Jaw osteosarcoma and pregnancy: a rare coexistence

Osteosarcoma of the jaw represents less than 1% of all head and neck malignancies. This malignancy in pregnant women occurs in one per 1000 deliveries. We report a case of a 29-year-old woman, in the 33rd week of gestation, who presented with an expansive tumor destroying the maxillary alveolar bone, histologically composed of pleomorphic, round, spindle, or epithelioid cells and osteoid/chondroid matrix. Upon final diagnosis of osteosarcoma, the lesion was excised. To the best of our knowledge, only 10 cases of jaw osteosarcoma in pregnant women have been reported to date in the English language literature. The use of ancillary examinations, malignancy diagnosis, and cancer treatment can be challenging during pregnancy. Knowledge about jaw osteosarcoma in pregnancy can increase healthcare providers' awareness, avoid delays and misdiagnosis and potentially improve maternal and neonatal outcomes.

Fibroma ossificante trabecular juvenil: um relato de caso / Juvenile ossifying fibroma: a case report

Objetivo: o fibroma ossificante trabecular juvenil (FOJT) é uma lesão fibro-óssea incomum, benigna, porém de comportamento agressivo. Ocorre, frequentemente, em crianças e adolescentes, com maior acometimento em maxila e mandíbula. O presente relato aborda os aspectos clínicos, imaginológicos, histopatológicos, imuno-histoquímicos e o tratamento de um caso de FOTJ. Além de discutir suas similaridades com o osteossarcoma de baixo grau e as alternativas para o seu diagnóstico. Relato de caso: paciente do sexo masculino, com 12 anos de idade, apresentando aumento de volume em mandíbula no lado esquerdo, perda de função mastigatória e de fala, com tempo de evolução de seis meses. Radiograficamente, a lesão apresentava aspecto unilocular, com limites definidos e de padrão misto. O tratamento consistiu em cirurgia de enucleação total da lesão, seguida de curetagem sob anestesia geral. A histopatologia e a ausência de marcação para MDM2 e CDK4 na imuno-histoquímica confirmaram o diagnóstico de FOTJ e excluíram o de osteossarcoma de baixo grau. No controle pós-operatório de 20 dias, o paciente relatou melhora da parestesia, da função mastigatória e da fala e ausência de dor. Não se observou recidiva no acompanhamento de 1 ano após a cirurgia. Considerações finais: a similaridade dos aspectos clínico, imaginológico e microscópico entre FOJT e osteossarcoma de baixo grau pressupõe o estudo imuno-histoquímico com os arcadores MDM2 e CDK4, minimizando equívocos no diagnóstico e orientando de forma mais eficiente o plano de tratamento adotado.(AU)

Objective: juvenile Trabecular Ossifying Fibromas (JTOF) are uncommon benign fibro-osseous lesions with a local aggressive behavior. They frequently occur in children and adolescents involving the jaw bones. This report addresses the clinical, imaging, histopathological, and immunohistochemical aspects and the treatment of a case of JTOF. In addition, it discusses similarities between JTOF and low-grade osteosarcomas and alternatives for their diagnosis. Case report: male patient, 12 years old, with a tumor in his left mandible, loss of masticatory function and speech, with a 6-month time course. Radiographically, the lesion had an unilocular aspect, with defined borders and a mixed image pattern. Treatment consisted surgical enucleation, followed by bone curettage, under general anesthesia. Histopathology presented fibrous stroma interspaced with bone trabeculae without signs of cellular malignancy. MDM2 and CDK4 markers in immunohistochemistry, confirmed the diagnosis of JTOF and excluded low-grade osteosarcoma. At the 20-day postoperative follow-up, the patient reported improvement in paresthesia, masticatory function and speech, and the absence of pain. Evaluation 1 year after surgery showed no signs of local recurrence. Final considerations: the similarity of clinical, imaging, and microscopic aspects between JTOF and low-grade osteosarcoma prompts an immunohistochemical study including MDM2 and CDK4 markers in order to improve final diagnosis and guide the treatment plan more efficiently.(AU)

Fibroblastic osteosarcoma in a white-eared opossum (Didelphis albiventris) / Osteossarcoma fibroblástico em gambá-de-orelha-branca (Didelphis albiventris)

Osteosarcoma is characterized by the production of osteoid or woven bone, using neoplastic osteoblasts. It is the most common primary bone neoplasm in canines and humans. This neoplasm was previously reported in all vertebrate classes, including a wide variety of mammals. However, there is no case report describing this neoplasm in Didelphis albiventris. Therefore, the objective of this manuscript is to describe the clinical-pathological aspects of fibroblastic osteosarcoma in D. albiventris. A wild adult male white-eared opossum (D. albiventris) arrived at the zoological park of the "Universidade de Caxias do Sul" with a swollen left thoracic limb. After a general clinical examination, the animal was transferred to the Veterinary Clinic of "Universidade de Caxias do Sul" for radiographic examination of the left thoracic limb and thorax. Additionally, some material was collected through fine needle aspiration (FNA) for cytologic evaluation. The radiographic findings and cytologic evaluation indicated osteosarcoma. The animal was euthanized due to severe clinical conditions and guarded prognosis. During necropsy, macroscopic analysis of the viscera was performed, fragments of various organs were collected and fixed in 10% neutral buffered formalin. All fragments were processed following routine histological techniques. The histopathological evaluation confirmed osteosarcoma, which was classified as a fibroblastic subtype. Case reports are crucial for the knowledge of incidence, prevalence, and behavior of the current mentioned disease, as well as other diseases, in species with such limited information. In order to obtain a decisive diagnosis, a few different examination methods were associated. Although the observations presented are based on a single case, this neoplasm had a similar clinical presentation to that described in other species.(AU)

O osteossarcoma é caracterizado pela produção de osteoide ou osso imaturo, por osteoblastos neoplásicos. É a neoplasia óssea primária mais comum em caninos e humanos. Essa neoplasia já foi relatada em todas as classes de vertebrados, incluindo uma grande variedade de mamíferos. Não havendo descrição dessa neoplasia até o momento em Didelphis albiventris. O objetivo deste trabalho é descrever aspectos clínico-patológicos de um caso de osteossarcoma fibroblástico em D. albiventris. Chegou para atendimento no Zoológico da Universidade de Caxias do Sul um gambá-de-orelha-branca (D. albiventris), macho, adulto, de vida livre com aumento de volume no membro torácico esquerdo. Após avaliação clínica geral, o animal foi encaminhado para a Clínica Veterinária da Universidade de Caxias do Sul para realização de radiografia do membro torácico esquerdo e de tórax, sendo também realizada coleta de material por punção aspirativa por agulha fina (PAAF) para avaliação citológica. Os achados radiográficos e da avaliação citológica foram sugestivos de osteossarcoma. Devido ao estado clínico grave e prognóstico reservado optou-se pela eutanásia. Durante a necropsia realizou-se a análise macroscópica das vísceras, foram coletados fragmentos de diversos órgãos, fixados em formalina 10%, processados pelas técnicas histológicas de rotina. Na avaliação histopatológica confirmou-se a suspeita de osteossarcoma sendo classificado no subtipo fibroblástico. A descrição de relatos é fundamental para conhecimento da incidência, prevalência e comportamento desta e de outras doenças em espécies que as informações são limitadas. A associação de diferentes métodos de exames foram necessários para a obtenção de um diagnóstico definitivo. Embora as observações apresentadas se baseiem em um caso único, esta neoplasia possuiu apresentação clínica semelhante a descrita em outras espécies.(AU)

Delays in the health care system for children, adolescents, and young adults with bone tumors in Brazil / Atrasos no sistema de saúde para crianças, adolescentes e adultos jovens com tumores ósseos no Brasil

ABSTRACT Objective: To identify delays in the health care system experienced by children and adolescents and young adults (AYA; aged 0-29 years) with osteosarcoma and Ewing sarcoma using information from the Brazilian hospital-based cancer registries. Methods: Patient data were extracted from 161 Brazilian hospital-based cancer registries between 2007 and 2011. Hospital, diagnosis, and treatment delays were analyzed in patients without a previous histopathological diagnosis. Referral, hospital, and health care delays were calculated for patients with a previous histopathological diagnosis. The time interval was measured in days. Results: There was no difference between genders in overall delays. All delays increased at older ages. Patients without a previous histopathological diagnosis had the longest hospital delay when compared to patients with a previous histopathological diagnosis before first contact with the cancer center. Patients with Ewing sarcoma had longer referral and health care delays than those with osteosarcoma who had a previous histopathological diagnosis before first contact with the cancer center. The North and Northeast regions had the longest diagnosis delay, while the Northeast and Southeast regions had the longest treatment delay. Conclusion: Health care delay among patients with a previous diagnosis was longer, and was probably associated with the time taken for to referral to cancer centers. Patients without a previous histopathological diagnosis had longer hospital delays, which could be associated with possible difficulties regarding demand and high-cost procedures. Despite limitations, this study helps provide initial knowledge about the healthcare pathway delays for patients with bone cancer inside several Brazilian hospitals.

RESUMO Objetivo: Identificar atrasos no sistema de saúde em crianças e adolescentes e adultos jovens (AAJ; até 29 anos) com osteossarcoma e sarcoma de Ewing com informações dos registros de câncer de base hospitalar do Brasil. Métodos: Os dados dos pacientes foram extraídos de 161 registros de câncer de base hospitalar brasileiros entre 2007 e 2011. Os atrasos no hospital, no diagnóstico e no tratamento foram analisados em pacientes sem um diagnóstico histopatológico anterior. Os atrasos no encaminhamento, no hospital e no sistema de saúde foram calculados para pacientes com diagnóstico histopatológico anterior. O intervalo de tempo foi medido em dias. Resultados: Não houve diferença entre os sexos nos atrasos em geral. Todos os atrasos aumentaram na faixa etária mais velha. Os pacientes sem um diagnóstico histopatológico anterior apresentaram o atraso hospitalar mais longo em comparação com os pacientes com diagnóstico histopatológico anterior antes do primeiro contato com o centro de câncer. Os pacientes com sarcoma de Ewing apresentaram atrasos no encaminhamento e no sistema de saúde mais longos do que os com osteossarcoma, que apresentaram diagnóstico histopatológico anterior antes do primeiro contato com o centro oncológico. As regiões Norte e Nordeste apresentaram o atraso mais longo no diagnóstico, ao passo que as regiões Nordeste e Sul apresentaram o atraso mais longo no tratamento. Conclusão: O atraso no sistema de saúde entre os pacientes com diagnóstico anterior foi maior e provavelmente associado ao tempo de encaminhamento para os centros oncológicos. Os pacientes sem um diagnóstico histopatológico anterior apresentaram atrasos mais longos no hospital, o que pode ser associado a possíveis dificuldades com relação à demanda e aos procedimentos de alto custo. Apesar das limitações, nosso estudo ajuda a fornecer um conhecimento inicial sobre os atrasos no sistema de saúde para tratamento de pacientes com câncer em vários hospitais brasileiros.

Lung metastases at the initial diagnosis of high-grade osteosarcoma: prevalence, risk factors and prognostic factors. A large population-based cohort study

ABSTRACT BACKGROUND: Osteosarcoma is the most prevalent malignant bone tumor in children and adolescents. Lung metastases are associated with poor prognosis. OBJECTIVE: The aim here was to explore the prevalence of and risk and prognostic factors for lung metastases in high-grade osteosarcoma patients. DESIGN AND SETTING: Retrospective cohort study based on the Surveillance, Epidemiology and End Results (SEER) database in the United States. METHODS: Data on 1,408 high-grade osteosarcoma patients registered in the SEER database between 2010 and 2015 were extracted. From these, all patients with high-grade osteosarcoma and initial lung metastasis were selected for analysis on risk and prognostic factors for lung metastases. Overall survival was estimated. RESULTS: There were 238 patients (16.90%) with lung metastases at diagnosis. Axial location, tumor size > 10 cm (odds ratio, OR 3.19; 95% confidence interval, CI: 1.58-6.45), higher N stage (OR 4.84; 95% CI: 1.94-12.13) and presence of bone metastases (OR 8.73; 95% CI: 4.37-17.48) or brain metastases (OR 25.63; 95% CI: 1.55-422.86) were significantly associated with lung metastases. Younger age and surgical treatment (hazard ratio, HR 0.46; 95% CI: 0.30-0.71) favored survival. Median survival was prolonged through primary tumor surgery. CONCLUSIONS: The factors revealed here may guide lung metastasis screening and prophylactic treatment for osteosarcoma patients. A primary tumor in an axial location, greater primary tumor size, higher lymph node stage and presence of bone or brain metastases were significantly correlated with lung metastases. The elderly group (≥ 60 years) showed significant correlation with poor overall survival. For improved survival among high-grade osteosarcoma patients with lung metastases, aggressive surgery on the primary tumor site should be encouraged.

Osteosarcoma "tipo osteoblastoma" de columna cervical: A propósito de un caso / Osteoblastoma-like osteosarcoma of the cervical spine. A case report

El osteosarcoma "tipo osteoblastoma", una variante menos agresiva del osteosarcoma, es una enfermedad poco frecuente y representa un desafío diagnóstico tanto clínico como histopatológico. Se han publicado escasos reportes de casos de este tumor. Presentamos a un paciente de 16 años con un osteosarcoma "tipo osteoblastoma" localizado en la columna cervical. La biopsia ósea bajo tomografía reveló una lesión con un patrón de crecimiento permeativo, con sospecha de osteosarcoma "tipo osteoblastoma". Se realizó una espondilectomía total en bloque mediante un doble abordaje. Se describen el caso clínico, la secuencia diagnóstica, la técnica quirúrgica y el seguimiento a 10 años. Nivel de Evidencia: IV

Osteoblastoma-like osteosarcoma, a less aggressive variant of osteosarcoma, is a rare tumor which presents a clinical and histopathological diagnostic challenge. We have found few reports of cases of this tumor in the literature. In this case, we describe an osteoblastoma-like osteosarcoma of the cervical spine in a 16-year-old patient. CT-guided bone biopsy showed a lesion with a permeated growth pattern, which led to the suspicion of an osteoblastoma-like osteosarcoma. A total en bloc spondylectomy was performed through a dual approach. We present the clinical case, the diagnostic sequence, the surgical technique and 10-year follow-up results. Level of Evidence: IV

Osteossarcoma: atualização / Osteosarcoma: update

Introdução: O osteossarcoma é o tumor maligno primário dos ossos mais frequente. Esta patologia é classicamente descrita como neoplasia de alto grau de células malignas que produzem osteoides. Objetivo: Abordar sobre sinais e sintomas associados ao osteossarcoma, bem como, diagnóstico e manejo. Método: Estudo realizado através de artigos, literatura e relatos de caso sobre osteossarcoma. Resultados: O diagnóstico precoce é decisivo quando discutimos sobre prognóstico, tratamento cirúrgico e quimioterápico para ressecção do tumor, na tentativa de evitar amputação de membros. Conclusão: Uma boa anamnese, reconhecendo desde os primeiros sinais e sintomas, até a chegada em um serviço de saúde terciário é de extrema importância. Além disso, devemos identificar as características clínicas e epidemiológicas desta doença, com auxílio de equipes multidisciplinares desde o diagnóstico até o tratamento destes pacientes.

Introduction: Osteosarcoma is the most frequently primary malignant bone tumor. Classically, this disease is described as high-grade neoplasm of malignant cells that produce osteoids. Objective: Approach to signs and symptoms related to osteosarcoma, as well as diagnosis and management. Method: Study performed through articles, literature and case reports osteosarcoma. Results: Early diagnosis is decisive when discussing prognosis, surgical and chemotherapy treatment for tumor resection in attempt to avoid limb amputations. Conclusion: A good medical history, recognizing from the first signs and symptoms until arrival in a tertiary health service, is extremely important. Furthermore, we should identify clinical and epidemiological characteristics of this disease with help of multidisciplinary teams, from diagnosis to treatment of these patients.

Osteosarcoma telangiectásico en un lactante / Telangiectatic osteosarcoma in an infant

Resumen: Introducción: El osteosarcoma es uno de los tipos de cáncer más común en la etapa escolar y en la adolescencia, y es el más común de los tumores óseos malignos en este grupo de edad. Frecuentemente, el osteosarcoma es hallado en los huesos largos de las extremidades. Existen muy pocos casos descritos en menores de 5 años. Hasta donde se sabe, no hay ningún caso reportado en lactantes. La variedad telangiectásica es poco común y no existen casos reportados antes de la edad preescolar. Caso clínico: Se presenta el caso clínico de una paciente lactante de 10 meses de edad que presentó un tumor lítico en la novena costilla derecha, que posterior a la resección fue clasificado como osteosarcoma telangiectásico. Conclusiones: El osteosarcoma telangiectásico en la edad pediátrica es poco común. A la fecha, la paciente ha presentado buena respuesta al tratamiento, aunque se desconoce el pronóstico y la supervivencia de este padecimiento.

Abstract: Background: Osteosarcoma is one of the most common types of cancer in childhood and adolescence and it is the most common malignant bone tumor in this group of age. Osteosarcoma is frequently found in long bones of the extremities. There are very few cases described in children under 5 years of age, and according to this review, none in infants. The telangiectatic variant is uncommon, and there are no reported cases before preschool age. Case report: A 10-month-old female infant with a lithic tumor of the ninth right rib, which was classified after resection as a telangiectatic osteosarcoma, is presented. Conclusions: Telangiectatic osteosarcoma in the pediatric age is very uncommon. To date, the patient has presented good response to treatment, although the prognosis and survival of this condition is unknown.

Osteosarcoma patient outcomes at a South African tertiary hospital

Background. Osteosarcoma is the most common primary malignant bone tumour. There is a high incidence of late presentation in the developing world, posing additional challenges in the treatment of this aggressive disease. Objective. To evaluate clinical outcomes of patients with osteosarcoma at Charlotte Maxeke Johannesburg Academic Hospital (CMJAH), a tertiary hospital in South Africa, and compare these with similar studies in the developing world.Methods. This was a retrospective study of 61 patients treated at CMJAH between 2007 and 2011, with a minimum follow-up of 1.3 years (range 1.3 - 6.3).Results. An average of 4.5 months elapsed before patients were first seen at the CMJAH tumour unit. Fifty-eight patients (95.1%) initially sought conventional medical care. Three patients (4.9%) presented with pathological fractures. All the patients underwent biopsy, performed an average of 3 weeks after arrival at the tumour unit. In most cases the delay was due to limited access to magnetic resonance imaging. Most patients (n=41, 67.2%) were at Enneking stage 2B, 4 (6.6%) were at stage 2A and 16 (26.2%) were at stage 3. Of the patients, 13 (21.3%) underwent limb salvage procedures, 33 (54.1%) had amputations, 4 (6.6%) refused further treatment and 11 (18.0%) received palliative care only; 55 patients (90.2%) received chemotherapy. Two patients developed local recurrence, one of whom had an amputation and the other further wide excision. Two patients received palliative radiotherapy. Of the patients, 82.0% were HIV-negative, 4.9% HIV-positive and the rest of unknown status. At the time of the study, all but two patients, who came from other countries, were traceable or known to have died. Our overall 1-year and 5-year survival rates were 62.7% (95% confidence interval (CI) 49.1 - 73.9) and 38.1% (95% CI 24.6 - 51.4), respectively. Male patients and those with a higher Enneking stage had a poorer prognosis.Conclusion. Although most patients sought conventional medical care, unacceptable delays worsened survival. However, our survival rates are better than those in other developing countries. We advocate that professional, public and political awareness of osteosarcoma be improved as a matter of urgency, to facilitate rapid tertiary referral and expedite management

Itinerário terapêutico de adolescentes com osteossarcoma: implicações para o diagnóstico precoce / Therapeutic study of teens with osteosarcoma: implications for early diagnosis / Itinerario terapéutico de adolescentes con osteosarcoma: implicaciones para el diagnóstico temprano

Vários fatores interferem na demora do diagnóstico e tratamento de adolescentes portadores de osteossarcoma. O objetivo deste artigo foi descrever o itinerário terapêutico de adolescentes portadores de osteossarcoma. Trata-se de estudo descritivo, do tipo relato de caso, realizado numa instituição pública especializada em Oncologia no estado do Rio de Janeiro. Foram realizados três estudos de casos de adolescentes portadores de osteossarcoma amputados com a descrição do itinerário terapêutico. Foram identificadas questões relacionadas a dificuldades do diagnóstico precoce, como a relação familiar com o adolescente, a fragilidade de uma rede de saúde adequada ao adolescente e a dificuldade de um diagnóstico de câncer infantil. O diagnóstico precoce deve ser divulgado por meio de um sistema de saúde integrado da atenção básica com o atendimento especializado. É fundamental, para que ocorra a detecção precoce do osteossarcoma nos adolescentes, um sistema de saúde integrado, da atenção básica com o atendimento especializado, a fim de evitar a descoberta de novos casos mais tardiamente.

Several factors interfere with the delay of diagnosis and treatment of adolescents suffering from osteosarcoma. The purpose of this article wasto describe the therapeutic itinerary of adolescents suffering from osteosarcoma. This is a descriptive study of type case report, carried out in apublic institution specialized in Oncology in the State of Rio de Janeiro. Three case studies of adolescents suffering from osteosarcoma amputees with the description of the itinerary were conducted. Related issues have identified the difficulties of early diagnosis, as the family relationship with the teenager; the fragility of a health network suitable for the teenager and the difficulty of a diagnosis of childhood cancer. Early diagnosis shouldbe disclosed through a system of integrated health basic attention with the specialized care. It is essential for early detection of osteosarcoma in adolescents, an integrated health system, basic care with specialized care, in order to prevent the discovery of new cases later.

Son varios los factores que interfieren en la demora del diagnóstico y tratamiento de adolescentes con osteosarcoma. El propósito de este artículoera describir el itinerario terapéutico de adolescentes con osteosarcoma. Se trata de un estudio descriptivo de caso llevado a cabo en una institución pública especializada en oncología en el estado de Río de Janeiro. Se realizaron tres estudios de caso de adolescentes con osteosarcoma amputados con la descripción del itinerario terapéutico. Se identificaron problemas relacionados con las dificultades del diagnóstico temprano, tales como larelación familiar con el adolescente; la fragilidad de la red de salud adecuada para el adolescente y la dificultad del diagnóstico de cáncer infantil.El diagnóstico temprano debe ser revelado a través de un sistema de atención básica de la salud integrado con atención especializada. Para la detección temprana del osteosarcoma en adolescentes es fundamental que el sistema de salud sea integrado, de atención básica con atención especializada, para evitar el descubrimiento de nuevos casos más tarde.

Characteristics and Outcomes of Second Malignant Neoplasms after Childhood Cancer Treatment: Multi-Center Retrospective Survey

This retrospective study investigated the clinical characteristics and outcomes of second malignant neoplasms (SMNs) in survivors of childhood cancer from multiple institutions in Korea. A total of 102 patients from 11 institutions who developed SMN after childhood cancer treatment between 1998 and 2011 were retrospectively enrolled. The most common primary malignant neoplasms (PMNs) were central nervous system (CNS) tumors (n = 17), followed by acute lymphoblastic leukemia (n = 16), non-Hodgkin lymphoma (n = 13), and osteosarcoma (n = 12). The most common SMNs were therapy-related myeloid neoplasms (t-MNs; acute myeloid leukemia [AML], 29 cases; myelodysplastic syndrome [MDS], 12 cases), followed by thyroid carcinomas (n = 15) and CNS tumors (n = 10). The median latency period was 4.9 years (range, 0.5-18.5 years). Among 45 patients with solid tumors defined as an SMN, 15 (33%) developed the lesion in a field previously subjected to radiation. The 5-year overall survival (OS) rate of patients with an SMN was 45% with a median follow-up time of 8.6 years. Patients with AML, MDS, and CNS tumors exhibited the poorest outcomes with 5-year OS rates of 18%, 33%, and 32%, respectively, whereas those with second osteosarcoma showed comparable outcomes (64%) to patients with primary counterpart and those with second thyroid carcinoma had a 100% OS rate. Further therapeutic efforts are recommended to improve the survival outcomes in patients with SMNs, especially in cases with t-MNs and CNS tumors.

Characteristics and Outcomes of Second Malignant Neoplasms after Childhood Cancer Treatment: Multi-Center Retrospective Survey

This retrospective study investigated the clinical characteristics and outcomes of second malignant neoplasms (SMNs) in survivors of childhood cancer from multiple institutions in Korea. A total of 102 patients from 11 institutions who developed SMN after childhood cancer treatment between 1998 and 2011 were retrospectively enrolled. The most common primary malignant neoplasms (PMNs) were central nervous system (CNS) tumors (n = 17), followed by acute lymphoblastic leukemia (n = 16), non-Hodgkin lymphoma (n = 13), and osteosarcoma (n = 12). The most common SMNs were therapy-related myeloid neoplasms (t-MNs; acute myeloid leukemia [AML], 29 cases; myelodysplastic syndrome [MDS], 12 cases), followed by thyroid carcinomas (n = 15) and CNS tumors (n = 10). The median latency period was 4.9 years (range, 0.5-18.5 years). Among 45 patients with solid tumors defined as an SMN, 15 (33%) developed the lesion in a field previously subjected to radiation. The 5-year overall survival (OS) rate of patients with an SMN was 45% with a median follow-up time of 8.6 years. Patients with AML, MDS, and CNS tumors exhibited the poorest outcomes with 5-year OS rates of 18%, 33%, and 32%, respectively, whereas those with second osteosarcoma showed comparable outcomes (64%) to patients with primary counterpart and those with second thyroid carcinoma had a 100% OS rate. Further therapeutic efforts are recommended to improve the survival outcomes in patients with SMNs, especially in cases with t-MNs and CNS tumors.

Undifferentiated osteosarcoma of the mandible oblique line: a case report / Osteossarcoma indiferenciado da mandíbula: relato de caso clínico na região do trígono retromolar

O osteossarcoma (OS) é o tumor maligno primário mais comum. Entretanto, o osteossarcoma localizado na mandíbula (JOS) é raro, agressivo e maligno, constituindo de 5-13% dos casos de OS do corpo todo. O JOS tem predileção pelo sexo masculino com idade de 34-36 anos. O prognóstico da doença está associado com diversas variáveis tais como a localização do tumor, fase de desenvolvimento, existência ou não de metástases, sexo, idade, e resposta do organismo ao tratamento. Este artigo relata um caso incomum de osteossarcoma localizado na região do trígono retromolar mandibular, composto por osso mesenquimal primitivo, que comumente ocorre nas regiões de extremidades e ossos longos...

This article reports a case of 82 years old woman, who consulted in a private radiology dental clinic for implants planning. In a panoramic radiography, a radiopaque area with sun-ray appearance located above the external oblique line on the right side was observed. The incisional biopsy showed presence of sarcomatous stroma presenting osteoid tissue with irregularly shaped and large amount of osteoblasts, varied sizes and shapes, with prominent nuclei, intensely colored, arranged in a disorderly way around trabecular bone. Histopathological diagnosis was obtained for undifferentiated osteosarcoma. Osteosarcoma located in the jaw (JOS) is rare and aggressive, constituting 5% to 13% of all cases of skeletal osteosarcoma. JOS has a male predilection in third decade of life...

Plain Radiologic Findings and Chronological Changes of Incipient Phase Osteosarcoma Overlooked by Primary Physicians

BACKGROUND: We assessed the plain radiographic characteristics of 10 cases of osteosarcomas during the initial painful period that had been overlooked by a primary physician. In addition, we evaluated chronologic changes in radiographic findings from initial symptomatic period to the time of accurate diagnosis. METHODS: The clinical records were reviewed for clinical parameters including age, sex, location, presenting symptoms, initial diagnosis, duration from initial symptoms to definite diagnosis, and initial and follow-up plain radiographic findings of the lesion. RESULTS: Initial clinical diagnoses included a sprain in 6, growing pain in 2, stress fracture in 1, and infection in 1 patient. Initial plain radiographic findings were trabecular destruction (100%), cortical disruption (60%), periosteal reaction (60%), and soft tissue mass (10%). Intramedullary matrix changes were osteosclerosis in 6 and osteolysis in 4 patients. On progression, 4 cases with minimal sclerosis changed to osteoblastic lesion in 3 patients and osteolytic lesion in 1. Four cases with faint osteolytic foci transformed into osteolytic lesion in 3 and mixed pattern in 1. CONCLUSIONS: Notable plain radiologic findings of incipient-stage osteosarcoma include trabecular disruption along with faint osteosclerosis or osteolysis. In symptomatic patients with trabecular destruction, additional imaging study including magnetic resonance imaging should be performed to exclude osteosarcoma in the incipient phase, even without radiologic findings suggesting malignant tumor, such as cortical destruction or periosteal reaction.

Fibrocartilaginous Intramedullary Bone Forming Tumor of the Distal Femur Mimicking Osteosarcoma

Fibrocartilaginous dysplasia (FCD) has occasionally led to a misdiagnosis and wrong decision which can significantly alter the outcome of the patients. A 9-yr-old boy presented with pain on his left distal thigh for 6 months without any trauma history. Initial radiographs showed moth eaten both osteolytic and osteosclerotic lesions and biopsy findings showed that the lesion revealed many irregular shaped and sclerotic mature and immature bony trabeculae. Initial diagnostic suggestions were varied from the conventional osteosarcoma to low grade central osteosarcoma or benign intramedullary bone forming lesion, but close observation was done. This study demonstrated a case of unusual fibrocartilaginous intramedullary bone forming tumor mimicking osteosarcoma, so that possible misdiagnosis might be made and unnecessary extensive surgical treatment could be performed. In conclusion, the role of orthopaedic oncologist as a decision maker is very important when the diagnosis is uncertain.

Extraskeletal osteosarcoma, telangiectatic variant arising from the small bowel mesentery

Extraskeletal osteosarcoma [EOS] is a highly aggressive and rare malignant soft tissue tumor, characterized by the production of neoplastic osseous tissue without attachment to the bone or periosteum. It rarely involves the visceral organs. Only 3 cases of mesenteric EOS have been reported in English literature. Here, we describe a male patient of 40 years, who was diagnosed to have EOS arising from small bowel mesentery This patient presented with lower gastrointestinal [GI] bleeding. Upper GI endoscopy and colonoscopy were normal. Computed tomography scan demonstrated a well defined multi-loculated mixed density mass lesion measuring about 13x7x7 cm in lower abdomen adjacent to small bowel loops with liver metastasis. Palliative en bloc resection of tumor with adjacent small bowel was performed. The histopathology revealed a telangiectatic type osteosarcoma of mesentery. Diagnosis of EOS, its management and the outcome in context of the current literature are discussed

MR Imaging Findings of a Primary Cardiac Osteosarcoma and Its Bone Metastasis with Histopathologic Correlation

An osteosarcoma of cardiac origin is extremely rare, and a comprehensive description of MR imaging (MRI) findings of cardiac osteosarcoma and its metastasis in the femur have not been reported in the literature. We present a case of cardiac osteosarcoma in a 47-year-old woman and its metastasis to the femur, focusing on the description of MRI findings of the cardiac and metastatic bony osteosarcoma with a histopathologic correlation.

Primary osteoliposarcoma of the bone.

Osteoliposarcoma are rare tumors of the bone. To our knowledge, so far only eight cases have been reported. Some of the reports have referred to these tumors as malignant mesenchymoma. We report a case of a male with osteoliposarcoma of the right femur who presented with pain and restricted movements of the right knee joint. Radiologically, a tumor in the lower end of the right femur was seen extending into the soft tissue. The biopsy was reported as osteosarcoma. The patient received three cycles of neo-adjuvant chemotherapy followed by limb-salvage surgery with provisions for a custom-made prosthesis. A histopathological study of the excision specimen revealed areas of pleomorphic liposarcoma with numerous osteoblasts associated with areas of osteoid surrounded by neoplastic cells. The final diagnosis was osteoliposarcoma. Only 21% tumor necrosis (effects of chemotherapy) was observed. Presently, 26 months following diagnosis, the patient is fine with no evidence of local recurrence or distant metastasis.