Juxtacortical osteosarcoma: clinical evolution and dedifferentiation related factors / Osteossarcoma justacortical: evolução clínica e fatores relacionados à desdiferenciação

ABSTRACT Objective: Evaluate risk factors related to clinical evolution and dedifferentiation of parosteal (juxtacortical) osteosarcoma to high-grade osteosarcoma. Methods: Retrospective cohort study performed over a period of 25 years, using data from medical records of patients diagnosed with parosteal osteosarcoma. The data were submitted to statistical analysis by Fisher's exact test and Student's t-test. Results: Of the 326 patients treated for osteosarcoma, we identified 17 patients diagnosed with parosteal osteosarcoma. Of these, 4 (23.5%) were not actually diagnosed with parosteal osteosarcoma and 4 did not have the minimum data required for analysis, being excluded from the study. Of the 9 patients studied, we observed that 3 (33.3%) evolved with tumor dedifferentiation to high-grade osteosarcoma. Moreover, 2 (66.7%) had local recurrence and 2 (66.7%) metastases. Conclusion: Age, sex, and the tumor size were not directly related to the dedifferentiation from parosteal osteosarcoma to high-grade osteosarcoma. The most aggressive clinical evolution - presence of local recurrences and metastasis - in parosteal osteosarcoma occurred in tumors with dedifferentiation, however, we cannot associate each other as cause and effect, but as related factors. Level of Evidence IV, Case Series.

RESUMO Objetivo: Avaliar fatores de risco relacionados à evolução clínica e à desdiferenciação do osteossarcoma justacortical (parosteal, paraosteal) em osteossarcoma de alto grau. Métodos: Estudo de coorte retrospectiva realizado num período de 25 anos. Foram utilizados dados de prontuários de pacientes com diagnóstico de osteossarcoma parosteal que, em seguida, foram submetidos à análise estatística pelo Teste Exato de Fisher e pelo Teste t de Student. Resultados: Foram tratados 326 pacientes com diagnóstico de osteossarcoma, dos quais 17 (5,21%) receberam diagnóstico de osteossarcoma parosteal, 4 (1,22%) foram diagnosticados com osteossarcoma convencional e 4 (1,22%) não tinham dados mínimos necessários para análise, sendo excluídos do estudo. Dos 9 (2,76%) pacientes estudados, 3 (0,92%) evoluíram com desdiferenciação do tumor para osteossarcoma de alto grau. Dois (0,84%) pacientes apresentaram recidiva local e 2 (0,84%%) apresentaram metástases. Conclusão: Os fatores idade, sexo e volume do tumor não estão diretamente relacionados com a desdiferenciação do osteossarcoma parosteal para osteossarcoma de alto grau. Apesar de a evolução clínica mais agressiva - presença de recidivas locais e metástase - no osteossarcoma parosteal ter ocorrido nos tumores com desdiferenciação, não é possível estabelecer uma relação de causa e efeito, apenas considerá-las como fatores relacionados. Nível de Evidência IV, Série de Casos.

Condrosarcoma periosteal mandibular / Mandibular periosteal chondrosarcoma

Los condrosarcomas son neoplasias malignas con diferenciación cartilaginosa, la mayoría de localización intraósea y en huesos largos; su incidencia en cabeza y cuello es baja y más aún, su localización periosteal y mandibular. Se presenta un caso de neoplasia maligna productora de matriz condroide, periosteal, de alto grado, recidivante y localizada en mandíbula.

The chondrosarcomas are malignant neoplasms characterized by cartilaginous differentiation, mainly located intraosseously, and in large bones. Their incidence in head and neck is rare and even more so when it is periosteal or located in the jaw. A case of a malignant tumor producing chondroid matrix, periosteal, of high-grade, recurrent and localized in the jaw is presente.

Características clínicas e epidemiológicas do paciente adolescente portador de osteossarcoma / Clinical and epidemiological characteristics of adolescent patients with osteosarcoma

O Osteossarcoma (OS) é uma neoplasia maligna que afeta o tecido ósseo, sem causa aparente, acomete mais o esqueleto apendicular, principalmente fêmur e tíbia de crianças e adolescentes. O tratamento consta de cirurgias de ressecção do tumor ou amputação de membros associados à quimioterapia. Quanto mais cedo se descobrir e tratar o OS, e quanto menor for a sua extensão melhor o prognóstico. Objetivo: Conhecer as características clínicas e epidemiológicas do paciente adolescente portador de OS atendido no Hospital do Câncer do Ceará (HCC) no Município de Fortaleza. Para isso foi investigado dados clínicos e epidemiológicos referentes aos pacientes adolescentes (de 10 à 19 anos) portadores de OS. Método: Realizou-se um estudo documental retrospectivo através dos prontuários dos pacientes atendidos nesse hospital no período de Janeiro de 2006 a Dezembro de 2007. Os dados foram coletados através de uma ficha semi-estruturada que constava de questões referentes às características clínicas e epidemiológicas dos pacientes, além dos dados de identificação. Foram selecionados 29 prontuários, sendo utilizados 26 para análise, pois 03 estavam incompletos por abandono de tratamento ou transferência hospitalar. Resultados: O OS foi mais frequente em pacientes do sexo masculino (57,7%), com idade entre 10 e 15 anos (73,1%), de raça afro-descendente (50%) e que residiam no interior (73,1%); 30,8% apresentavam história de câncer na família. O sintoma em comum detectado na queixa principal foi a dor (24 pacientes), seguida do aumento do volume local (20) e trauma prévio (08). O fêmur foi acometido em 65,4% dos casos, com ocorrência de metástase (76,9%), quase sempre ao diagnóstico e sua maioria (15 pacientes) para o pulmão. O tratamento consistia em quimioterapia (96,2%) associada a ressecção cirúrgica (69,2%) e amputação (73,1%) ou substituição por endoprótese. Outras especialidades como a fisioterapia foi prescrita em 42,3% dos casos tendo início no período do pós-operatório (23,1%) e geralmente para tratar as complicações. O paciente portador de OS se caracteriza por ser homem, afro-descendente, proveniente do interior e com antecedentes familiares de câncer. Conclusão: É necessário e importante conhecer as características clínicas e epidemiológicas do paciente portador de OS, redirecionando o olhar dos profissionais de saúde para a importância da inclusão de uma equipe multidisciplinar ao diagnóstico.

Osteosarcoma (OS) is a malignant neoplasia that affects the bone tissue with no known cause. It most commonly affects the appendicular skeleton, mainly the femur and tibia bones, in children and adolescents. The surgery treatment consists of a resection of the tumor or amputation of the affected limb, associated with chemotherapy. The earlier it is diagnosed and treated, and the smaller it is, the better the prognosis will be. Objective: The main objective of this study was to present the clinical characteristics and epidemiology of adolescent patients from the Ceará Cancer Hospital (HCC) in the city of Fortaleza. Clinical and epidemiologic data about these patients, who ranged from the age of 10 to 19 years old, was investigated in order to fulfill our objectives with this study. Method: A retrospective cohort study was developed gathering information from patients' charts from the above-mentioned hospital from the period of January 2006 to December 2007. The data was collected through a semi-structured form that consisted of questions that appraised both the clinical and epidemiological characteristics of the patients, as well as the demographic data. After selecting 29 cases, only 26 were used for the analysis due to either incomplete information, abandonment of treatment, or hospital transference. Results: Our results show that Osteosarcoma was found to be more frequent in male patients (57.7%), in patients between 10 and 15 years old (73.1%), in afro-descendant patients (50%), and in patients that came from more rural areas (73.1%). This study also shows that 30.8% of the patients presented a family history of cancer. The symptom that these patients seemed to have in common was pain (24 patients), followed by an increase of local volume (20 patients), and previous trauma (8 patients). The femur bone was affected in 65.4% of the cases, with occurrence of metastasis (76.9%), almost always during the diagnosis and with the majority (15 patients) to the lungs. The treatment consisted of chemotherapy (96.2%) associated with the surgical resection (69.2%) and amputation (73.1%) or limb replacement. Other treatments such as Physical Therapy were prescribed in 42.3% of the cases, mostly during post-surgery (23.1%), and generally just to treat the complications. Conclusion: This study suggests that the common profile of a patient with Osteosarcoma is male, afro-descendant, coming from the more rural areas and presenting a family history of cancer. In conclusion, this study suggests the importance of knowledge about both clinical and epidemiological characteristics of Osteosarcoma patients, so that there is more regard from health professionals concerning multidisciplinary teamwork at the time of the diagnosis.

Juxtacortical Spots on Fluid-Attenuated Inversion Recovery Images in Cryptogenic Transient Ischemic Attack

BACKGROUND AND PURPOSE: Juxtacortical spots are detected frequently on fluid-attenuated inversion recovery (FLAIR) images, but have not been extensively researched in patients with transient ischemic attack (TIA). We hypothesized that juxtacortical spots on FLAIR images are partly associated with right-to-left shunt (RLS) in TIA without clear etiology. The possibility of an association between the presence of RLS and juxtacortical spots on FLAIR images in patients with TIA without clear etiology was investigated, and the imaging findings of patients with and without RLS were compared. METHODS: This was a retrospective study of TIA patients who visited our tertiary stroke center consecutively within 72 hours of TIA onset. Cryptogenic TIA was defined as no clear etiology despite a routine diagnostic workup. The presence of RLS was examined by transcranial Doppler with an agitated saline test or transesophageal echocardiography. Juxtacortical spots were defined as small and round hyperintensities in the juxtacortex on FLAIR images, excluding white-matter hyperintensities. RESULTS: Of the 132 patients with cryptogenic TIA examined for this study, 70 (53.0%) had RLS. Juxtacortical spots on FLAIR images were detected more frequently in patients with RLS than in those without. The independent factors for the presence of juxtacortical spots were RLS [odds ratio (OR)=3.802, 95% confidence interval (95% CI)=1.74-8.2; p=0.001] and age (OR=1.058, 95% CI=1.01-1.10; p=0.004) by multivariate analysis. The number of juxtacortical spots was significantly higher among patients with a moderate-to-large RLS than in those with a small or no RLS. CONCLUSIONS: The findings of the present study demonstrate a significant association between the presence of RLS and the occurrence of juxtacortical spots on FLAIR images in patients with cryptogenic TIA.

Juxtacortical Glomus Tumor of the Tibia with an Uncertain Malignant Potential

Glomus tumors are mesenchymal neoplasms that are composed of modified smooth muscle cells of the normal glomus body. Most glomus tumors are benign and they occur in the distal extremities, and particularly the subungual lesions that occur in the hand, the wrist and the foot. We report here on a case of a solid type glomus tumor that had an uncertain malignant potential with a juxtacortical location at the distal tibia, and there were no neoplastic erosion of the cortical surface and no periosteal reaction. The tumor cells showed mild nuclear atypia and moderate mitotic activity (3-5/10HPF). Prominent intranuclear cytoplasmic pseudoinclusions were also observed. This case is interesting due to the very unusual tumor location of the juxtacortical area of the long bone, the atypical histologic features and the unique cytological finding of cytoplasmic intranuclear inclusions throughout the tumor cells.

Parosteal Ossifying Lipoma of Femur: A Case Report

Parosteal lipoma is a rare and generally asymptomatic benign tumor of mature adipose tissue that is located in direct apposition to the external surface of the bone. These tumors are occasionally associated with reactive changes in the underlying bone. The reactive bone formation is generally restricted to the base of the tumor, that is, near the periosteum. We recently experienced a case of parosteal lipoma in the proximal femur, which displayed exuberant bone and cartilage formation, and this led us to a misdiagnosis of chondroblastic osteosarcoma on the initial biopsy. We report here on this case with a special emphasis on making the differential diagnosis from osteosarcoma.

Periosteal Osteosarcoma Of The Mandible: Report Of A Case

Juxtacortical osteogenic sarcoma of chondroblastic type on the maxilla / 대한구강악안면방사선학회지

39-year-old female had been treated for the exophytic mass on buccal aspect of the left maxillary posterior area 2 years and 8 months ago. Tentative diagnosis was obtained as fibrous dysplasia on clinical and radiographic examinations and histopathologic findings revealed as osteochondroma after bone trimming at that time. She revisited for the treatment of recurred lesions. We reviewed this case with clinical, radiologic and histopathologic standpoints retrospectively, and came to a conclusion that the tumor primarily occurred was juxtacortical osteogenic sarcoma and recurred due to inadequate treatment and then expanded over intramedullary. This case shows that the diagnosis of osteosarcoma should take account of the patient history, clinical, radiographic and histopathologic findings and it requires attentive follow up check. Retrospectively reviewed results were as follows ; At first visit, oral examination revealed a bony hard swelling on the buccal aspect of the left maxillary posterior area. Radiographically, a dense radiopaque mass was noted on the site. The lesion showed hot uptake of 99mTc-MDP. Histopathologic diagnosis was done as osteochondroma, but it was considered as osteogenic sarcoma when compared with the recurrent lesion. When she revisited for the treatment of multiple bony swelling on the left maxilla, radiograms showed typical features of malignancy such as widening of periodontal ligament space and sun-ray appearace, and coincided with benign characters as follows ; relatively well circumscribed lesion and expansion and displacement of the adjacent structures. Finally, histopathologic findings of the lesion was well differentiated chondroblastic osteogenic sarcoma.