regeneration with the Ilizarov external fixation (1 patient). Curettage was performed on 6 patients due to recurrence or progression after the initial treatment. Among these patients, one was diagnosed with AD from the biopsy of the recurrent lesion. This patient was further treated by segmental resection and pasteurization. After the initial pathology slides of the 13 patients were reviewed with immunohistochemical cytokeratin staining, one patient diagnosis was changed from osteofibrous dysplasia to osteofibrous dysplasia-like adamantinoma. CONCLUSION: Some patients with osteofibrous dysplasia require close observation because of the high association risk between osteofibrous dysplasia and adamantinoma, Immunohistochemical staining may be helpful in differentiating these two diagnoses.

Osteofibrous Dysplasia-Like Adamantinoma: A Case Report with its Immunohistochemical and Ultrastructural Studies

Osteofibrous dysplasia (OFD)-like adamantinoma is a rare skeletal tumor that is characterized by the predominant OFD-like pattern with scattered epithelial nests. Adamantinoma shares clinical features (the majority of lesions in the tibia and the prevalent age group), radiologic findings (radiolucency with sclerotic shadow), and pathologic similarities (particularly the presence of scattered cytokeratin-positive stromal cells) with OFD. We describe a case of OFD-like adamantinoma. Epithelial cell nests express the epithelial membrane antigen, pancytokeratin, CK14, and collagen type IV. Ultrastructurally, the oval to spindle cells in the epithelial foci had abundant tonofilaments, and well-formed desmosomes with dense plaques, of which well preserved desmosomes are demonstrated for the first time in OFD-like adamantinoma. These immunohistochemical and ultrastructural findings further support that the origin of epithelial cells of classic and OFD-like adamantinoma are epithelial cells transformed from fibroblastic cells in the proliferating osteofibrous tissue.

Ossifying fibroma / 대한정형외과학회잡지

Ossifying fibroma is not a well recognized entity, and only rarely affects the long bones. The main differential diagnosis is with fibrous dysplasia and with adamantinoma, non-ossifying fibroma of the long bone. Ossifying fibroma seldom has even a moderate tendency to progress during childhood, but it recurs frequently after curettage or subperiosteal resection. Any progression of the lesion comes to an end after puberty. So, surgery should be delayed as long as possible. But, if the lesion is rapidly progressive, or if a patient has repeated fractures, it would be necessary to resort to wide extraperiosteal resection. We have experienced 7 cases of ossifying fibroma from Octover 1979 to November 1988. The results were as follows; 1. The male and female ratio, was 5 to 2 and six patients were at their first decade, and one patient was 13 years old. 2. The lesion site was six cases in the tibia, one case in the fibula. 3. In six patients, we had done wide resection with free-vasculaized fibular graft in five cases and wide resection of the distal one-third of the fibula in one case. Two patients among them had recurrence. One case of recurrence was performed incomplete wide resection with free vascularized fibular graft because the lesion was too close to the distal epiphysis of the tibia.