Fibroma ossificante juvenil trabecular: relato de caso / Juvenile trabecular ossifying fibroma: case report / Fibroma osificante trabecular juvenil: reporte de un caso

O fibroma ossificante juvenil trabecular (FOJTr) é uma lesão fibro-óssea benigna rara de comportamento agressivo, alto potencial de recorrência, e acometimento no esqueleto craniofacial de crianças e adolescentes. Uma paciente do gênero feminino, 8 anos de idade, compareceu ao ambulatório de Patologia Oral e Maxilofacial da Universidade de Gurupi ­ UNIRG para avaliação clínica de um aumento de volume na região de corpo da mandíbula do lado esquerdo. Não havia sintomatologia dolorosa e sequer desconforto. Nos exames de imagem (radiografia panorâmica e tomografia computadorizada) foram observados uma extensa área radiolúcida que se estendia desde o primeiro molar permanente com rizogênese incompleta até o incisivo central do lado oposto. Após a realização da biópsia incisional e laudos histopatológicos realizou-se a remoção completa da lesão incluindo os remanescentes decíduos sobrejacentes ao fibroma. Nas imagens de controle pós-operatório aos 90 dias (radiografia panorâmica e tomografia computadorizada), notou-se sinais de neoformação óssea com espessamento basilar e os germes dos dentes permanentes em franco desenvolvimento. Diante disso, ressalta-se a importância do conhecimento dos aspectos clínicos, radiográficos e histopatológicos para a realização de um correto diagnóstico e tratamento adequado afim de reduzir as altas taxas de recidivas... (AU)

Trabecular juvenile ossifying fibroma (TrJOF) is a rare benign fibro-osseous lesion, with aggressive behavior, high recurrence potential, which affects the craniofacial skeleton of children and adolescents. This paper aims to describe a clinical case in a female patient, 8 years old, who attended the Oral and Maxillofacial Pathology outpatient clinic ­ Faculty of Dentistry ­ University of Gurupi - UNIRG, city of Gurupi - TOCANTINS - BRAZIL for clinical evaluation of an increased in volume in the region of the mandible body, on the left side. There was no painful symptomatology or even discomfort. Imaging examinations (panoramic radiography and computed tomography (CT) showed an extensive radiolucent area that extended from the first permanent molar with incomplete root formation to the central incisor on the opposite side. After performing an incisional biopsy and histopathological examination, the lesion was completely removed included the remainder deciduous teeth overlying the tumor. In the postoperative control images at 90 days (panoramic radiography and CT), signs of bone neoformation with basilar thickening and the germs of the permanent teeth in full development were noted. In view, this importance of knowledge of clinical, radiographic and histopathological aspects is emphasized for the realization of a correct diagnosis and adequate treatment in order to reduce the high rates of relapses... (AU)

El fibroma osificante trabecular juvenil (TRFOJ) es una lesión fibroósea benigna rara con comportamiento agresivo, alto potencial de recurrencia y afectación del esqueleto craneofacial de niños y adolescentes. Paciente femenina de 8 años de edad que acude al ambulatorio de Patología Oral y Maxilofacial de la Universidad de Gurupi - UNIRG para evaluación clínica de aumento de volumen en la región del cuerpo mandibular del lado izquierdo. No presentaba sintomatología dolorosa ni molestias. Los exámenes de imagen (radiografía panorámica y tomografía computarizada) mostraron una extensa área radiolúcida que se extendía desde el primer molar permanente con formación radicular incompleta hasta el incisivo central del lado opuesto. Tras realizar la biopsia incisional y los informes histopatológicos, se procedió a la extirpación total de la lesión, incluidos los remanentes caducos que recubrían el fibroma. En las imágenes de control postoperatorio a los 90 días (radiografía panorámica y tomografía computarizada), se observaron signos de neoformación ósea con engrosamiento basilar y los gérmenes de los dientes permanentes en pleno desarrollo. Por tanto, es importante conocer los aspectos clínicos, radiográficos e histopatológicos para la realización de un diagnóstico correcto y un tratamiento adecuado con el fin de reducir las altas tasas de recaídas... (AU)

Osteocondroma solitario de la escápula: localización poco habitual para un tumor frecuente / A solitary osteochondroma of the scapula: Uncommon location for a common tumor

El osteocondroma es un tumor óseo benigno que afecta, en general, a niños y adultos jóvenes. Se localiza habitualmente en las metáfisis de los huesos largos alrededor de la rodilla, aunque también puede afectar a huesos planos como la escápula.Se presenta el caso clínico de un niño de 11 años con tumoración dolorosa en la superficie dorsal de la escápula de un año de evolución. Se diagnostica un osteocondroma escapular, localización poco frecuente para este tumor. Debido a la persistencia de la sintomatología, se realizó exéresis quirúrgica

Osteochondroma is a benign bone tumor that usually affects children and young adults. It is typically located in the metaphysis of long bones around the knee, although it could also affect flat bones like scapula. We report the case of a 11-year-old child with one year of evolution painful tumor at the dorsal surface of the scapula. He was diagnosed with osteochondroma of the scapula, uncommon location for this tumor. Due to the persistence of the symptomatology surgical excision was performed.

Adenopatia cervical: uma apresentação rara de câncer da próstata / Cervical lymphadenopathy ­ a rare presentation of prostate cancer

A metastização ganglionar cervical por neoplasia da próstata é rara, sendo ainda menos frequente como manifestação inicial da doença. O presente estudo é um relato de um caso clínico de uma pessoa do sexo masculino, com 72 anos, que apresentava massa cervical esquerda, indolor, com 2 meses de evolução e dores ósseas lombar e torácica. A citologia aspirativa por agulha fina com estudo imuno-histoquímico revelou positividade para o antígeno prostático específico, concluindo se tratar de metástase ganglionar de carcinoma da próstata. Analiticamente, constatou-se que o valor do antígeno prostático específico foi maior que 1.000ng/mL, além da elevação da fosfatase alcalina. A cintilografia óssea de corpo inteiro revelou envolvimento ósseo secundário. Após o diagnóstico, o paciente iniciou hormonoterapia e recusou radioterapia com intuito paliativo. Oito meses após o diagnóstico, constatou-se a recorrência da doença, com elevação do valor do antígeno prostático específico novamente. Dessa forma, relata-se um caso de neoplasia da próstata com metastização óssea e ganglionar cervical esquerda em um indivíduo assintomático do ponto de vista urológico. Salienta-se que, no diagnóstico diferencial de adenopatias cervicais, deve-se considerar a neoplasia da próstata em pessoas do sexo masculino. (AU)

Cervical lymph nodes involvement is rare in prostate cancer and uncommon as an initial manifestation. This study is a clinical case report of a 72-year-old man who presented with a left cervical painless mass of 2-month progression, and bone pain on the lumbar and thoracic regions. Fine-needle aspiration cytology with immunohistochemistry staining was performed and revealed positivity for prostate-specific antigen consistent with prostate adenocarcinoma metastasis. Blood tests revealed a prostate-specific antigen of more than 1,000ng/mL, as well as high alkaline phosphatase. Whole-body bone scan showed secondary bone involvement. Following diagnosis, the patient started hormonal therapy and refused palliative radiotherapy. Eight months after diagnosis, recurrence was observed, with prostate-specific antigen elevation again. Thus, a clinical case of prostate cancer with bone and cervical lymph node metastasis in a patient with no urologic symptoms is reported. It should be noted that prostate cancer shall always be considered in the differential diagnosis of cervical lymphadenopathies in male patients. (AU)

Utilidad diagnóstica de la biopsia de médula ósea en pediatría / Diagnostic utility of biopsy of bone marrow in Pediatry

La biopsia de médula ósea (BMO) es un procedimiento invasivo que ha ganado campo en la práctica médica ya que se realiza para el diagnóstico, estadificación y seguimiento de enfermedades hematológicas y no hematológicas, benignas o neoplásicas, entre otros. El objetivo fue establecer el rol de la BMO en las hemopatías en Pediatría en el ION SOLCA Guayaquil- Ecuador. Se utilizó un estudio descriptivo retrospectivo donde se incluyeron a todos los pacientes pediátricos menores de 18 años de edad que se sometieron a BMO, desde Julio de 2014 a Julio de 2017 en el hospital. De las 1511 BMO realizadas en el periodo de estudio, 869 correspondieron a biopsias pediátricas, de las cuales el 57,08% fueron varones. La edad mediana fue 5 (RIC: 3-10) años. El tamaño promedio de la BMO fue de 0,74 (0,1-2,5) cm, con una celularidad media de 20% (4-100%). El motivo de consulta más frecuente fue la fiebre (22,67%). En el hemograma se detectó más frecuentemente bicitopenia (44,65%) y pancitopenia (24,63%). La Leucemia Linfoblástica Aguda (LLA) fue la enfermedad hematológica maligna más comúnmente encontrada (19,59%). Solo un 0,12% correspondió al grupo de Síndromes Mielodisplásicos (SMD), mientras que un 0,23% fueron Neoplasias Mieloproliferativas (NMP). El 26,93% de las biopsias no fueron aptas para el diagnóstico, el 48,45% se encontraron libres de enfermedad de base. La enfermedad oncohematológica pediátrica más frecuente es la LLA, mientras que los SMD y las NMP son infrecuentes. El rol del patólogo y de la BMO es fundamental en el diagnóstico de las enfermedades hematológicas, siempre en integración con la clínica y los exámenes complementarios.

Bone marrow biopsy (BMB) is an invasive procedure that has gained ground in medical practice since it is performed for the diagnosis, staging and monitoring of hematological and non-hematological, benign or neoplastic diseases, among others. This work aims to establish the role of the BMB in hematological diseases in Pediatrics in the ION SOLCA Guayaquil ­ Ecuador. A non-experimental design study, descriptive type was used, that included all pediatric patients under 18 years of age who submitted a BMB, from July 2014 to July 2017 in the hospital. Of the 1511 BMB performed in the study period, 869 corresponded to pediatric biopsies, of which 57.08% belong to male patients. The median age was 5 (interquartile range: 3 - 10) years. The average size of the BMB was 0.74 (0.1 - 2.5) cm, with an average cellularity of 20% (4 - 100%). The most frequent reason for consultation was fever (22.67%). In the complete blood count, bicytopenia (44.65%) and pancytopenia (24.63%) were detected most commonly. Acute Lymphoblastic Leukemia (ALL) was the most frequent malignant hematologic disease (19.59%). Only 0.12% corresponded to the group of Myelodysplastic Syndromes (MDS), while 0.23% were Myeloproliferative Neoplasms (MPN). 26.93% of the biopsies were not apt for diagnosis, 48.45% were free of base disease. The most cfrequent pediatric onco-hematologic disease is ALL, while MDS and MPN are infrequent. The role of the pathologist and the BMP is fundamental in the diagnosis of hematological diseases, always in integration with the clinic and complementary examinations.

Diagnóstico diferencial entre tumores oseos y osteomielitis en niños. A propósito de un caso / Differential diagnosis between bone tumors and osteomyelitis in children. About a case

La osteomielitis aguda y el tumor de Ewing, dos entidades óseas tan diferentes, una de naturaleza infecciosa como la osteomielitis, y otra de naturaleza neoplásica como el tumor de Ewing, pueden coincidir en sus características clínico-radiográficas y ser motivo, por ello, de confusión. No son infrecuentes, de hecho, las referencias a diagnósticos erróneos en este sentido.

Acute osteomyelitis and Ewing's tumor, two bone entities as different, one of an infectious nature such as osteomyelitis, and another of a neoplastic nature such as Ewing's tumor, may coincide in their clinical-radiographic characteristics and, therefore, cause confusion. In fact, references to erroneous diagnoses in this regard are not uncommon.

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.

Tumores y seudotumores primarios de la mano en adultos. Análisis epidemiológico de casos, manejo y evolución / Primary tumours and pseudotumors of the hand in adults. epidemiological analysis of cases, management and evolution

Resumen: Introducción: Los tumores en la mano son poco frecuentes y los tumores malignos son particularmente raros; sin embargo, las lesiones seudotumorales primarias de la mano, como gangliones, son muy frecuentes. La mayoría de los tumores que se encontraron en el hueso y tejido blando pueden presentarse de igual manera en la mano, pero su frecuencia y distribución es distinta. A pesar de la baja incidencia de los tumores benignos y malignos en la mano, éstos pueden ser localmente invasivos o comprometer su función. El objetivo principal de este estudio es determinar la epidemiología, tipos, localización y manejo de los tumores y seudotumores de la mano en adultos en población mexicana. Material y métodos: Se diseñó un estudio retrospectivo mediante revisión de expedientes clínicos de pacientes de nuestra institución con diagnóstico de tumores o seudotumores en la mano de Enero de 2007 a Diciembre de 2016 evaluando variables como edad, género, lateralidad, abordaje diagnóstico, estirpe histológica, localización y tratamiento. Resultados: Nuestro estudio comprende 215 pacientes con una edad promedio de 44.23 años, predominantemente mujeres. Los seudotumores son los que con más frecuencia se detectan (quiste sinovial), seguidos de los tumores benignos de tejido blando, tumores óseos benignos y, por último, los tumores malignos. Discusión: Después de todo, sólo sugerimos una clasificación para la mano que es sencilla y reproducible y dar orientación para el tratamiento. La necesidad de un sistema de clasificación es inminente para estandarizar los diagnósticos, guiar futuras investigaciones, enfocar tratamientos y preservar la función de la mano, independientemente de la epidemiología que se observe.

Abstract: Introduction: Tumors in the hand are rare, and malignant tumors in the hand are peculiar; However, primary pseudotumoral lesions of the hand, such as ganglion, are very frequent. Most of the tumors found in the bone and soft tissue can be presented in the same way in the hand but their frequency and distribution is different. Despite the low incidence of benign and malignant tumors in the hand, these can be locally invasive or compromise the hand function. The main objective of this study is to determine the epidemiology, types, location and management of tumors and pseudotumors of the hand in adults in the Mexican population. Material and methods: We designed a retrospective study by review of clinical records of patients in our institution with diagnosis of tumors or pseudotumors in hand from January 2007 to December 2016 evaluating variables such as age, gender, laterality, Diagnosis, histological lineage, localization and treatment. Results: Our study comprises 215 patients with an average age of 44.23 years, predominantly women. Pseudotumors are the most frequently encountered (synovial cyst), followed by benign soft tissue tumors, benign bone tumors, and, finally, malignant tumors. Discussion: After all we suggests a possible new classification for the hand that is simple, reproducible and give guidance for treatment. The need for a classification system is imminent to standardize diagnostics, guide future research, focus treatments and preserve hand function.

Osteosarcoma parostal desdiferenciado en diáfisis de cúbito metastásico a pulmón / Dedifferentiated diaphyseal juxtacortical osteosarcoma of ulna with metastasis to the lung

Resumen: El osteosarcoma parostal desdiferenciado es una variante en la cual un osteosarcoma de alto grado coexiste con un osteosarcoma parostal. Se presenta el caso de un paciente femenino de 20 años; acudió con seis meses de evolución de dolor y limitación funcional en el antebrazo derecho sin causa aparente; se le realizaron radiografías, donde se observó una lesión tumoral en la diáfisis del cúbito derecho. A la exploración física, presentó dolor a la palpación en la diáfisis del cúbito y limitación a la pronosupinación. La tomografía axial computada de tórax reveló enfermedad metastásica en el lóbulo superior del pulmón izquierdo. Se le efectuó una biopsia incisional de la tumoración en el cúbito derecho, con reporte de osteosarcoma parostal desdiferenciado. Por ello, inició manejo con quimioterapia neoadyuvante con cisplatino y doxorrubicina hasta completar tres ciclos. El tratamiento quirúrgico consistió en resección intercalar de la diáfisis del cúbito derecho más reconstrucción de injerto autólogo microvascularizado de la diáfisis del peroné derecho y estabilización del injerto con placa de compresión dinámica (DCP) 3.5 mm y placa tercio de caña. En el mismo procedimiento se le efectuó metastasectomía pulmonar por toracoscopía. El reporte histopatológico postquirúrgico, con necrosis de 100%. Actualmente, la paciente se encuentra asintomática, sin datos de actividad tumoral. El osteosarcoma parostal desdiferenciado es una patología rara, pero que se debe sospechar como diagnóstico diferencial ante un osteosarcoma parostal; se debe tener en cuenta que esta enfermedad puede generar metástasis por su patrón desdiferenciado. Es importante planificar un tratamiento quirúrgico que permita una adecuada reconstrucción funcional, siempre teniendo en cuenta el principio oncológico.

Abstract: Dedifferentiated parosteal osteosarcoma is a variant in which a high grade osteosarcoma coexists with a parosteal osteosarcoma. We report the case of a 20-year-old female patient who presented with six months of evolution of pain and functional limitation of the right forearm, with no apparent cause; radiographs were performed, observing a tumoral lesion in the diaphysis of the right ulna. Physical examination showed pain upon palpation in the diaphysis of the ulna and limitation of prone-supination. Axial computed tomography of the thorax revealed metastatic disease in the upper lobe of the left lung. An incisional biopsy was performed on the right ulna, with a report of dedifferentiated parosteal osteosarcoma. Therefore, the patient was managed with neoadjuvant chemotherapy with cisplatin and doxorubicin until completing three cycles. Surgical treatment consisted of intercalary resection of the diaphysis of the right ulna, plus reconstruction of the microvascularized autologous graft of the right fibular diaphysis and graft stabilization with 3.5 mm dynamic compression plate (DCP) and one-third tubular plate. In the same procedure, pulmonary metastasectomy was performed by thoracoscopy. Post-surgical histopathological report with 100% necrosis. Currently, the patient is asymptomatic, with no evidence of tumor activity. Dedifferentiated parosteal osteosarcoma is a rare pathology, but should be suspected as a differential diagnosis in the presence of a parosteal osteosarcoma; it should be taken into account that this disease can metastasize due to its dedifferentiated pattern. It is important to plan a surgical treatment that allows an adequate functional reconstruction, always taking into account the oncological principle.

Phosphaturic mesenchymal tumor (PMT): exceptionally rare disease, yet crucial not to miss

Phosphaturic mesenchymal tumors (PMTs) are very rare tumors which are frequently associated with Tumor Induced Osteomalacia (TIO), a paraneoplastic syndrome that manifests as renal phosphate wasting. The tumor cells produce a peptide hormone-like substance known as fibroblast growth factor 23 (FGF23), a physiologic regulator of phosphate levels. FGF23 decreases proximal tubule reabsorption of phosphates and inhibits 1-α-hydroxylase, which reduces levels of 1-α, 25-dihydroxyvitamine D3. Thus, overexpression of FGF23 by the tumor cells leads to increased excretion of phosphate in the urine, mobilization of calcium and phosphate from bones, and the reduction of osteoblastic activity, ultimately resulting in widespread osteomalacia. Patients typically present with gradual muscular weakness and diffuse bone pain from pathologic fractures. The diagnosis is often delayed due to the non-specific nature of the symptoms and lack of clinical suspicion. While serum phosphorus and FGF23 testing can assist in making a clinical diagnosis of PMT, the responsible tumor is often difficult to locate. The pathologic diagnosis is often missed due to the rarity of PMTs and histologic overlap with other mesenchymal neoplasms. While patients can experience severe disabilities without treatment, excision is typically curative and results in a dramatic reversal of symptoms. Histologically, PMT has a variable appearance and can resemble other low grade mesenchymal tumors. Even though very few cases of PMT have been reported in the world literature, it is very important to consider this diagnosis in all patients with hypophosphatemic osteomalacia. Here we present a patient who suffered for almost 5 years without a diagnosis. Ultimately, the PMT was located on a 68Ga-DOTA TATE PET/CT scan and subsequently confirmed by histologic and immunohistologic study. Interestingly, strong positivity for FGFR1 by IHC might be related to the recently described FN1-FGFR1 fusion. Upon surgical removal, the patient's phosphate and FGF23 levels returned to normal and the patient's symptoms resolved.

Fibroma osificante periférico de establecimiento precoz: reporte de un caso / Early onset peripheral ossifying fibroma: Presentation of a case

RESUMEN: El fibroma osificante periférico (FOP) es una hipertrofia benigna de los tejidos. Se localiza en la región de la papila interdental, proveniente de células que derivan del ligamento periodontal. Generalmente mide menos de 1,5cm de diámetro, siendo muy raro en recién nacidos. El objetivo de la presentación de este caso clínico es describir un caso de FOP en un bebé de 6 meses de edad. La niña presentaba una lesión nodular localizada en la mucosa gingival, en el área de incisivos inferiores, de aproximadamente 1,5cm diámetro, de consistencia firme, lisa, y coloración igual a la de la mucosa subyacente. Un diente neonatal había sido removido a los 2 meses de nacida. El FOP es una lesión poco común y que raramente puede encontrarse en bebés menores de un año de edad; por este motivo es importante realizar el diagnóstico adecuado, para evitar cualquier complicación debido a su establecimiento precoz y rápido crecimiento.

ABSTRACT: The peripheral ossifying fibroma (POF) is a rare, reactive and inflammatory gingival overgrowth, arising on the interdental papilla, from cells derived of the periodontal ligament. Usually its diameter is less than 1.5 cm. It's uncommon in newborns, frequently occurring in teenagers and young adult. The purpose of this report is to describe a case of FOP in a six months old baby. She had a nodular lesion, located on the gingival mucosa of the lower incisor area, of approximately 1.5 cm; it was firm, smooth, pedunculated, with an equal color of the underlying mucosa. A neonatal tooth had been removed four months back. Although FOP is a rare injury, it can be found in infants with less than one year of age, which is why it is important to make the right diagnosis, and an opportune treatment, to avoid further complications due to its early establishment and rapid growth.

Osteocondromas: presentación del osteocondroma subungueal / Osteochondromas: introducing the subungual osteochondroma

Resumen: Introducción: Los osteocondromas subungueales son tumoraciones benignas de la región sub- o periungueal que ocasionan elevación, ulceración y deformidad a dicho nivel. Objetivos: Investigar la incidencia de osteocondroma subungueal en un segmento de población pediátrica. Material y métodos: Estudio retrospectivo, transversal, descriptivo y observacional, basado en la revisión de expedientes con resultado histopatológico de osteocondroma de 2001 a 2014. Resultados: Cuatro de los osteocondromas correspondieron a la presentación subungueal, con una distribución por sexos de 1:1 y un promedio de edad de 9.5 años, relación 3:1, derecho:izquierdo; el cuarto dedo fue el más afectado. Discusión: El osteocondroma sunbungueal presenta una incidencia de 8.5% de todos los osteocondromas.

Abstract: Introduction: Subungual osteochondromas are benign tumors of the sub- or periungual region, causing lifting, ulceration and deformity at that level. Objective: To research the incidence of subungual osteochondromas in a specific pediatric population. Material and methods: Retrospective, transversal, descriptive and observational study based on the review of records with a histopathological result of osteochondroma from 2001 to 2014. Results: The pathological assessment featured four osteochondromas that corresponded to the subungual presentation, with an equal gender distribution of 1:1, an average age of 9.5 years, right: left ratio of 3:1; the 4th finger was the most affected. Discussion: Subungual osteochondroma has an incidence of 8.5% of all osteochondromas.

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.

Avaliação da influência do tempo de diagnóstico no tratamento de pacientes com condrossarcoma / Evaluation of influence of the diagnostic time on the treatment of patients with chondrosarcoma

O condrossarcoma é um tumor ósseo maligno proveniente de cartilagem hialina. É o segundo tumor primário do osso mais frequente com 20% dos casos, sendo mais frequente em homens do que em mulheres entre a terceira e sexta década de vida. O diagnóstico é feito através de radiografia, tomografia e biópsia, onde no exame histopatológico é evidente o aumento de celularidade, núcleos aumentados e células binucleadas. O tumor é classificado em graus 1 (G1), 2 (G2), 3 (G3), desdiferenciado, mesenquimal e de células claras. O tratamento é feito através de cirurgia, pois a maioria dos condrossarcomas não responde bem a quimioterapia e radioterapia. O objetivo deste estudo foi realizar uma avaliação epidemiológica retrospectiva dos pacientes com condrossarcoma tratados no INTO e identificar se o tempo de início dos sintomas até o diagnóstico tem influência na escolha do método cirúrgico para tratamento desses pacientes. Foram avaliados 65 pacientes de ambos os sexos, com a faixa etária variando entre 6 e 86 anos e com idade média de 50 anos. Identificamos 47 (72,3%) lesões acometendo o esqueleto apendicular e 18 (27,7%) o esqueleto axial. O tempo de diagnóstico destes pacientes variou de 1 a 108 meses, como uma média de 20,5 meses. Nós observamos que o tempo de diagnóstico não influenciou no tipo cirúrgico escolhido Entretanto, verificamos que o maior impacto sobre a escolha do tipo de tratamento cirúrgico foi do estadiamento (P<0,001), com consequência na taxa de cirurgia radical especialmente nos pacientes com condrossarcoma com estadiamento IIB e III. O conjunto destes dados nos permite concluir que o tempo prolongando de diagnóstico não influencia no tipo de tratamento cirúrgico adotado e nem na incidência de metástase e mortalidade dos pacientes avaliados em nosso estudo

Chondrosarcoma is a malignant bone tumor, originating from hyaline cartilage being the second most frequent primary bone tumor with 20% of the cases, is more frequent in men than in women between the third and sixth decade of life. The diagnosis is made by radiography, tomography and biopsy, where the histopathological examination evidences the increase of cellularity, enlarged nuclei and binucleate cells, the chondrosarcomas are divided into G1, G2, G3, dedifferentiated, mesenchymal and clear cells. Treatment has done through surgery, as most chondrosarcomas do not respond well to chemotherapy and radiation therapy. The objective of this study was to conduct a retrospective epidemiological evaluation of patients with chondrosarcoma treated at INTO and to identify whether the time from onset of symptoms to diagnosis delay has influence on the choice of surgical method performed for treatment of these patients. We evaluated 65 patients of both gender, ranging in age from 6 to 86 years, with a mean age of 50 years. We identified 47 (72.3%) lesions affecting the appendicular skeleton and 18 (27.7%) the axial skeleton. The time of diagnosis of these patients ranged from 1 to 108 months, as an average of 20.5 months. We observed that the time of diagnosis did not influence the surgical type performed (P <0.92). However, it was verified that the major impact on the type of surgical treatment was patient staging (P <0.001), with a Radical surgery especially in patients stage IIB and III. All these data allow us to conclusion that the prolonged time of diagnosis does not influence the type of surgical treatment chosen and in incidence of metastasis and mortality of the patients evaluated in our study

Osteocondroma escapular: un caso infrecuente / Scapular osteochondroma: an infrequent case

El osteocondroma de la escápula es un tumor beningno muy raro por su ubicación, que produce dolor y disfunción mecánica de la articulación cuando se establece en la superficie ventral de la escápula, afecta principalmente a los varones en los períodos del crecimiento óseo, entre 10 y 30 años de edad, representan el 14,4 % de todos los tumores de la escápula y el 49 % de los tumores benignos de la escápula. Surgen en las regiones metafisarias de los huesos largos (fémur, húmero, tibia), causando irritación mecánica que conduce a impotencia funcional, bursitis, e incluso fractura de la escápula, pocos casos en huesos planos han sido descritos. El diagnostico se realiza con la valoración física, clínica y exámenes complementarios, debiendo ser confirmado por el histopatológo previa biopsia. La indicación quirúrgica incluye la disfunción mecánica de una articulación o tendón en relación con el tamaño de la lesión, y del cambio sarcomatoso siendo el procedimiento de elección la escisión quirúrgica abierta. A continuación se describe el caso de una joven de 11 años a quien se diagnosticó un osteocondroma subescapular, previabiopsia escisional.

Osteochondroma of the scapulais a rare benign tumor in view of its location, which produces pain and mechanical dysfunction of the joint when it is on the ventral surface of the scapula, mainly affecting men during bone growth periods, betweenthe ages of 10 and 30, accounting for 14.4 % of allscapula tumors and 49 % of benign tumors of thescapula. They arise in the metaphyseal regions ofthe long bones (femur, humerus, tibia), causing mechanical irritation leading to functional impotence,bursitis, and fracture of the scapula. Few cases inflat bones have been described. Diagnosis is made with physical, clinical and complementary examinations, and must be confirmed by prior histopathology biopsy. The surgical indication includes mechanical dysfunction of a joint or tendon in relationto the size of the lesion, sarcomatous change being the procedure of choice in open surgical excision. The case of an 11-year-old girl who was diagnosed with a subscapular osteochondroma after previous excisional biopsy, is described below.

Sarcoma de Ewing extraóseo en espacio epidural / Extraosseous Ewing's sarcoma in spinal epidural space

El sarcoma extraóseo de Ewing pertenece a la familia de tumores neuroectodérmicos que derivan de la cresta neural, característico por su agresividad. Varias localizaciones se han descrito, sin embargo, la presentación epidural es rara. El pronóstico ha mejorado por nuevos esquemas oncológicos y están en estudio otras dianas terapéuticas.

Extraosseous Ewing's sarcoma belongs to the family of neuroectodermal tumors that derive from the neural crest, characteristic of its aggressiveness. Several locations have been described, however,epidural presentation is rare. Prognosis has been improved by new oncological regimens and other therapeutic targets are being studied.

Intraosseous lipoma of the iliac: case report / Lipoma intraósseo do ilíaco: relato de caso

Lipomas are benign tumors that attack fat cells and most often affecting soft tissues in adulthood. On rare occasions, they may affect bones, preferentially the metaphyses of the long bone. They are generally asymptomatic and radiography shows radiolucent lesions with a thin sclerotic rim or radiodense lesions with a thick sclerotic rim. Malignant transformation of these tumors is rare, as is their recurrence, and there is no need for surgery in most cases. In this report, we present a rare case of intraosseous lipoma in the iliac bone.

Os lipomas são tumores benignos que acometem células adiposas, mais comumente afetam os tecidos moles na idade adulta. Raramente podem afetar os ossos, preferencialmente metáfises dos ossos longos. São geralmente assintomáticos, na radiografia verifica-se lesão radiotransparente, com uma fina borda esclerótica ou lesão radiodensa com uma espessa borda esclerótica. A transformação maligna do tumor é rara, assim como a recorrência, sem necessidade cirúrgica na maioria dos casos. Neste relato apresentamos um caso raro de lipoma intraósseo do ilíaco.

Quiste óseo aneurismático sólido del raquis dorsal / Solid aneurysmal bone cyst of dorsal spine

El quiste óseo aneurismático sólido es un tumor de naturaleza neoplásica indefinida, de comportamiento benigno, crecimiento rápido y agresivo. Representa menos del 8 % de todos los quistes óseos aneurismáticos. Se presenta un caso de quiste óseo aneurismático sólido de mediastino posterior con toma espinal torácica. La ausencia de signos característicos en las pruebas de imagen y la presencia de rasgos similares al tumor de células gigantes y el osteosarcoma, hacen imprescindible la confirmación anatomopatológica para un tratamiento adecuado de la enfermedad. La exéresis quirúrgica total de la tumoración, el aporte de injerto óseo y la fijación del segmento afecto constituyen el procedimiento de elección.

The solid aneurysmal bone cyst is a malignant tumor of undefined nature, benign behavior; it has fast and aggressive growth. It represents less than 8 % of all aneurysmal bone cysts. A case of posterior mediastinum solid aneurysmal bone cyst with thoracic spinal making is presented here. The absence of characteristic signs in the imaging and the presence of similar features of giant cell tumor and osteosarcoma, make it imperative the pathologic confirmation for proper treatment of the disease. Total surgical excision of the tumor, the bone grafting and fixation segment affection are the procedures of choice.

Le kyste osseux anévrismal solide est une tumeur de nature néoplasique indéfinie, caractérisée par un comportement bénin et une croissance rapide et agressive. Il représente moins de 8 % de tous les kystes osseux anévrismaux. Un cas de kyste osseux anévrismal solide au niveau de médiastin postérieur (d’atteinte spinale thoracique) est présenté. L’absence de signes caractéristiques dans l’imagerie RM et la présence de traits similaires à la tumeur à cellules géantes et à l’ostéosarcome rendent nécessaire la confirmation anatomopathologique pour un traitement approprié de cette pathologie. L’exérèse totale de la tumeur, la greffe osseuse et la fixation du segment affecté constituent la technique opératoire de choix.

Tumor fibromixoma lipoesclerosante óseo: Presentación de un caso y revisión de la literatura / Bone liposclerosing myxofibrous tumor: Case presentation and literature review

El tumor fibromixoma lipoesclerosante óseo (TFMLE) fue descrito inicialmente por Ragsdale en 1986, como una lesión fibroósea polimórfica de hueso con una mezcla de elementos histológicos incluyendo lipoma, fibroxantoma, mixoma, mixofibroma, necrosis grasa, osificación isquémica, áreas de displasia fibrosa y rara vez cartílago o cambios quísticos. La localización más frecuentemente descrita es en la región intertrocantérica del fémur. Es un hallazgo en pacientes asintomáticos o bien estar asociado a fractura. Radiológicamente es una lesión lítica, geográfica, de margen bien definido y habitualmente esclerótico, en algunos casos puede observarse mineralización en el interior de la lesión o un cierto grado de expansión en el contorno. Se ha descrito la estrecha relación del TFMLE con la displasia fibrosa por las características histológicas y la presencia de una mutación Gsα, otra hipótesis de la etiología de la lesión incluye la reacción de la displasia fibrosa a la fatiga por estrés.

The bone liposclerosing myxofibrous tumor (LSMFT) was initially described by Ragsdale in 1986 as a polymorphic fibroosseous bone lesion with a mix of histologic elements that include lipoma, fibroxanthoma, myxoma, myxofibroma, fat necrosis, ischemic ossification, areas of fibrous dysplasia, and infrequent presence of cartilage or cystic changes. The most frequently reported location is the intertrochanteric area of the femur. Radiologically it is a lytic, geographic lesion, with well-defined margins and usually sclerotic. In some cases findings include mineralization inside the lesion or a certain degree of expansion to the contour. The close relationship between LSMFT and fibrous dysplasia has been described based on the histologic characteristics and the presence of the Gsα mutation. Another hypothesis of the etiology of the lesion is the reaction of fibrous dysplasia to stress.

Solitary Osseous Metastasis of Rectal Carcinoma Masquerading as Osteogenic Sarcoma on Post-Chemotherapy Imaging: A Case Report

Solitary metastases from colorectal carcinoma in the absence of hepatic or pulmonary metastases are rare. These can have a diverse imaging appearance, particularly after chemotherapy. It is important identify patients with solitary skeletal metastases, as they have a better prognosis than those with multiple skeletal or visceral metastases. We describe an unusual case of a solitary metastasis to the femur in a case of colon carcinoma that went undiagnosed and later presented with imaging features of osteogenic sarcoma.

Hepatocellular Carcinoma with Cervical Spine and Pelvic Bone Metastases Presenting as Unknown Primary Neoplasm / 대한소화기학회지

The occurrence of hepatocellular carcinoma (HCC) is closely associated with viral hepatitis or alcoholic hepatitis. Although active surveillance is ongoing in Korea, advanced or metastatic HCC is found at initial presentation in many patients. Metastatic HCC presents with a hypervascular intrahepatic tumor and extrahepatic lesions such as lung or lymph node metastases. Cases of HCC presenting as carcinoma of unknown primary have been rarely reported. The authors experienced a case of metastatic HCC in a patient who presented with a metastatic bone lesion but no primary intrahepatic tumor. This case suggests that HCC should be considered as a differential diagnosis when evaluating the primary origin of metastatic carcinoma.