Primary malignant giant cell tumor of the sternum

Primary malignant giant cell tumor (PMGCT) is a diagnosis based on the presence of a high-grade sarcomatous component along with a typical benign giant cell tumor (GCT). We report the first case of PMGCT of the sternum in a 28-year-old male with painless swelling over the manubrium sterni. The differential diagnoses of PMGCT and giant cell-rich osteosarcoma were considered. Surgical resection was performed, and the reconstruction was done with a neosternum using polymethyl methacrylate and prolene mesh. At 30 months follow-up, the patient is disease-free.

Tumor de células gigantes de rodilla: reporte de caso de un tumor benigno potencialmente agresivo / Giant cell tumor of the knee: case report of a potentially aggressive benign tumor

El tumor óseo de células gigantes (TOCG) es un tumor óseo primario que se presenta con más frecuencia en la tercera edad de la vida, ubicándose principalmente a nivel del fémur distal. El desarrollo de una biopsia para su establecer su diagnóstico y poder comenzar con rapidez el tratamiento adecuado, constituye un procedimiento fundamental para poder establecer la resección quirúrgica lo más temprano posible. En este artículo se presenta un caso de TOCG en una paciente de 30 años. Se discute su etiopatogenia, signos y síntomas, diagnóstico, como así también los procedimientos diagnóstico y terapeútico adecuados para lograr una resolución positiva de esta patología.

The giant cell tumor of bone (OCD) is a primary bone tumor that occurs most frequently in the third age of life, being located mainly at the distal femur. The development of a biopsy to establish the diagnosis and to begin appropriate treatment quickly is a fundamental to establish as early as possible surgical resection procedure. In this article a case of TOCG is presented in a patient of 30 years. Its etiology, signs and symptoms, diagnosis, as well as an adequate diagnostic and therapeutic procedures to achieve a positive resolution of this pathology is discussed.

Tumor de células gigantes do esterno / Giant cell tumor of the sternum

Relatamos o caso de uma paciente de 74 anos portadora de tumor de células gigantes em osso esterno. A apresentação clínica e radiológica indicava neoplasia primária de osso esterno. A paciente realizou exames complementares e cirurgia. O exame patológico confirmou o diagnóstico. Comumente observado em ossos longos no esqueleto apendicular, esse tipo de tumor caracteriza-se por sua agressividade local e pelo potencial metastático. Também fazemos uma revisão sobre o tema.

We report the case of a 74-year-old female patient diagnosed with a giant cell tumor of the sternum. The clinical and radiological presentation was indicative of a primary tumor of the sternum. The patient underwent complementary tests and surgery. The pathological examination confirmed the diagnosis. Commonly observed in the long bones of the appendicular skeleton, this type of tumor is characterized by its local aggressiveness and metastatic potential. We also review the literature on the topic.

Clonality analysis of giant cell lesions of the jaws

Despite the importance of clonality to understand the pathogenesis and progression of tumors, it has not been investigated yet in giant cell lesions of the jaws. The aim of this study was to analyze the clonality of peripheral giant cell lesions (PGCL) and central giant cell lesions (CGCL) of the jaws. Six samples of PGCL and 5 samples of CGCL were analyzed in this study using the polymorphic human androgen receptor locus (HUMARA) assay. Three out of the 5 samples of the CGCL and 3 out of 6 samples of PGCL exhibited a monoclonal pattern. Our findings demonstrate that some giant cell lesions of the jaws are clonal, which indicate that these lesions may have a common genetic mechanism of development. Further studies are necessary to better elucidate the molecular mechanisms involved in the pathogenesis of such lesions.

Apesar da importância que a clonalidade das lesões tem para o entendimento da patogênese e progressão dos tumores, ainda não foi feita essa investigação em lesões de células gigantes dos maxilares. O objetivo desse trabalho foi analisar a natureza clonal de lesões periféricas de células gigantes (LPCG) e de lesões centrais de células gigantes (LCCG). Foram analisadas nesse estudo 6 amostras de LPCG e 5 amostras de LCCG, sendo todas elas provenientes de pacientes do sexo feminino. Para essa investigação foi utilizado o método baseado na região polimórfica do exon um do gene humano para oreceptor de andrógeno (HUMARA). Três das 5 amostras de LCCG e 3 das 6 amostras de LPCG exibiram um padrão monoclonal. Nossos resultados demonstram que algumas lesões de células gigantes dos maxilares apresentam uma natureza monoclonal indicando que essas lesões podem ter um mecanismo genético comum de desenvolvimento. Outros estudos são necessários para uma maior compreensão dos mecanismos moleculares envolvidos na patogênese dessas lesões.

Primary malignancy in giant cell tumor: a case report / Malignização primária no tumor de células gigantes: um estudo de caso

Context: Primary malignancy in giant cell tumor (PMGCT) is rare. It is defined as a high-grade sarcoma originating in a giant cell tumor (GCT) and seems to behave less aggressively than its secondary counterpart does. Case report: This report presents the case of a 39-year-old female with pain in her left shoulder for one month. Radiography showed a pathological fracture of the proximal humerus associated with an osteolytic lesion. Histopathological analysis showed typical areas of GCT juxtaposed with a sarcomatous component. Conclusions: PMGCT seems to behave less aggressively than secondary malignancy in GCT, and it may simulate its more common benign counterpart clinically and radiographically. However, it requires a more aggressive type of treatment.

Contexto: Malignização primária no tumor de células gigantes (MPTCG) é rara. Ela é definida como um sarcoma de alto grau originário de um tumor de células gigantes que parece ser menos agressivo que o tipo secundário. Relato de Caso: Relatamos um caso de uma paciente de 39 anos de idade, com dor no ombro esquerdo há um mês. A radiografia mostrou uma fratura patológica do úmero proximal associada a uma lesão osteolítica. O exame histopatológico revelou típicas áreas de tumor de células gigantes justapostas por um componente sarcomatoso. Conclusão: MPTCG parece se comportar menos agressivamente que a neoplasia secundária do tumor de células gigantes (TCG), e pode simular o TCG, que é mais comum, tanto clínica como radiograficamente. Entretanto, exige tratamento mais agressivo.

Multiple brown tumors of the orbital walls: case report / Tumor marrom múltiplo das paredes orbitárias: relato de caso

We report the case of a 40-year-old woman with chronic renal failure and secondary hyperparathyroidism that presented with slowly progressive proptosis of the right eye and mass sensation in the temporal and frontal orbital margins. Computerized tomography scan revealed two separate hyperdense and well-circumscribed lesions in the right orbital walls. A biopsy followed by histopathologic study revealed a dense lesion, with fibrous proliferation associated with osseous metaplasia and osteoclastic activity in the tumor, compatible with the diagnosis of brown tumor. The patient was submitted to surgical removal of the parathyroid glands that resulted in marked improvement in her condition and regression of the orbital tumors. Although the occurrence of more than one separate bone lesion in the orbit usually suggests metastasis, our case shows that brown tumors should also be included in the differential diagnosis of such lesions, particularly in patients with hyperparathyroidism.

Este trabalho relata o caso de uma paciente de 40 anos, com insuficiência renal crônica e hiperparatireoidismo secundário que se apresentou com proptose progressiva e tumoração nas regiões lateral da órbita e superior da órbita. Tomografia computadorizada revelou duas lesões ósseas separadas, bem delimitadas e hiperdensas nas paredes orbitárias. Uma biópsia seguida de estudo histopatológico revelou um tumor denso, com proliferação fibrosa associada a metaplasia óssea e atividade osteoclástica no tumor, características compatíveis com tumor marrom. A paciente foi submetida a remoção das glândulas paratiróides que resultou em melhora dramática do seu estado geral e regressão dos tumores orbitais. Embora a presença de mais de uma lesão óssea separada na órbita geralmente sugira o diagnóstico de lesões metastáticas, nosso caso evidencia que tumores marrons devem também ser incluídos no diagnóstico diferencial, particularmente em pacientes com hiperparatireoidismo.

Estudio radiológico simple en el diagnóstico del tumor de células gigantes en población pediátrica: correlación anatomoradiológica / Plain radiological study in giant cell tumor (GCT) diagnosis among pediatric patients: anatomo-radiological correlation

Background. Giant cell tumor (GCT) is an uncommon primary bone neoplasm in pediatric patients. Plain radiograph of the affected area is the first diagnostic approach in most cases. Objective. To show radiographic features that could allow the radiologist to suspect the diagnosis of GCT through plain radiological study. Methods. Records from the National Bone Tumor File between 1959 and 1999 were retrospectively analyzed. Twenty-nine cases of patients under 20 years with biopsy proven diagnosis of GCT were found. The radiological study was available in 14 cases. Results. From a total of 29 patients, 83 percent were females and 17 percent males. Age ranged from 7 to 19 years, with an average of 16.3 years. In cases with radiological study, 93 percent of them presented epiphyseal involvement of long bones. Osteolytic lesions with metaphyseal extension were the most frequent radiological pattern. One case showed malignancy with metastases. Conclusions. GCT is regarded as an infrequent occurrence in pediatric patients and its diagnosis can be suspected based on plain radiographic findings.

El tumor de células gigantes es un tumor óseo primario, poco frecuente en población pediátrica. En la mayoría de los casos, la radiografía simple es la primera aproximación diagnóstica, por lo que nuestro objetivo es mostrar las características radiológicas que permiten sospechar el diagnóstico del tumor de células gigantes en el estudio radiológico simple. Se analizó retrospectivamente los datos del Registro Nacional de Tumores Óseos, entre los años 1959 y 1999, encontrando 29 casos de tumor de células gigantes histológicamente confirmados en menores de 20 años; en 14 se dispuso de estudio radiológico. El 83 por ciento (24/29) correspondió a mujeres y 17 por ciento (5/29) a hombres, con edades entre 7 y 19 años (promedio = 16.3 años). Radiológicamente, el 93 por ciento mostró compromiso epifisiario de huesos largos y el patrón más frecuente fue osteolítico con extensión metafisiaria.

A correlação entre exames de imagem, características anatomopatológicas e imunoistoquímicas num caso de tumor de céculas gigantes e agressivo do osso, com localização em coluna / Correlation between imaging tests and anatomicopathological and immunohistochemical characteristics in a case of severe giant cell tumor of bone located at spine

O Tumor de Células Gigantes (TGC), é um tumor benigno, com ocorrência de recidiva em cerca de 20-34 por cento dos casos. A localização habitual é na epífise dos ossos longos. O objetivo deste trabalho é relatar um caso de TGC em vértebra, com diagnóstico inicial de Cisto Osseo Aneurismático (COA), e discutir os diagnósticos diferenciais possíveis, correlacionando-os com as características dos exames de imagens. Paciente com 37 anos, do sexo feminino, com quadro clínico de dor na coluna e paraparesia há 2 meses. O diagnóstico inicial foi de COA. Na radiografia a lesão era lítica, com, erosão e destruição da cortical..A tomografia e ressonância evidenciavam lesão cística e hemorrágica, com extensão para partes moles. A revisão das lâminas e análise do espécime cirúrgico ressecado, submetido à coloração HE e imunoistoquímica com marcador para p53, permitiram o diagnóstico de TGC. Muitas lesões, benignas apresentam células gigantes multinucleadas. Os exames de imagem nem sempre permitem um diagnóstico conclusivo. O diagnóstico definitivo de TGC depende do exame anatomopatológico, com avaliação cuidadosa do componente estromal e a imunoexpressão positiva para a proteína p53. O tratamento é a ressecção cirúrgica, com margens amplas seguida por instrumentação nos casos de tumor localizados na coluna vertebral.

Giant Cell Tumor (GCT) is a benign tumor, with a recurrence rate of about 20 percent - 34 percent of the cases. It is usually located at long bone epiphysis. The objective of this study is to report a GCT case in a vertebra, which was early diagnosed as Aneurysmal Bone Cyst (ABC), and to discuss potential differential diagnosis, correlating them to patterns shown on imaging tests. This patient is a 37 year-old female, with clinical picture of pain in spine and paraparesis that started two months earlier. An early diagnosis of ACB was delivered. At X-ray, the injury was lithic, with erosion and cortical destruction. Tomography and resonance showed a cystic and hemorrhagic injury, extending to soft parts. Slides review and the analysis of dried surgical matter submitted to HE and immunohistochemical staining with p53 marker allowed for GCT diagnosis. Many benign lesions present with multi-nucleated giant cells. Imaging tests not always enable a conclusive diagnosis. A definite GCT diagnosis depends on anatomicopathological test, with careful evaluation of the stromal component and positive immunoexpression for p53 protein. Treatment is delivered as surgical resection, with wide margins, followed by instrumentation in cases of tumors located at spine.

An unusual presentation of giant cell tumour (osteoclastoma).

The number of well-documented true giant cell tumours arising in any of the craniofacial bones is small, but they do exist. A 19 year old female, Ms. KS, presented with complain of progressive enlargement of facial bones especially jaw bones, then orbit symmetrically since the age of 7. There was bilateral gross enlargement of mandible, maxilla, orbital walls, causing displacement of eye medially and upwards. The visual acuity of both eyes were 6/36 and 6/18 with best correction. Extra ocular movements were restricted because of bony growth and conjunctiva over inferior fornix were keratinized due to exposure. Fine needle aspiration (FNAC) from the side of bony growth showed plenty of osteoclasts with multinucleated giant cells. The level of serum alkaline phosphatase were highly increased. She underwent orbitotomy and a part of tissue was sent for biopsy which revealed multiples of mononucleargiant cells and tumour cells.

Granuloma de células gigantes central: revisão de literatura / Central giant cell granuloma: review of literature

De acordo com a Organização Mundial de Saúde (OMS) o Granuloma de Células Gigantes Central (GCGC) é uma lesão intra-óssea que consiste de tecido fibroso com focos múltiplos de hemorragia, agregação de células gigantes multinucleadas e, ocasionalmente, trabéculas de tecido ósseo. É uma lesão benigna incomum que soma menos de 7 por cento de todas as lesões benignas dos maxilares em regiões dentadas. Há controvérsias sobre a postura de alguns autores em diferenciar o Granuloma de Células Gigantes Central do Tumor de Células Gigantes. A etiologia ainda é incerta e várias teorias são propostas na literatura. Apresentam uma grande variação na apresentação clínica e na evolução, dependendo do grau de agressividade que demonstram. Acomete principalmente adultos jovens, com predileção pelo sexo feminino (2:1) e cerca de 2/3 dos casos relatados ocorreram na mandíbula, freqüentemente na região anterior. A escolha do tratamento, bem como a taxa de recorrência está na dependência de fatores como a idade do paciente, localização, extensão e comportamento clínico da lesão

Giant cell tumour of talus with osteosarcomatous transformation in a young male: a case report.

A case of osteosarcomatous transformation in a benign giant cell tumour is described. Spontaneous malignant transformation is rare in a giant cell tumour with only 17 cases reported in the literature till date. In addition, a giant cell tumour arising in the bones of the hands and feet is in itself a rarity. In this instance the lesion presented in the talus of the left ankle in a 13 year old male.

Secondary malignant giant cell tumour of bone--a study of five cases with short review of literature.

Secondary malignant giant cell tumour of bone occurs as a result of previous attempts at local control of a benign giant cell tumour of bone (GCT). Out of the total 445 conventional benign GCT of bone, therapeutic irradiation was given in 39 cases as the lesions were located in the vertebrae and pelvic bones where debulking surgery was not possible and the tumours were pressing on the spinal cord. The patients were followed up for 21 years. Out of 39 cases, 5 patients developed sarcomas of which 3 were fibrosarcomas, 1 was malignant fibrous histiocytoma while 1 was an osteosarcoma. All the patients developing post-radiation sarcomas died within a few months due to lung metastasis. In conclusion, all the patients with benign GCT of bone treated with radiation must be followed life long as they are prone to develop sarcomas.

Giant Cell Tumor Originating from the Anterior Arc of the Rib

We report a case of giant cell tumor originating from the anterior arc of the rib. The tumor and the surrounding chest wall were completely resected, and the chest wall defect was covered with Marlex mesh. Giant cell tumor of the bone usually originates from the epiphysis of long bones. Even when the tumor occur in ribs, it usually occur in the posterior aspect. However, giant cell tumor should be included in the differential diagnosis of a tumor originating from the anterior arc of the ribs.

Multifocal giant cell tumour of bone in a skeletally immature patient--a case report.

Giant cell tumor of bone is usually seen in adults affecting a single bone. Multiple giant cell tumour of bone occurring in skeletally immature patients is extremely rare. Multifocal giant cell tumor of bone in a ten year old boy involving upper end of humerus and tibia is being reported for its extreme paucity in literature.

Primary malignant giant cell tumour of sacrum--a case report.

A rare case of primary malignant GCT of sacrum in a 35-year-old female is reported.

Factors regulating the metastatic potential of benign giant cell tumour of bone--study of an unusual case with short review of literature.

Benign giant cell tumour of bone with metastases to other bones and lungs is extremely rare. Benign metastasising giant cell tumour is distinctly separate from multicentric giant cell tumour, primary and secondary malignant giant cell tumour. Factors regulating the local recurrence and metastatic potential of this benign tumour depend on its aggressiveness which can be better assessed by clinical and radiological parameters rather than the histopathological appearance. A benign giant cell tumour of ischium with metastasis to vertebra and lung over an eleven year period is discussed. Extreme paucity of literature prompted to publish the article. A short review of factors determining the recurrence and metastatic spread of benign giant cell tumour of bone is highlighted.

Primary malignant giant cell tumour of bone--a study of two cases with short review.

Primary malignant giant cell tumour of bone is extremely rare. It is distinctly separate from benign metastasising giant cell tumour of bone and secondary malignant giant cell tumour which occurs in response to radiotherapy and repeated curettage of benign giant cell tumor. The tumor has high mortality rate. It usually affects lower end of femur and upper end of tibia. Two usually affects lower end of femur and upper end of tibia. Two cases, on involving upper end of tibia and other in vertebra are discussed. Extreme paucity of literature prompted to publish this article. A short review of radiological appearance, histopathological findings and treatment modalities is highlighted.

Tumor de células gigantes: análise da invasäo articular, fratura patológica, recidiva local e metástase para o pulmäo / Giant cell tumor: analysis of joint invasion, pathologic fracture, local recurrence, and metastasis to the lung

Os autores apresentam avaliaçäo de 39 tumores de células gigantes quanto a patologia, recidiva local, invasao articular e metástases pulmonares, em pacientes atendidos no período de janeiro de 1985 a dezembro de 1995, com seguimento médio de 47 meses. As lesöes foram estadiadas em graus através das radiografias, tomografias e ressonância magnética e segundo a agressividade delas. Baseados no estadiamento pré-operatório, os pacientes foram tratados através de curetagem ou ressecçäo da lesäo com reconstruçäo do defeito criado por meio da utilizaçäo de auto-enxerto, homoenxerto, cimento acrílico ou endoprótese nao convencional. A fratura patológica foi encontrada em 12 (30,7 por cento) pacientes, a recidiva tumoral em 8 (20,5 por cento) pacientes, a invasäo articular em 2 (5,1 por cento) pacientes e a metástase pulmonar em 1 (2,6 por cento) paciente. A presença de fratura patológica nao interferiu no tratamento e näo houve relaçäo entre sua presença e o aumento do número de recidivas locais. Em dois pacientes que apresentavam invasäo articular, a cirurgia consistiu na ressecçäo da articulaçäo comprometida e no paciente com metástase pulmonar, a imunoterapia foi o tratamento de escolha. Os autores discutem os índices de 30,7 por cento de fraturas patológicas, 20,5 por cento de recidivas locais, 5,1 por cento de invasäo articular e 2,6 por cento de metástase pulmonar e compararam os dados encontrados com a literatura.