Displasia fibrosa maxilar. Del diagnóstico al tratamiento. Reporte de caso / Maxillary fibrous dysplasia. From diagnosis to treatment: Case report / Displasia fibrosa maxilar. Do diagnóstico ao tratamento: Relato de caso

La displasia fibrosa se puede presentar, en cualquier persona, por una mutación en los primeros meses de gestación. Se trata de lesiones óseas benignas en pacientes jóvenes, en las que el tejido óseo normal es sustituido por tejido conectivo fibroso, debido a una alteración funcional de las células, siendo esta la antesala de una neoplasia. El objetivo de éste artículo es presentar un caso local de Displasia Fibrosa Maxilar, enfatizando el manejo clínico, radiográfico, pre y postoperatorio más pertinente. Por lo tanto, se describe el caso de un paciente adolescente de género masculino, afectado por displasia fibrosa en hemimaxilar derecho, a quien le fueron realizados los estudios imagenológicos e histopatológicos necesarios para obtener el diagnóstico definitivo y decidir el abordaje menos invasivo posible cumpliendo con estándares de estética facial; se realizó abordaje intrabucal para shaving óseo, con sedación consciente bajo estricta vigilancia de la especialista en anestesiología y reanimación. Una vez logrado el contorno deseado mediante el uso de piezas de mano de alta y baja velocidad, se realizó la sutura de los tejidos y el paciente egresó ambulante y con buen estado general de salud.

Fibrous dysplasia can occur in any person due to a mutation in the first months of gestation. These are benign bone lesions in young patients, in which the normal bone tissue is replaced by fibrous connective tissue, due to a functional alteration of the cells, being this the prelude to a neoplasm. The aim of this article is to present a local case of Maxillary Fibrous Dysplasia, emphasizing the most relevant clinical, radiographic, pre and postoperative management. Therefore, we describe the case of an adolescent male patient, affected by fibrous dysplasia in the right hemimaxillary, who underwent the necessary imaging and histopathological studies to obtain the definitive diagnosis and decide the least invasive approach possible in compliance with facial aesthetic standards; an intraoral approach was performed for bone shaving, with conscious sedation under strict supervision of the specialist in anesthesiology and resuscitation. Once the desired contour was achieved through the use of high and low speed handpieces, the tissues were sutured and the patient was discharged ambulatory and in good general health.

A displasia fibrosa pode ocorrer em qualquer pessoa devido a uma mutação nos primeiros meses de gestação. São lesões ósseas benignas em pacientes jovens, nas quais o tecido ósseo normal é substituído por tecido conjuntivo fibroso, devido a uma alteração funcional das células, sendo esse o prelúdio de uma neoplasia. O objetivo deste artigo é apresentar um caso local de Displasia Fibrosa Maxilar, enfatizando o manejo clínico, radiográfico, pré e pós-operatório mais pertinente. Portanto, descrevemos o caso de um paciente adolescente do sexo masculino, afetado por displasia fibrosa no hemimaxilar direito, que foi submetido aos exames de imagem e histopatológicos necessários para obter um diagnóstico definitivo e decidir sobre a abordagem menos invasiva possível, em conformidade com os padrões estéticos faciais; foi realizada uma abordagem intraoral para raspagem óssea, com sedação consciente sob estrita supervisão do especialista em anestesiologia e ressuscitação. Depois que o contorno desejado foi obtido com o uso de peças de mão de alta e baixa velocidade, os tecidos foram suturados e o paciente recebeu alta ambulatorial em bom estado geral de saúde.

Solitary fibrous tumor: Two cases report with a focus on the differential diagnosis / Tumor Fibroso Solitário: Relato de dois casos com foco no diagnóstico diferencial

Solitary fibrous tumor (SFT) is a neoplasm of fibroblastic/myofibroblastic origin with intermediate biological behavior. We report here two cases of SFT affecting an unusual anatomical site in 58-year-old and 40-year-old female patients and discuss the differential diagnosis of this lesion. In case 01, the lesion showed the clinical appearance of an asymptomatic "blister" with normal color, rubbery consistency, measuring 0.3 cm, and affected the lower lip; while in case 02, a symptomatic red nodular lesion with a soft consistency and measuring 0.5 cm affected the floor of the mouth. Excisional biopsies were performed. Microscopically, two well-delimited benign neoplasms were observed, exhibiting the proliferation of ovoid to spindle-shaped mesenchymal cells, vascular spaces with staghorn arrangement, and the absence of mitosis figures. Immunohistochemistry was performed in case 01 to assist in the diagnosis. Weak and diffuse immunostaining was observed for α-SMA and intense and diffuse immunopositivity for Bcl-2 and CD34. Based on histopathological and immunohistochemical features, a diagnosis of SFT was rendered in both cases. The low occurrence and nonspecific clinical features of oral SFT may make its clinical diagnosis difficult. Also, morphological and immunohistochemical are essential for differential diagnosis with other mesenchymal neoplasms.(AU)

O tumor fibroso solitário (TFS) é uma neoplasia de origem fibroblástica/miofibroblastica com comportamento biológico intermediário. Nesse artigo relatamos dois casos de TFS afetando sítios anatômicos incomuns em pacientes do sexo feminino de 58 anos e 40 anos e discutir os seus diagnósticos. No caso 01 clinicamente a lesão apresentou um aspecto de "bolha" assintomática, coloração normal da mucosa, consistência borrachoide medindo 0,3 cm, em região de lábio inferior, enquanto que no caso 02, como uma lesão sintomática, vermelha, nodular com consistência mole e medindo 0,5 cm afetando o assoalho bucal. As biópsias excecionais foram realizadas. Microscopicamente, observamos duas lesões neoplásicas bem delimitadas exibindo uma proliferação de células mesenquimais variando de ovoides a fusiformes, vasos sanguíneos em formato de "chifre de veado", com ausência de figuras de mitoses. No caso 01 foi realizado análise imuno-histoquímica para auxiliar no diagnóstico. Foi observado uma marcação fraca e difusa de α-SMA e uma intensa e difusa imunopositividade para o Bcl-2 e CD34.Baseado nos achados histopatológicos e imuno-histoquímicos o diagnóstico de TFS foi estabelecido em ambos os casos. A baixa ocorrência e os achados clínicos inespecíficos do TFS oral podem dificultar o diagnóstico clínico. Além disso, as análises morfológicas e imuno-histoquimicas são essenciais para realização do diagnóstico diferencial com outras neoplasias mesenquimais.(AU)_

Abordagem orbital para excisão de tumor fibroso solitário no seio frontal relativo a um caso clínico / Orbital approach for solitary fibrous tumor in the frontal sinus excision: case report / Abordaje orbitario para escisión de un tumor fibroso solitario en seno frontal: a propósito de un caso clínico

Apresentamos um caso de tumor fibroso solitário do seio frontal que herniava para a cavidade orbitária. Esta patologia é rara e cerca de trinta casos foram relatados na literatura mundial. O manejo foi feito com um grupo multidisciplinar e uma abordagem combinada transnasal e endoscópica externa através de uma incisão no nível do ângulo interno superior da órbita. A grande maioria da massa tumoral foi retirada e a fístula óssea foi selada com a colocação de uma malha orbital, que foi recoberta por uma membrana autóloga de fatores de crescimento, obtida e processada do sangue do próprio paciente. Os resultados imediatos foram muito bons e o acompanhamento é necessário para o controle do tumor... (AU)

We present a case of a solitary fibrous tumor of the frontal sinus that herniated within the orbital cavity. This pathology is rare and around thirty cases have been reported in the world literature. Management was done with a multidisciplinary group and a combined transnasal endoscopic and external approach through an incision in superior internal angle of the orbit. The tumor mass was removed, and the supraorbital bone was sealed with an orbit mesh, which was covered with an autologous membrane of growth factors, obtained, and processed from the patient's own blood. The immediate result was excellent, and follow-up is required to control the tumor... (AU)

Presentamos un caso de un tumor fibroso solitario de seno frontal que se hernió dentro de la cavidad orbitaria. Esta patología es rara y se han reportado una treintena de casos en la literatura mundial. El manejo se hizo con un grupo multidisciplinario y un abordaje combinado endoscópico transnasal y externo a través de una incisión a nivel de ángulo superior interno de la órbita. Se retiró la masa tumoral en su gran mayoría, y se selló la fístula ósea con la colocación de una malla para órbita, la cual se cubrió con una membrana autóloga de factores de crecimiento, obtenida y procesada de la propia sangre del paciente. Los resultados inmediatos fueron muy buenos y se precisa seguimiento para control de la tumoración... (AU)

Tumor fibroso solitario (TFS) parafaríngeo: reporte de caso y revisión de literatura / Parapharyngeal solitary fibrous tumor (SFT): case report and literature review

El espacio parafaríngeo es una región donde puede surgir un amplio espectro de tumores, que son poco frecuentes y comprenden solo el 0,5 al 1,5% de las neoplasias de cabeza y cuello. Generalmente, son hallazgos y suelen presentarse como masas asintomáticas. Incluye neoplasias primarias, extensión directa de regiones adyacentes y enfermedad metastásica. La literatura describe múltiples patologías e histologías que pueden surgir de esta zona, entre ellas, tenemos los tumores fibrosos solitarios, que son un espectro de neoplasias mesenquimatosas fibroblásticas de histogénesis incierta, que generalmente involucran la pleura. Son lesiones muy poco frecuentes y su localización parafaríngea es excepcional, siendo su primer reporte en la literatura en 1993. Se presenta el caso de un tumor fibroso solitario del espacio parafaríngeo evaluado en nuestro centro, con su enfrentamiento diagnóstico, resolución quirúrgica y seguimiento respectivo, con el objetivo de presentar esta patología poco frecuente a nuestros colegas y contribuir al conocimiento médico.

The parapharyngeal space is a region where a wide spectrum of tumors can arise, which are rare and comprise only 0.5% to 1.5% of head and neck neoplasms. They are generally findings and usually present as asymptomatic masses. Includes primary neoplasms, direct extension from adjacent regions, and metastatic disease. The literature describes multiple pathologies and histologies that can arise from this area, among them, we have solitary fibrous tumors, which are a spectrum of fibroblastic mesenchymal neoplasms of uncertain histogenesis, which are generally the pleura. It is a very rare lesion and its parapharyngeal location is exceptional, being its first report in the literature in 1993. We present the case of a solitary fibrous tumor of the parapharyngeal space evaluated in our center, with its diagnos- tic confrontation, surgical resolution and respective follow-up, with the aim of presenting this rare pathology to our colleagues and contributing to medical knowledge.

Tumor fibroso solitario de próstata: informe de caso y revisión de la literatura / Solitary fibrous tumor of the prostate: case report and literature review

El tumor fibroso solitario (TFS) es una neoplasia mesenquimatosa de tipo fibroblástico que, a pesar de ser localizado principalmente en pleura, se ha observado en otros órganos como la próstata. Por su parte, el tumor fibroso solitario de la próstata es una neoplasia de baja incidencia, crecimiento lento y potencial maligno incierto, que generalmente se compone de células fusiformes de apariencia citológicamente benignas, dispuestas en una arquitectura desorganizada, mezcladas con colágeno y pequeños vasos sanguíneos. Establecer su diagnóstico se ha vuelto más reproducible desde la identificación de la fusión de los genes NAB2-STAT6 por biología molecular, que lleva a la sobreexpresión de STAT6 por inmunohistoquímica, el cual es un marcador muy sensible y específico para TFS. Presentamos el caso clínico de un paciente que debutó con síntomas de compresión vesical, en quien se identificó una masa con epicentro en la próstata que infiltraba la vejiga y llegaba a la pared rectal, y que luego de estudios de patología, inmunohistoquímica y pruebas moleculares se clasificó como un TFS de la próstata, finalmente tratado con cistoprostatectomía radical más derivación urinaria

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm of fibroblastic type, which despite being located mainly in the pleura, has been observed in other organs such as the prostate. On the other hand, solitary fibrous tumor of the prostate is a rare neoplasm, slow growing, and of uncertain malignant potential, which is generally composed of spindle cells of cytologically benign appearance, arranged in a disorganized architecture, mixed with collagen and small blood vessels. Establishing its diagnosis has become more reproducible since the identification of the NAB2-STAT6 gene fusion by molecular biology, leading to the overexpression of STAT6 by immunohistochemistry, a very sensitive and specific marker for SFT. We present a clinical report of a patient who consulted with symptoms of bladder compression, in whom a mass was identified with the epicenter in the prostate infiltrating into the bladder and reaching the rectal wall. Following histopathology study, immunohistochemistry and molecular tests it was classified as a SFT of the prostate, finally treated with radical cystoprostatectomy plus urinary shunt

Undifferentiated pleomorphic sarcoma: prognostic factors in 42 extremity cases / Sarcoma pleomórfico indiferenciado: fatores de prognóstico em 42 casos em extremidades

ABSTRACT Introduction: Soft tissue undifferentiated pleomorphic sarcoma (UPS) in extremities is considered a rare neoplasm, corresponding to 5% of soft tissue sarcomas (STS) today. The objective was to evaluate prognostic factors related to death, local recurrence (LR), and impact on survival rates. Methods: A retrospective study including 42 patients with UPS in extremities treated surgically in a single center. Comparisons were made between demographic data, characteristics of the neoplasia, and treatment. Between the variables with statistical significance, logistic regression analysis was used. Survival rates were evaluated using Kaplan-Meier plots. To compare the effect of variables on survival rates, the Log-Rank test was used. Results: Age group of patients was from 25 to 85 years (mean 58 years), with a mean follow-up of 29.6 months. The variables with the highest effect on survival rates were sizes larger than 15 cm (T4) with p = 0.01, presence of metastatic lesions, and prognostic stage IV according to the American Joint Committee of Cancer (AJCC) with p < 0.001. The mean survival was 25.9 months. Metastasis and stage IV of AJCC were associated with a reduction in patient survival (17.8 months) with Log-Rank test p < 0.001. Conclusion: The main factors of poor prognosis related to mortality and reduction of survival of UPS in extremities were metastatic lesions and stage IV of AJCC. Level of Evidence III, Retrospective Study.

RESUMO O sarcoma pleomórfico indiferenciado (SPI) de tecidos moles em extremidades, neoplasia rara, correspondente a 5% dos sarcomas de tecidos moles (STM). Objetivo: Avaliar fatores de prognóstico relacionados a óbito, recorrência local (RL) e impacto na sobrevida geral. Métodos: Estudo retrospectivo de 42 pacientes com SPI tratados cirurgicamente em centro único. Foram comparados dados demográficos, características da neoplasia e de tratamento; e para as variáveis com significância estatística, foi realizada análise de regressão logística. A sobrevida foi avaliada através de gráficos de Kaplan-Meier; e os efeito das variáveis sobre a sobrevida, por meio do teste de log-rank. Resultados: Os pacientes tinham idades entre 25 e 85 anos (média de 58 anos), com seguimento ambulatorial médio de 29,6 meses. As variáveis com maior relação com o desfecho óbito foram tamanho maior que 15 cm (T4), com p = 0,01, metástases e estágio IV de prognóstico da American Joint Committee on Cancer (AJCC), com p < 0,001. A sobrevida média dos pacientes foi de 25,9 meses. Presença de metástase e estágio IV da AJCC foram associados à redução na sobrevida dos pacientes (17,8 meses; p < 0,001). Conclusão: Os principais fatores de mau prognóstico relacionados ao óbito e à redução da sobrevida dos pacientes com SPI foram doença metastática e estágio IV da AJCC. Nível de Evidência III, Estudo Retrospectivo.

Histiocitoma fibroso maligno: estudio hisotpatologico e inmunohistoquímico / Malignant Fibrous Histiocytoma: Histopathological and Immunohistochemical Study

El histiocitoma fibroso maligno (MFH) es el tumor de tejido blando más común en adultos. Generalmente se considera que surge de las células mesenquimales primitivas que muestran diferenciación histiocítica y fibroblástica parcial. Las observaciones inmunohistoquímicas sugieren que la expresión de marcadores del músculo liso en el llamado MFH es el resultado de la diferenciación miofibroblástica. El presente estudio tiene como objetivo correlacionarse entre el subtipo histipatológico y los parámetros clínicos, calificar los casos de MFH dependiendo de los criterios histopatológicos para la clasificación, y examinar los casos inmunohistoquímicamente para la diferenciación miofibroblástica utilizando marcadores musculares lisos en casos de MFH como una ayuda para un diagnóstico preciso para un diagnóstico preciso. . Este estudio incluye 26 muestras de tejidos blandos diagnosticados como MFH recolectados de laboratorios histopatológicos privados y gubernamentales en Basrah durante el período de enero de 2000 a octubre de 2005. 4 casos adicionales (un leiomioma, dos fibromas y un fibrosarcoma se tomaron como control positivo y negativo. Los casos de MFH (77%) estaban en el grupo de edad de 45 a 60 años. La edad media fue de 53.5 años con una relación hombre / mujer de 1.3: 1. Diecinueve casos (73%) se ubicaron en las extremidades principalmente en las extremidades inferiores. Diecisiete años. Los casos (65.4%) fueron primarios. Veintidós (84.8%) eran de subtipo pleomórfico, dos eran mixoides y 2 eran inflamatorios. Todos los casos recurrentes se consideraban como el Grado III, de los diecisiete casos principales eran de grado III, por lo que veinte y veinte Tres casos (88.5%) fueron de grado III, los 3 casos restantes fueron de grado II. No se registró tumor de grado I. La mayoría de los casos de subtipo pleomórficos (95.5%) fueron de grado III. Ambos casos de subtipo mixoide fueron de grado Ii. Desmin expr Se encontró la en 3 casos (11.5%), ninguno para la actina del músculo liso o la proteína S-100. No hubo correlación entre la expresión de desmin y el sitio tumoral, subtipo o grado, así como, con la edad y el sexo de los pacientes. Se encontró una fuerte asociación entre la expresión de desmin y los tumores recurrentes del 33,3%

Malignant fibrous histiocytoma (MFH) is the most common soft tissue tumor in adult. It is generally regarded as arising from primitive mesenchymal cells that show partial histiocytic and fibroblastic differentiation. Immunohistochemical observations suggest that the expression of smooth muscle markers in the so called MFH is a result of myofibroblastic differentiation. The present study is aimed to correlate between histipathological subtype and clinical parameters, to grade the MFH cases depending on the histopathological criteria for grading, and to examine the cases immunohistochemically for myofibroblastic differentiation using smooth muscle markers in cases of MFH as an aid for accurate diagnosis. This study including 26 soft tissue specimens diagnosed as MFH collected from private and governmental histopathological laboratories in Basrah during the period from January 2000 to October 2005. Additional 4 cases (one leiomyoma, two fibromas and one fibrosarcoma were taken as control positive and negative. Twenty cases of MFH (77%) were in the age group 45-60 years. The mean age was 53.5 year with male to female ratio of 1.3: 1. Nineteen cases (73%) were located in the extremities mainly the lower limbs. Seventeen cases (65.4%) were primary. Twenty two (84.8%) were of pleomorphic subtype, two were myxoid and 2 were inflammatory. All the recurrent cases were regarded as grade III, from the seventeen primary cases fourteen were of grade III, so twenty three cases (88.5%) were of grade III, the remaining 3 cases were of grade II. No grade I tumor was recorded. The majority of pleomorphic subtype cases (95.5%) were of grade III. Both cases of myxoid subtype were of grade II. Desmin expression was found in only 3 cases (11.5%), none for smooth muscle actin or S-100 protein. There was no correlation between desmin expression and tumor site, subtype or grade, as well as, with age and sex of the patients. A strong association between desmin expression and recurrent tumors 33.3% was found.

Mixofibrosarcoma mandibular de bajo grado en un adulto / Low grade mandibular myxofibrosarcoma in an adult

Se describe el caso clínico de un paciente de 61 años de edad, con antecedente de buena salud, atendido en el St. Paul's Milennium Medical Hospital de Etiopía por presentar, desde hacía 2 años, una lesión en la encía marginal, con abombamiento de ambas corticales, que se extendía del primer premolar inferior derecho al izquierdo, por lo cual se decidió ingresarlo en el Servicio de Cirugía Maxilofacial para su estudio y tratamiento. Se trataba de un tumor poco frecuente y de gran tamaño, con amplia diseminación ganglionar hacia los niveles IV y V de ambas partes del cuello, de manera que se realizó el procedimiento quirúrgico. La evolución fue favorable en el posoperatorio inmediato y egresó de la institución 7 días después, pero continuó con radioterapia. Y seguimiento por consulta externa durante 24 meses después.

The clinical case of a 61-year-old patient, with a history of good health, treated at St. Paul's Milennium Medical Hospital in Ethiopia for presenting, for 2 years, a lesion in the marginal gum, with bulging of the gums, is described. both cortices, which extended from the lower right to the left first premolar, for which it was decided to admit him to the Maxillofacial Surgery Service for study and treatment. It was a rare and large tumor, with extensive lymph node dissemination to levels IV and V in both parts of the neck, so the surgical procedure was performed. The evolution was favorable in the immediate postoperative period and he was discharged from the institution 7 days later, but continued with radiotherapy. And follow-up by outpatient service for 24 months afterwards.

Tumor fibroso solitario gigante de pleura / Giant solitary fibrous tumor of the Pleura

Resumen Objetivo: Reportar el caso de una masa gigante en hemitórax izquierdo de 19 cm de diámetro en un paciente de 59 años que debutó con disnea, tos y dolor torácico, confirmándose por estudio imagenológico. Materiales y Método: Registro clínico de un paciente al cual se le diagnostica tumor fibroso solitario de pleura, siendo intervenido quirúrgicamente para exéresis de la lesión. Resultados: Se realiza toracotomía posterolateral izquierda para exéresis de tumor gigante, requiriendo además, resección de diafragma y pericardiectomía parcial con evolución favorable. Discusión: El tumor fibroso solitario es una neoplasia rara derivada del mesénquima que afecta más comúnmente a la pleura, típicamente bien circunscrita, pediculada, con vasos dentro del pedículo tumoral, pudiendo llegar a ser de gran tamaño, siendo considerados gigantes cuando tienen más de 15 cm de diámetro. Conclusión: El diagnóstico correcto es de vital importancia, ya que con la resección quirúrgica es potencialmente curable. El tratamiento quirúrgico puede efectuarse por toracotomía o videotoracoscopia, dependiendo del tamaño del tumor. A pesar del comportamiento benigno, requiere seguimiento a largo plazo debido a la tendencia a la recidiva.

Aim: To report the case of a 19 cm diameter giant mass in the left hemithorax in a 59-year-old patient who presented with dyspnea, cough and chest pain, confirmed by imaging study. Materials and Method: Clinical record of a patient who was diagnosed with a solitary fibrous tumor of the pleura, undergoing surgery to excise the lesion. Results: A left posterolateral thoracotomy was performed to excise the giant tumor, also requiring resection of the diaphragm and partial pericardiectomy with favorable evolution. Discussion: The solitary fibrous tumor is a rare neoplasm derived from the mesenchyme that most commonly affects the pleura, typically well circumscribed, pedunculated, with vessels within the tumor pedicle, and can become large, being considered giant when they are larger than 15 cm diameter. Conclusión: The correct diagnosis is of vital importance, since surgical resection it a potentially curable treatment. Surgical treatment can be performed by thoracotomy or videothoracoscopy, depending on the size of the tumor. Despite the benign behavior, it requires long-term follow-up due to the tendency to recur.

Tumor fibroso solitario de la pleura / Solitary fibrous tumor of the pleura

El tumor fibroso solitario de la pleura (TFSP) representa una neoplasia benigna de evolución habitualmente silente, hallazgo incidental y heterogeneidad en su presentación. Raramente se malignizan y la sintomatología se relaciona fundamentalmente con el compromiso por compresión de las estructuras vecinas. Su tratamiento es la cirugía. Se describe el caso de un paciente masculino de 49 años con antecedentes de hipertensión arterial, diabetes mellitus y obesidad que consultó al Servicio de urgencias por disnea. Se solicitó tomografía de tórax que evidenció voluminosa masa de densidad de partes blandas en hemitórax izquierdo con desplazamiento del mediastino y descenso del diafragma del mismo lado. La fibrobroncoscopia no evidenció lesión endoluminal. Fue evaluado por Cirugía del Tórax. Se realizó abordaje de la masa mediante toracotomía postero lateral izquierda. El estudio anatomopatológico informó formación tumoral de 25 × 16 × 13 cm, peso de 1905 gr y en la microscopía se describió proliferación celular fibroblástica, células fusiformes de núcleos pequeños ovoides y escaso citoplasma dispuestas en haces desordenados con colágeno interpuesto. La inmunohistoquímica informó vimentina y CD34 positivos. Se realizó el diagnóstico de tumor fibroso solitario de la pleura. La evolución posterior fue buena.

The solitary fibrous tumor of the pleura (SFTP) represents a benign neoplasm of commonly silent evolution, incidental finding and heterogeneous presentation. It rarely becomes malignant and symptoms mainly result from the involvement of neighboring structures due to compression. It is treated with surgery. We describe the case of a 49-year-old male patient with history of arterial hypertension, diabetes mellitus and obesity who consulted the Emergency Services because he was experiencing dyspnea. We requested chest tomography that showed a voluminous soft tissue density mass in the left hemithorax with mediastinal shift and decreased diaphragm on the same side. The fibrobronchoscopy didn't show endoluminal lesion. The patient was evaluated by the Thorax Surgery staff. The mass was treated by means of left posterolateral thorachotomy. The anatomopathological study reported the formation of a tumor of 25 × 16 ×13 cm and 1905 gr, and the microscopy described fibroblast cell proliferation, spindle cells of small ovoid nuclei and very little cytoplasm placed in disorganized bundles with collagen interposition. The immunohistochemistry disclosed positive vimentin and CD34. The patient was diagnosed with solitary fibrous tumor of the pleura, with good subsequent evolution.

Tumor fibroso solitario gigante de pleura. Serie de casos / Giant Solitary Fibrous Tumor of the Pleura. Case Series

Resumen Introducción. El tumor fibroso solitario de pleura (TFSP) es una neoplasia poco frecuente, con aproximadamente 1.000 casos reportados en la literatura mundial. La aproximación diagnóstica inicial se realiza con estudios imagenológicos. Métodos. De forma retrospectiva, se recopilaron cuatro casos de pacientes con TFSP gigante operados en nuestra institución. Se describen las características sociodemográficas, clínicas, imagenológicas, macroscópicas y microscópicas de cada caso. Resultados. Todos los pacientes de la serie cursaron con manifestaciones clínicas, con un promedio de 23,75 meses de evolución. El 50% de los tumores se localizaron en la cavidad pleural derecha y el 50% en la izquierda. En tomografía computarizada (TC) de tórax, los cuatro casos se presentaron como una masa sólida, de densidad heterogénea, con diámetros mayores entre 17 y 22 cm y contornos variables (lisos en tres casos y lobulados en un paciente). Se observaron calcificaciones intratumorales en dos casos y derrame pleural en tres pacientes. En cirugía, todas las masas presentaron pedículos. El análisis histológico e inmuno-químico confirmó la naturaleza benigna de tres casos y malignidad en una de las neoplasias. Conclusiones. Los TFSP generalmente son benignos y de buen pronóstico. Sin embargo, entre 10 y 20% de esos tumores son malignos. Las imágenes diagnósticas pueden sugerir el diagnóstico de TFSP, pero la confirmación de la naturaleza de la lesión debe realizarse con el análisis histopatológico de toda la pieza quirúrgica.

Abstract Introduction. Solitary fibrous tumor of the pleura (SFTP) is a rare neoplasm, with ~1.000 cases reported in the worldwide literature. The initial diagnostic approach is performed by imaging studies. Methods. Retrospectively, we collected four cases of patients with giant SFTP operated in our institution. The sociodemographic, clinical, imaging, macroscopic, and microscopic characteristics of each case are described. Results. All the patients in the series had clinical manifestations, with an average of 23.75 months of evolution. 50% of the tumors were located in the right pleural cavity and 50% in the left. In chest computed tomography (CT), the four cases presented as a solid mass, of heterogeneous density, with greater diameters between 17 and 22 cm, and variable contours (smooth in three cases and lobulated in one patient). Intratumoral calcifications were observed in two cases and pleural effusion in three patients. In surgery, all masses presented pedicles. The histological and immunochemical analysis confirmed the benign nature of three cases and malignancy in one of them. Conclusions. SFTPs are usually benign and have a good prognosis. However, between 10 and 20% of these tumors are malignant. Diagnostic images may suggest the diagnosis of SFTP, but confirmation of the nature of the lesion should be made with the histopathological analysis of the entire surgical specimen.

Fibrohistiocitoma maligno de face: uma apresentação rara / Malignant fibrohistiocytoma of face: a rare presentation

Contexto: O fibrohistiocitoma maligno é um sarcoma de tecidos moles muito agressivo, com rara apresentação limitada à pele e tecido subcutâneo em face. O diagnóstico é anatomopatológico com auxílio da imuno-histoquímica. Descrição do caso: Este artigo relata o caso de um paciente com diagnóstico de fibrohistiocitoma maligno restrito à face com boa resposta terapêutica após exérese cirúrgica. Discussão: Tendo em vista a raridade dessa afecção, dificuldade diagnóstica devido ao quadro inespecífico e com rápida evolução, é importante lembrar desse possível diagnóstico e atuar precocemente. Conclusões: O diagnóstico precoce interfere de forma significativa na evolução do quadro, sendo necessária a manutenção do acompanhamento oncológico e dermatológico com o intuito de detectar precocemente recidivas locais e metástases a distância.

Histiocitoma fibroso maligno no tornozelo: relato de caso / Malignant fibrous histiocytoma in ankle: case report

Histiocitoma fibroso maligno (MFH) ou sarcoma pleomórfico indiferenciado (UPS) é um sarcoma moderadamente agressivo, capaz de invadir estruturas adjacentes. Trata-se de neoplasia mesenquimal que predomina em homens entre a sexta e sétima décadas de vida. Localiza-se, principalmente, nos membros inferiores, podendo acometer cabeça e pescoço, tronco e retroperitônio, com tendência à recorrência e à metástase local. O presente relato tem como objetivo apresentar um caso de MFH no tornozelo de uma mulher de 49 anos, com invasão óssea adjacente, que evoluiu com amputação transtibial. São abordados aspectos clínicos, radiológicos, histopatológicos e terapêuticos, salientando-se a importância do diagnóstico precoce.

Malignant fibrous histiocytoma (MFH) or undifferentiated pleomorphic sarcoma (UPS) is a sarcoma capable of invading adjacent structures. It is a mesenchymal neoplasia that predominates in men between the sixth and seventh decades of life. It is located mainly in the lower limbs and may affect the head and neck, trunk, and retroperitoneum, presenting a tendency to recurrence and local metastasis. This report aims to present a case of MFH in the ankle of a 49-year-old woman with an adjacent bone invasion, which evolved with transtibial amputation. Clinical, radiological, histopathological, and therapeutic aspects were addressed, highlighting the importance of early diagnosis

El análisis inmunohistoquímico para el diagnóstico del tumor fibroso solitario/hemangiopericitoma / Immunohistochemical analysis for the diagnosis of solitary fibrous tumor/hemangiopericytoma

Resumen El tumor fibroso solitario/ hemangiopericitoma (TFS/HP) es un tumor extraaxial de origen mesenquimático de infrecuente observación, que usualmente se confunde con el meningioma, del cual puede ser clínica y radiológicamente indistinguible. El análisis molecular con la detección de la expresión nuclear STAT6 (signal transducer and activator of transcription 6) o la fusión NAB2-STAT6 (NGFI-A binding protein 2) es recomendable para confirmar el diagnóstico. Presentamos 3 casos clínicos, 2 mujeres y 1 varón, con diagnóstico anatomopatológico de meningioma meningotelial en el primer caso; y los casos 2 y 3 con sospecha radiológica de meningioma. La revisión anatomopatológica con estudio molecular permitió certificar el diagnóstico de TFS/ HP. Para el diagnóstico diferencial entre TFS/HP meníngeo y meningioma, se recomienda buscar la expresión de STAT6 como primer paso o la fusión NAB2-STAT6. La revisión de las muestras de biopsia debe estar garantizada en todos los pacientes, inclusive en aquellas que fueron estudiadas en Servicios de Patología Nivel 3.

Abstract The solitary fibrous tumor/ hemangiopericytoma (TFS/HP) is a rare mesenchymal extraaxial tumour. TFS/HP can sometimes be difficult to distinguish from other extra-axial tumors like meningioma, which can be clinically and radiologically indistinguishable. Molecular analysis with STAT6 (signal transducer and activator of transcription 6) nuclear expression or NAB2-STAT6 (NGFI-A binding protein 2) fusion is recommended to confirm the diagnosis. We present 3 cases, 2 women and 1 male, with pathological diagnosis of meningothelial meningioma in the first case; cases 2 and 3 with radiological suspicion of meningioma. The pathological review with molecular study certified the diagnosis of TFS/HP. For differential diagnosis between meningeal TFS/HP and meningioma, it is recommended to look for STAT6 expression as a first step, or NAB2-STAT6 fusion in order to confirm TFS/HP. The review of biopsy samples must be guaranteed in all patients, including those who were studied in Pathology Services Level 3.

Fibroma Xantoma Atípico a Propósito de un caso de una mujer en edad mediana / Atypical Xanthoma Fibroma Based on a case of a middle-aged woman

RESUMEN El fibroxantoma atípico es un raro tumor mesenquimatoso de malignidad intermedia. Se lo considera en la actualidad una variantesuperficial delhistiocitoma fibroso maligno. Afecta en su mayoría a pacientes de edades avanzadas y con historia de foto daño. El pronóstico es favorable con la extirpación completa de la lesión. Exhibeíndices bajos de metástasis. Presentamos el caso de una paciente con clínica, histología e inmunomarcación compatibles con fibroxantoma atípico.El interés de esta presentación, se fundamenta en la escasa prevalencia documentada de esta patología en la adultez, sin evidenciar participación infiltrativa del tejido óseo-medular en el control evolutivo.

SUMMARY Atypical fibroxanthoma is a rare mesenchymal tumor of intermediate malignancy. It is currently considered a superficial variant of undifferentiated pleomorphic sarcoma. It mostly affects elderly patients with a history of photo damage. The prognosis is favorable with the complete removal of the lesion. Exhibits low rates of metastasis. We present the case of a patient with symptoms, histology and immunostaining compatible with atypical fibroxanthoma. The interest of this presentation is based on the low documented prevalence of this pathology in adulthood, without evidence of infiltrative participation of bone-marrow tissue in evolutionary control.

Tumor fibroso solitario hepático gigante. Informe de caso en la Argentina / Giant solitary fibrous tumors of the liver. A case report in Argentina

RESUMEN Los tumores fibrosos solitarios hepáticos, una neoplasia poco frecuente, son tumores benignos del tejido submesotelial, pero con un potencial maligno no definido. Suelen presentarse asintomáticos, pero pueden debutar como una masa abdominal palpable y con síntomas de plenitud. Por imágenes pueden ser confundidos con otros tumores y está indicado el tratamiento quirúrgico. Presentamos el caso de una paciente de 70 años resuelto por hepatectomía lateral izquierda, y realizamos una revisión bibliográfica del tema.

ABSTRACT Solitary fibrous tumors of the liver are uncommon benign tumors originating from the submesothelial tissue with non-well-defined malignant potential. Most cases present as asymptomatic, some cases show abdominal bloating and a palpable mass. They may mimic other tumors on imaging tests and surgery is indicated. We report a case of a 70-year-old female patient treated with left lateral liver resection with literature review of the condition.

Dermatofibrossarcoma protuberante mimetizando queloide / Dermatofibrosarcoma protuberans simulating keloid

O dermatofibrossarcoma protuberante é um sarcoma localmente agressivo, de malignidade intermediária, que predomina na faixa de adultos jovens à meia-idade. Lesões congênitas ou na infância são raras, mas foram relatadas. Nos estágios precoces, pode ser mal diagnosticado e ser incompletamente excisado, o que aumenta o risco de recorrências. Relata-se o caso de paciente do sexo masculino, de 24 anos, com história de mácula hipercrômica em região abdominal desde o nascimento que evoluiu para tumoração após duas abordagens cirúrgicas. Biópsia e imuno-histoquímica confirmaram o diagnóstico de dermatofibrossarcoma protuberante, e a lesão foi excisada com margem de 3cm

Dermatofibrosarcoma protuberans is a locally aggressive sarcoma of intermediate malignancy that predominates in the range of young adults to middle age. Congenital or childhood lesions are rare but have been reported. It can be misdiagnosed and incompletely excised in the early stages, which increases the risk of recurrence. The present article reports the case of a 24-year-old male patient presenting a hyperchromic macula in the abdominal region from birth that evolved into a tumor after several surgical approaches. Biopsy and immunohistochemistry confirmed the diagnosis of dermatofibrosarcoma protuberans, and the lesion was excised with a 3 cm margin

Dermatoscopia do dermatofibroma aneurismático: relato de dois casos / Dermoscopy of aneurysmal dermatofibroma: report of two cases

O dermatofibroma (DF) aneurismático é um tumor benigno raro, de origem na derme, considerado uma variante de dermatofibroma. É prevalente nos membros inferiores de mulheres acima de 30 anos. Sua etiologia é desconhecida, e o exame histopatológico confirma o diagnóstico. Este trabalho apresenta dois casos de DF aneurismático, em pacientes jovens, do sexo masculino, e com lesões semelhantes: nódulo hipercrômico único, de aproximadamente 1,5cm e crescimento progressivo. O objetivo deste relato é demonstrar uma variante pouco frequente de dermatofibroma e ressaltar o possível diagnóstico diferencial com outros tumores por meio do exame dermatoscópico.

Aneurysmal dermatofibroma (DF) is a rare benign tumor originating in the dermis, considered a variant of dermatofibroma. It is more prevalent in women over 30 years of age, in the lower limbs. Its etiology is unknown and histopathological examination confirms the diagnosis. This study reports two cases of aneurysmal DF in young patients, both men, presenting similar lesions: single hyperchromic nodule, with approximately 1.5 cm and progressive growth. This report aims to demonstrate an uncommon variant of dermatofibroma and to highlight the possible differential diagnosis with other tumors through dermoscopic examination

First report of hemosiderotic adenodermatofibroma in Brazil / Primeiro relato de adenodermatofibroma hemossiderótico no Brasil

ABSTRACT Hemosiderotic adenodermatofibroma is a recently recognized lesion, characterized by a dermal nodule with cystic structures of an apocrine gland, surrounded by a dermatofibroma-like stroma with hemosiderotic macrophages. We present the first case report of this entity in Brazil together with representative images, in addition to a review on the subject and discussion about the apocrine origin of this lesion.

RESUMEN El dermatoadenofibroma hemosiderótico es una lesión recientemente descrita, caracterizada por un nódulo dérmico con estructuras quísticas de una glándula apocrina, rodeado por un estroma del tipo dermatofibroma con macrófagos hemosideróticos. Presentamos el primer reporte de caso de esa entidad en Brasil, junto a imágenes representativas, además de una revisión del tema y discusiones acerca del origen apocrina de esa lesión.

RESUMO Adenodermatofibroma hemossiderótico é uma lesão recentemente conhecida, caracterizada por um nódulo dérmico com estruturas císticas de uma glândula apócrina, circundado por um estroma do tipo dermatofibroma com macrófagos hemossideróticos. Apresentamos o primeiro relato de caso dessa entidade no Brasil junto a imagens representativas, além de uma revisão do assunto e discussões sobre a origem apócrina dessa lesão.

Tumor fibroso de la túnica vaginal / Fibrous tumor of the vaginal tunic

RESUMEN Se presenta el caso de un paciente de 21 años de edad que refiere aumento paulatino del escroto de varios meses de evolución, sin dolor u otros síntomas. Con el objetivo de exponer una lesión benigna paratesticular clasificada como, tumor fibroso de la túnica vaginal, se practicó una escrototomía con toma de biopsia, exéresis de la tumoración y se conservó el testículo. El tejido fibroso fue puesto en evidencia con la biopsia intraoperatoria, posteriormente la histopatología hizo el diagnóstico de tumor fibroso. La orquiectomía es posible evitarla cuando nos encontramos ante lesiones paratesticulares que son benignas en la mayoría de los casos(AU)

ABSTRACT We present the case of a 21-year-old patient who reports a gradual increase in the scrotum of several months of evolution, without pain or other symptoms. In order to expose a benign paratesticular lesion classified as fibrous tumor of the vaginal tunic, a scrototomy was performed with biopsy, excision of the tumor and the testicle was preserved. The fibrous tissue was revealed with the intraoperative biopsy, later histopathology made the diagnosis of fibrous tumor. Orchiectomy can be avoided when we are faced with paratesticular lesions that are benign in most cases(AU)