Treatment of Patients Diagnosed with Giant Cell Tumor of Bone: Experience of a Philanthropic Hospital in the State of Piauí, Brazil / Tratamento de pacientes com diagnóstico de tumor ósseo de células gigantes: Experiência de um hospital filantrópico no Piauí, Brasil

Abstract Objective To evaluate the treatment of patients with giant cell tumors of bone treated from 2009 to 2019 in a philanthropic hospital, as well as to try and determine the regional clinical and epidemiological profile, aiming to enrich the Brazilian data set and compare our findings with those of the literature. Methods An analytical, observational, and cross-sectional study with retrospective data collection and a quantitative approach, analyzing medical records of patients with giant cell tumors treated at a philanthropic hospital from 2009 to 2019. Results We evaluated 49 medical records; 55.1% of the patients were women, 53.1% were aged between 20 and 40 years, 69.4% of the cases were Campanacci grade III, and 30.6% affected the proximal end of the tibia. The rate of pathological fractures secondary to the tumor and pulmonary metastasis was low. More than 69% of the patients underwent intralesional surgery. Recurrence occurred in 16.3% of the cases. Conclusion The criteria used for the diagnosis, classification, and treatment at our service followed the standards established by the literature, and they can guide further research and improve local prognosis in the future.

Resumo Objetivo Avaliar o tratamento fornecido a pacientes com diagnóstico de tumor de células gigantes ósseo atendidos no período de 2009 a 2019 em um hospital filantrópico, bem como determinar o perfil clínico e epidemiológico regional, visando enriquecer os dados nacionais e comparar os achados com a literatura existente. Métodos Estudo analítico, observacional e transversal, com coleta retrospectiva e abordagem quantitativa, com análise de prontuários de pacientes diagnosticados com tumor de células gigantes atendidos em um hospital filantrópico no período de 2009 a 2019. Resultados Foram avaliados 49 prontuários, sendo que 55,1% eram de mulheres, com 53,1% dos casos na faixa etária de 20 a 40 anos, 69,4% de casos de grau III de Campanacci, e 30,6% acometendo a extremidade proximal da tíbia. Observou-se baixo índice de fratura patológica secundária ao tumor e de metástase pulmonar. A cirurgia intralesional foi realizada em 69,5% dos pacientes. Houve recidiva em 16,3% dos casos. Conclusão Os critérios usados para diagnóstico, classificação e tratamento em nosso serviço seguiram os padrões estabelecidos pela literatura, e podem orientar novas pesquisas e melhorar o prognóstico local futuramente.

Cirugías ablativas por causas oncológicas. 17 años de experiencia en una Unidad Especializada del Estado Monagas, Venezuela / Ablative surgeries for oncological causes. 17 years experience in a Monagas specialized unit

Estudio retrospectivo, sobre pacientes a quienes se les practicó cirugías ablativas por causas oncológicas: un total de 76 casos, predominando en la serie: el sexo masculino, la segunda década de la vida y como motivo de consulta: aumento de volumen con un 73,5%, el osteosarcoma fue la neoplasia más frecuente con un 34%, la fractura patológica acompañó a los tumores en el 50% de los casos, el hueso más afectado fue el fémur, los estadios según Enneking predominantes fueron los II b y III. Se practicaron más cirugías en miembros inferiores, siendo la amputación transfemoral la más frecuente. La intención de los procedimientos fue predominantemente curativa con un 69% sobre las intenciones paliativas, se practicaron más amputaciones en niveles oncológicos radicales que amplias. Se obtuvo una sobrevida global de 53,4% a los dos años de seguimiento, el miembro fantasma estuvo presente como complicación por encima del 77% de los casos operados(AU)

Retrospective study on patients who underwent ablative surgeries for oncological causes: a total of 76 cases, predominantly in the series: male sex, second decade of life and as a reason for consultation: volume increase with 73,5%. Osteosarcoma was the most frequent neoplasm with 34%, pathological fracture accompanied the tumors in 50% of cases, the most affected bone was the femur, the predominant Enneking stages were IIb and III. More surgeries were performed on the lower limbs, with transfemoral amputation being the most frequent. The intention of the procedures was predominantly curative with 69% of the palliative intentions, more amputations were performed at radical oncological levels than extensive ones. An overall survival of 53.4% was obtained at two years of follow-up, the phantom limb was present as a complication in over 77% of operated cases.

Tumor de células gigantes en rótula manejado con patelectomía / Giant cell tumor in patella managed with patellectomy

Se presenta un caso de gonalgia por causa tumoral en rótula en una paciente femenina. Por el incremento de sintomatología y limitación funcional en corto tiempo, se había programado para biopsia y aplicación de injerto óseo y/o polimetilmetacrilato, pero en el acto quirúrgico fue necesario realizar patelectomía total por el masivo compromiso rotuliano ocasionado por un tumor de células gigantes (TCG). Nivel de Evidencia: V

We present a case of knee pain due to a tumor in the patella in a female patient. To which, due to the increase in symptoms and functional limitation in a short time, a biopsy and application of a bone graft or polymethylmethacrylate had been scheduled, but it was necessary to perform patellectomy due to the massive patellar involvement caused by the GCT. Level of Evidence: V

Tumores de células gigantes en el antebrazo / Tumors of giant cells in the forearm

Se presentan los casos clínicos de 2 pacientes atendidos en la consulta de Ortopedia del Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba. Uno había sido operado de un tumor de células gigantes en el tercio proximal del radio izquierdo hacía 3 años, con evolución satisfactoria hasta que comenzó a presentar aumento de volumen y dolor en el tercio distal de dicho radio, que limitaba la flexoextensión de la muñeca; el otro había sufrido un traumatismo en el tercio distal del antebrazo izquierdo y presentaba aumento de volumen entre los límites de la cara dorsal y ventral del antebrazo y la muñeca. Se realizó la exéresis de los tumores, con resección en bloque y fijación externa con injerto de hueso del banco destinado a ello en esta provincia. Ambos egresaron sin complicaciones y se indicó seguimiento por ortopedia y oncología.

The case reports of 2 patients assisted in the out-patient Orthopedics Deparment of Dr Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba are presented. One had been surgically treated 3 years ago due to a giant cells tumor in the proximal third of the left radius, with a satisfactory clinical course until it began to present volume increase and pain in the distal third of his radio that limited the wrist's flexoextension; the other patient had suffered a traumatism in the distal third of the left forearm and it presented increase of volume between the egdes of the dorsal and ventral sides of the forearm and the wrist. The exeresis to the tumors, with resection in block and external fixation with bank bone implants dedicated to this in the province were carried out. Both patients were discharged without complications and Orthopedics and Oncology follow-up were indicated.

Tumor de células gigantes espinal en niños: Reporte de dos casos / Spinal giant cell tumor in children. Report of two cases

El tumor de células gigantes espinal es una neoplasia de estirpe benigna y comportamiento local agresivo, de presentación rara en la población pediátrica. El dolor asociado al compromiso neurológico es la presentación típica en niños. La resección amplia del tumor y la descompresión de los elementos neurales asociada a fusión instrumentada permiten obtener una columna estable, preservar o restaurar la función neural y evitar la recidiva tumoral. Se presentan dos pacientes con tumor de células gigantes espinal en esqueleto inmaduro, sometidos a resección y estabilización mediante artrodesis instrumentada, sin recidiva en el posoperatorio alejado. La presentación clínica, la edad de los pacientes y los resultados quirúrgicos a largo plazo hacen de extremo valor el reporte de estos casos en esqueletos inmaduros. Nivel de Evidencia: III

Spinal giant-cell tumor is a benign neoplasm with locally aggressive behavior, and rare in the pediatric population. Pain associated with neurological manifestations is the typical presentation in children. Tumor wide resection and decompression of neural elements together with instrumented spinal fusion allow to obtain a stable column, preserve or restore neural function and avoid tumor recurrence. We present two patients with spinal giant-cell tumor in the immature skeleton who underwent resection and stabilization by instrumented spinal fusion, without recurrence in the long-term postoperative period. Reporting of these cases is extremely valuable due to the clinical presentation, age of patients, and long-term postoperative results in the immature skeleton. Level of Evidence: III

Fibroma gingival de células gigantes de tamaño inusual / Gingival giant cell fibroma of unusual size

Giant cell fibroma is a benign oral fibrous tumor and it is typically an asymptomatic sessile or pedunculated mass that is usually less than 1 cm in diameter. The lesion consists of uninflamed fibrous tissue in which there are numerous large uninucleated or multinucleated spindle- and stellate-shaped cells with prominent basophilic cytoplasm. The purpose of this paper is to report a case of a gingival giant cell fibroma of abnormal size. A 31-year-old white woman was referred to the dental service for evaluation of a growth on the mandibular gingival. The intraoral examination revealed a 3.0 × 1.5 cm exophytic gingival mass located in the lingual gingiva of the right mandibular permanent first and second molars. The differential diagnosis included peripheral ossifying fibroma, peripheral giant cell granuloma, and giant cell fibroma. Complete surgical excision of the lesion was performed and the diagnosis of giant cell fibroma was made. No complications or recurrence of the lesion have been noted after 4 years of follow-up. Although giant cell fibromas are benign lesions in which simple surgical excision is curative, it is very important that dental and medical professionals recognize it in light of the frequency of occurrence and the need for a precise diagnosis(AU)

El fibroma de células gigantes es un tumor fibroso benigno de la mucosa bucal que típicamente se presenta como una masa asintomática sésil o pediculada generalmente menos de 1 cm de diámetro. La lesión consiste en tejido fibroso no inflamado en el que se encuentran numerosas células fusiformes y estrelladas de gran tamaño, mononucleares o multinucleadas con prominente citoplasma basófilo. El propósito de este trabajo es describir el caso de un fibroma gingival de células gigantes de tamaño inusual. Una mujer blanca de 31 años de edad se presentó al servicio dental para la evaluación de un crecimiento en la encía mandibular. El examen clínico intrabucal reveló una masa gingival exofítica de 3,0 cm x 1,5 cm situado en la encía lingual en el área de los primeros y segundos molares permanentes mandibulares del lado derecho. El diagnóstico diferencial incluyó fibroma osificante periférico, granuloma periférico de células gigantes y fibroma de células gigantes. Se realizó la escisión quirúrgica completa de la lesión y el diagnóstico definitivo fue de fibroma de células gigantes. No se han observado complicaciones o recurrencia de la lesión después de 4 años de seguimiento. Aunque los fibromas de células gigantes son lesiones benignas en las que la escisión quirúrgica simple es curativa, es muy importante que los profesionales dentales y médicos reconozcan la necesidad de un diagnóstico preciso en vista de la frecuencia de aparición(AU)

Giant-cell tumor: analysis on the importance of early diagnosis and the epidemiological profile / Tumor de células gigantes: análise sobre importância do diagnóstico precoce e perfil epidemiológico

This study aimed to ascertain the relationship between early diagnosis of giant-cell tumors (GCT) and their prognosis, by correlating the time of symptom onset with the staging of the injury (through the Campanacci classification at the time of diagnosis), and with the type of treatment. The secondary objective of the study was to outline the epidemiological profile of patients with GCT in the region where the data were gathered, and to compare them with data in the literature. METHODS: The authors present an evaluation on 61 patients diagnosed with bone GCT, with regard to the site of involvement, age, initial symptoms, time of symptom onset, classification and type of treatment, among patients attended between May 1994 and August 2009. RESULTS: The threshold indicated as the limit for Campanacci stage I tumors to be the commonest diagnosis, with a 98.2% chance that the treatment would be non-aggressive, was 2 months after symptom onset. This finding was statistically significant (p = 0.017). Every additional month increased the chance that a patient would be diagnosed with an advanced-stage tumor by 10.94%, in relation to the chances of having the other two stages of the tumor. CONCLUSION: The study result not only suggests that the alternative hypothesis that the earlier the diagnosis of GCT is, the less severe the lesion will be, has been confirmed; but also especially predicts the relationship between the time of symptom appearance and the severity of the tumor.

Presumir a relação entre o diagnóstico precoce do tumor de células gigantes (TCG) e o seu prognóstico, relacionar o tempo de surgimento dos sintomas com o estadiamento da lesão, por meio da classificação de Campanacci no momento do diagnóstico, e com tipo de tratamento. O objetivo secundário do estudo é traçar o perfil epidemiológico dos pacientes com TCG da região onde foram colhidos os dados e compará-lo com dados da literatura. MÉTODOS: Avaliação de 61 pacientes diagnosticados com tumor de células gigantes ósseo quanto ao local de acometimento, idade, sintomatologia inicial, tempo do surgimento dos sintomas, classificação e tipo de tratamento em pacientes atendidos entre maio de 1994 e agosto de 2009. RESULTADO: Aponta o marco de dois meses após o início da sintomatologia como data limite, quando seria mais comum o diagnóstico de tumor estágio I de Campanacci e com 98,2% de chance de ser tratado de modo não agressivo, dados com relevância estatística (p = 0,017). A cada aumento de um mês a chance de um paciente ser diagnosticado com tumor em estágio avançado é 10,94% maior do que em relação aos outros dois estágios do tumor. CONCLUSÃO: O resultado do estudo sugere não somente a confirmação da hipótese opcional de que quanto mais precoce o diagnóstico de TCG, menos grave é a lesão, mas, principalmente, prediz a relação do tempo de surgimento do sintoma com a gravidade do tumor.

A Tenosynovial Giant Cell Tumor Arising from Femoral Attachment of the Anterior Cruciate Ligament

The localized type of tenosynovial giant cell tumor usually occurs on the palmar side of fingers and toes. Tenosynovial giant cell tumors of the tendon sheath are rarely intra-articular. We report a giant cell tumor of the tendon sheath arising from femoral attachment of the anterior cruciate ligament and its treatment with arthroscopy in a 28-year-old man.

Tumor marrom bilateral do hiperparatiroidismo primário em mandíbula: relato de caso / Bilateral brown tumor of primary hyperparathyroidism in the mandible: case report

OBJETIVOS: Apresentar um caso de envolvimento mandibular bilateral por tumor marrom do hiperparatireoidismo primário e discutir a diferenciação do tumor marrom do hiperparatireoidismo com a lesão central de células gigantes. DISCUSSÃO E CONCLUSÃO: A diferenciação é estabelecida pela confirmação do distúrbio endócrino, fator determinante no tratamento do tumor. O tratamento foi a resolução da endocrinopatia pela exérese de adenoma nas glândulas paratireoide.

OBJECTIVES: To presente a cased of bilateral mandibular involvement by a brown tumor of hyperparathyreoidism,in which the treatment consisted in resolution of the endocrinopathy by neoplasm exeresis from the parathyroid glands. DISCUSSION AND CONCLUSION: The correct differentiation of the hyperparathyroidism brown tumor from the central giant cell lesion is an essential factor to avoid diagnostic errors and unnecessary treatments.This differentiation is established by the confirmation of endocrinal disturbance, a determinant factor in the treatment of the tumor.

Fibroma de células gigantes: una presentación inusual en un niño de 2 años de edad / Giant cell fibroma: an unusual presentation in a child of 2 years old

El objetivo de este artículo es relatar un caso clínico de fibroma de células gigantes en un niño de 2 años de edad. Asimismo, presentar una revisión de literatura sobre esta entidad y discutir sobre posibles factores involucrados a su etiología

The aim of this paper is to report a clinical case of giant cell fibroma in a 2-years-old child. Also present a review of literature from this entity and discuss possible factors involved in its etiology

O objetivo deste artigo é relatar um caso clínico de fibroma de células gigantes em uma criança de 2 anos de idade. Além disso, apresentar uma revisão de literatura sobre esta entidade e discutir sobre possíveis fatores associados a sua etiologia

Fibromiomatosis uterina gigante: reporte de un caso / Giant uterine fibromyomatosis: case report

Comunicar el caso clínico de una paciente joven, con diagnóstico de fibromiomatosis uterina gigante, quien fue resuelta quirúrgicamente en forma satisfactoria. Se describe el caso, la técnica quirúrgica empleada y los hallazgos transoperatorios observados. Hospital Universitario “Dr. Luis Razetti”. Barcelona, Estado Anzoátegui. Se realizó histerectomía abdominal total, salpingectomía bilateral y ooforectomía derecha. El peso de la pieza quirúrgica fue de 13 kg. Durante el acto operatorio, se diagnosticó una lesión incidental de la vejiga, la cual fue reparada exitosamente. La evolución posoperatoria fue satisfactoria y la paciente manifestó un alto grado de satisfacción con el resultado quirúrgico. La fibromiomatosis uterina gigante es una entidad patológica benigna de muy escasa incidencia. Su resolución representa un reto para el equipo quirúrgico debido al gran volumen de la pieza quirúrgica y a las variaciones en la distribución de los órganos intraabdominales, originadas por el recimiento uterino.

Including MIR of a primary bone leiomyosarcoma that radiologically mimics a giant cell tumor.

The authors present a case of a 42-year-old female who developed a leiomyosarcoma of the right proximal tibia that appeared radiologically similar to a giant cell tumor Histology revealed spindle cells running in whorl-like fashion with focal atypia and low mitotic figures. The immuno-stains revealed positive reactivity for alpha-smooth muscle (SMA), muscle actin and cytokeratin (AE1/AE3). The authors rendered a diagnosis of low-grade leiomyosarcoma of bone. The lesion was considered a primary lesion since the patient did not have other leiomyomatous tumors. The MRI showed hypo- to iso- signal intensity on T1-weighted imaging and heterogeneous intensity on T2-weighted imaging. This was likely due to admixed fibrotic tissue in the lesion. The tumor cells were not positive for Ebstein-Barr virus by in-situ hybridization as seen in leiomyomatous tumors in immunodeficiency patients.

Giant Cell Tumor of Soft Tissue: a Case with Atypical US and MRI Findings

We report the case of a giant cell tumor with diffuse interstitial hemorrhaging and unusually prominent cystic components in the soft tissue of the thigh which has not been reported previously. Magnetic resonance image (MRI), showed signal intensity typical of a giant cell tumor. However, because of its conspicuous large well-circumscribed cystic components, the differential diagnoses, based on the image findings from an ultrasonography (US) and MRI, were complicated epidermoid cyst, cystic change of a neurogenic tumor, and a parasitic cyst.

Tumor central de células gigantes: reporte de un caso y revisión de la literatura / Central giant cell tumor: a case report and literature review

Lesiones de células gigantes son un grupo de diversas patologías con etiología desconocida, poco entendidas y con dificultad de diagnostico. Son procesos no neoplásicos de crecimiento lento, circunscrito, normalmente asintomático. Las lesiones de celulas gigantes se manifiestan generalmente en huesos largos, siendo raro su aparición en los maxilares. A seguir describiremos un caso de tumor de celulas gigantes en el hueso maxilar removido a través de procedimiento quirúrgico. Paciente del género femenino, 33 años, raza negra, se presento al servicio de Cirugía y Traumatología Bucomaxilofacial del Hospital Regional "José de Simone Neto", en junio de 2003, reclamando de inchazo cerca de la nariz. Nel plan de tratamiento se opto por la biopsia incisional, obteniendo de esta forma el diagnostico y así poder realizar posteriormente el procedimiento quirúrgico correspondiente. Los resultados de la biopsia con los aspectos radiográficos en las diferentes etapas de la lesión, concluimos que se trataba de un caso de Tumor Central de Células Gigantes. Es prudente la realización de una biopsia incisional para el diagnóstico histopatológico definitivo de determinadas lesiones para la ejecución de un plan de tratamiento correcto y seguro

Giant cells lesions are a group of diverse pathologies with unknown etiology, few knowledge and difficulty diagnostic. They are non-neoplasics processes with slow growth, circumscribed, normally asymptomatic. The giant cells lesions occur generally in long bones, being rare his appearance in maxillaries. We will describe a case of giant cells tumor in the maxilla removed through surgical procedure. Female patient, 33 years, black race, appeared in the service of Bucomaxilofacial Surgery and Traumatology of the Hospital Regional "Jose de Simone Neto", in June of 2003, complaining of a growth near the nose. The treatment plan was incisional biopsy, obtaining from this, the diagnosis to make the corresponding surgical procedure later. Result of the biopsy with the radiographic aspects in different stages of the lesion, concluded that was a case of Central Tumor of Giant Cells. It is prudent the realization of an incisional biopsy to have the histopathologic diagnosis to determine the correct and safe treatment plan for those lesions

[Osteomalacia and giant cell tumor: a rare case]

Oncogenic octeomalacia is an unusual and rare clinicopathologic syndrome characterized by mesenchymal tumors that apparently produce osteomalacia and biochemical abnormalities consisting of hypophosphatemia and normocalcemia. We have investigated the mechanism by which a giant cell tumor of bone caused biopsy-proved osteomalacia in a 50-year-old woman. A 50-year-old woman presented with generalized bone and pelvicrural pain, associated with fatiguability and muscle weakness. The diagnosis of osteomalacia was retained, associated with a giant cell tumor. The coexistence of giant cell tumor of bone and osteomalacia suggested the diagnosis of oncogenic osteomalacia. Resolution of the biochemical abnormalities of the syndrome after tumor resection, established this diagnosis. oncogenic osteomalacia can be a form of vitamin-D-refractory osteomalacia due to altered vitamin D3 metabolism

Management of Giant Cell Tumour : a Nigerian Experience

Giant cell tumours (GCT) are the commonest bone tumours worldwide. It is rarely malignant but when it does it progresses to fibrosarcoma with high mortality. Otherwise it causes poor cosmesis; disability and pathological fractures. A total of 19 cases of histologically established Giant cell tumour of the bone were reviewed prospectively in a 5 year study. 14 cases were benign; 4 malignant and one was a malignant transformation. Lesions around the knee accounted for 42.2of the cases; but the radius was the commonest single bone affected with 26.3. Eleven patients had curettage; five of them had autogenous bone grafting while the remaining six had bone grafting and plate augmentation. One patient had fore-quarter amputation while seven had tumour resection. There was no recurrence recorded among those that had currretage and autogenous bone grafting. 33of those that had curettage and bone cementing as well as 16.6of those that had resection presented with recurrence. One patient died within 3 months of surgery due to metastasis to the lungs; liver and spleen. Mean follow up was 9.2 months (range of 2 to 60 months). With early presentation; curretage and bone grafting is often effective; late presentation however has an increased risk of recurrence due to soft tissue involvement; dearth of investigative tools and financial constraints

Spectrum of bone tumors in Chiang Mai University Hospital, Thailand according to WHO classification 2002: A study of 1,001 cases.

OBJECTIVE: The aim of the present study was to determine the spectrum, frequency and demographics of bone tumors. MATERIAL AND METHOD: A retrospective study of the 1,001 bone tumor specimens from the files at the Pathology Department of the Chiang Mai University Hospital, Thailand from 2000 to 2004. RESULTS: From the study, 41 were non-neoplastic mass lesions, and 960 were neoplastic, with 856 (89%) as primary and 104 (11%) as metastatic tumors. In the primary tumor group, 654 (76%) cases were of hematologic origin, and 202 (24%) were non-hematologic. The most common benign bone tumors were giant cell tumor (n = 37), osteochondroma (n = 25), and chondroma (n = 15). The most common malignant bone tumors were lymphoma-leukemia (n = 583), metastatic malignancy (n = 104), plasma cell myeloma (n = 71), and osteosarcoma (n = 58). CONCLUSION: The present study showed a higher frequency of osteosarcoma (68%), lower frequencies of chondrosarcoma (12%) and Ewing sarcoma (4%) among primary non-hematologic malignant bone tumors when compared with similar studies based on Western patients. Whether these differences reflect differences in the ethnic population or in practice patterns remains to be determined