Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso / Persistent spontaneous pneumothorax as a primary manifestation of primary synovial sarcoma of the lung: a case report

El sarcoma sinovial pleuropulmonar (SSPP) es un tumor primario de pulmón, maligno, infrecuente en pediatría (prevalencia 0,1-0,5 %) que afecta predominantemente a adolescentes y adultos jóvenes. Se ha descrito una sobrevida global cercana al 30 % a los 5 años. Se reporta el caso de un paciente de 12 años de edad, previamente sano, que presentó tos, dolor torácico y disnea de comienzo súbito, como manifestación inicial de neumotórax izquierdo, el que persistió a los 4 días y requirió resección quirúrgica de lesión bullosa pulmonar. Se realizó diagnóstico histológico de sarcoma sinovial pleuropulmonar confirmado por estudio molecular, que evidenció la translocación cromosómica entre el cromosoma X y el 18: t(X;18) (p11.2;q11.2) de la pieza quirúrgica extirpada. Ante pacientes con neumotórax persistente o recidivante, es importante descartar causas secundarias, entre ellas, sarcoma sinovial pleuropulmonar. Su ominoso pronóstico determina la necesidad de arribar a un diagnóstico temprano e implementar un tratamiento agresivo

Pleuropulmonary synovial sarcoma (PPSS) is a primary malignancy of the lung, uncommon in pediatrics (prevalence: 0.1­0.5%) that predominantly affects adolescents and young adults. Overall survival has been reported to be close to 30% at 5 years. Here we report the case of a previously healthy 12-year-old male patient who presented with cough, chest pain, and dyspnea of sudden onset as initial manifestation of left pneumothorax, which persisted after 4 days and required surgical resection of pulmonary bullous lesion. A histological diagnosis of pleuropulmonary synovial sarcoma was made and confirmed by molecular study, which showed chromosomal translocation between chromosomes X and 18: t(X;18) (p11.2;q11.2) in the surgical specimen removed. In patients with persistent or recurrent pneumothorax, it is important to rule out secondary causes, including pleuropulmonary synovial sarcoma. Such poor prognosis determines the need for early diagnosis and aggressive treatment.

Sarcoma sinovial primario pulmonar: un caso inusual en pediatría / Primary pulmonary synovial sarcoma: an unusual case in pediatrics

El sarcoma sinovial primario de pulmón (SSPP) localizado en bronquio, es una entidad no reportada en la edad pediátrica. Se presenta el caso de un niño de siete años con antecedente de aparentes neumonías recurrentes derechas de siete meses de evolución; en la evaluación por neumología pediátrica se destaca en las radiografías de tórax, la presencia de atelectasias recurrentes en lóbulo medio e inferior derecho, por lo que se realiza broncoscopia, donde se observa una masa obstruyendo el 100% de la luz del bronquio fuente derecho y se sospecha tumor carcinoide. Se realiza extirpación de masa endobronquial con fines diagnósticos y terapéuticos, incluyendo resección segmentaria bronquial y anastomosis término-terminal. El respectivo análisis inmunohistoquímico muestra hallazgos sugestivos de sarcoma sinovial monofásico. Se descarta compromiso tumoral extrapulmonar, por lo que se diagnostica como tumor primario de bronquio. Se administraron 7 ciclos de quimioterapia y 31 sesiones de radioterapia. Actualmente en control, sin evidencia de metástasis, tumores residuales o recidivas.

Primary Synovial Sarcoma of Lung (PSSL) located in the bronchus is an unreported entity in pediatric age. We present the case of a 7-year-old child with a history of apparent recurrent right pneumonia of 7 months of evolution; in the evaluation by pediatric pulmonology, the presence of recurrent atelectasis in the middle and lower right lobe is highlighted on chest X-rays, so bronchoscopy is performed, where a mass is observed obstructing 100% of the right bronchus lumen and carcinoid tumor is suspected. Endobronchial mass resection is performed for diagnostic and therapeutic purposes, including bronchial segmental resection and termino-terminal anastomosis. The respective immunohistochemical analysis shows suggestive findings of monophasic synovial sarcoma. Extrapulmonary tumor involvement is ruled out, so it is diagnosed as a primary bronchial tumor. 7 cycles of chemotherapy and 31 sessions of radiation therapy are given. Currently in control, with no evidence of metastasis, residual tumors, or recurrence.

Sarcoma sinovial da laringe: Relato de caso sob a perspectiva da deglutição, voz e qualidade de vida / Synovial Sarcoma of the Larynx: Case report from the perspective of swallowing, voice and quality of life / Sarcoma de laringe sinovial: reporte de un caso desde la perspectiva de la deglución, la voz y la calidad de vida

Introdução: O sarcoma sinovial é um tumor agressivo comumente encontrado nos membros inferiores e raramente descrito na laringe. A literatura recomenda tratamento multimodal com radioterapia, quimioterapia e laringectomia total. Objetivo: Descrever os impactos funcionais e de qualidade de vida em voz e deglutição de um adolescente que desenvolveu um sarcoma sinovial monofásico da laringe. Método: Adolescente de 16 anos que desenvolveu um sarcoma sinovial monofásico da laringe na infância, submetido a uma aritenoidectomia extendida por acesso laringofaríngeo associado à radioquimioterapia aos 11 anos de idade. O tratamento oncológico menos agressivo foi adequado para o controle oncológico e resultados funcionais do caso aqui descrito, apresentando voz com qualidade satisfatória e via oral exclusiva. A avaliação endoscópica demonstrou uma laringe com proporções infantis, revelando a interrupção do crescimento no momento do tratamento. O paciente apresenta sobrevida de 5 anos e 6 meses, superior à sobrevida média de dois anos descrita na literatura, referindo boa qualidade de vida. Conclusão: O adolescente apresenta controle oncológico e resultados funcionais de voz e deglutição satisfatórios, sem grandes interferências em sua qualidade de vida. O suporte familiar e atendimento multiprofissional especializado demonstraram-se primordiais.

Introduction: Synovial sarcoma is an aggressive tumor commonly found in the lower limbs and rarely described in the larynx. The literature recommends multimodal treatment with radiotherapy, chemotherapy and total laryngectomy. Purpose: To describe the functional and quality of life impacts on voice and swallowing of an adolescent who developed a monophasic synovial sarcoma of the larynx. Method: A 16-year-old adolescent who developed a monophasic synovial sarcoma of the larynx in childhood, who underwent an extended arytenoidectomy due to laryngopharyngeal access associated with radiotherapy at the age of 11. The less aggressive cancer treatment was adequate for cancer control and functional results of the case described here, presenting a voice with satisfactory quality and exclusive oral route. The endoscopic evaluation showed a larynx with infantile proportions, revealing growth interruption at the time of treatment. The patient has a 5-year and 6-month survival rate, higher than the average two-year survival rate described in the literature, referring to a good quality of life. Conclusion: The adolescent has oncological control and satisfactory functional results of voice and swallowing, without major interferences in his quality of life. Family support and specialized multidisciplinary care proved to be paramount.

Introducción: El sarcoma sinovial es un tumor agresivo que se encuentra comúnmente en las extremidades inferiores y rara vez se describe en la laringe. La literatura recomienda el tratamiento multimodal con radioterapia, quimioterapia y laringectomía total. Propósito: Describir los impactos funcionales y de calidad de vida en la voz y la deglución de un adolescente que desarrolló un sarcoma sinovial monofásico de la laringe. Método: Un adolescente de 16 años que desarrolló un sarcoma sinovial monofásico de la laringe en la infancia, que se sometió a una aritenoidectomía prolongada debido al acceso laringofaríngeo asociado con radioterapia a la edad de 11 años. El tratamiento contra el cáncer menos agresivo fue adecuado para el control del cáncer y los resultados funcionales del caso descrito aquí, presentando una voz con calidad satisfactoria y una vía oral exclusiva. La evaluación endoscópica mostró una laringe con proporciones infantiles, revelando la interrupción del crecimiento en el momento del tratamiento. El paciente tiene una tasa de supervivencia a 5 y 6 meses, superior a la tasa de supervivencia promedio de dos años descrita en la literatura, que se refiere a una buena calidad de vida. Conclusión: El adolescente tiene control oncológico y resultados funcionales satisfactorios de voz y deglución, sin grandes interferencias en su calidad de vida. El apoyo familiar y la atención multidisciplinaria especializada resultaron ser primordiales.

Cinematic rendering for three-dimensional reconstructions of the chest wall: a new reality / Uma nova realidade: renderização cinematográfica para reconstruções tridimensionais da parede torácica

ABSTRACT Computed tomography with multiple detectors and the advancement of processors improved rendered images and three-dimensional reconstructions in clinical practice. Traditional axial slices form non-intuitive images because they are seen in only one plane. The three-dimensional reconstructions can show structures details and diseases with complex anatomy in different perspectives. Cinematic rendering is a newly three-dimensional reconstruction technique, already approved for clinical use, which can produce realistic images from traditional computed tomography data. The algorithm used is based on light trajectory methods and the global lighting model, which simulate thousands of images from all possible directions. Thus, the technique shapes the physical propagation of light and generates a realistic three-dimensional image with depth, shadows and more anatomic details. It is a multidimensional rendering acquired through complex lighting effects. The aim of this article was to show the advance of three-dimensional technology with the cinematic rendering in images exams of the thoracic wall.

RESUMO A tomografia computadorizada com os múltiplos detectores e o avanço dos processadores melhoraram as imagens renderizadas e as reconstruções tridimensionais na prática clínica. Os cortes axiais tradicionais formam imagens não intuitivas, pois são vistas em apenas um plano. Já as reconstruções tridimensionais podem exibir detalhes anatômicos em diferentes perspectivas das estruturas e de doenças com anatomia complexa. A renderização cinematográfica é uma técnica de reconstrução tridimensional recentemente introduzida, já aprovada para uso clínico, que pode produzir imagens realistas a partir de dados tradicionais da tomografia computadorizada. O algoritmo usado é baseado em métodos de trajetória da luz e no modelo de iluminação global, os quais simulam milhares de imagens de todas as direções possíveis. Assim, a técnica molda a propagação física da luz e gera uma imagem tridimensional realista, com profundidade, sombras e mais detalhes da alteração anatômica. É uma renderização multidimensional adquirida por efeitos de iluminação complexos. O objetivo deste artigo foi mostrar o avanço da tecnologia tridimensional com a renderização cinematográfica nos dos exames de imagens da parede torácica.

Sarcoma sinovial monofásico de tipo fibroblástico. Evaluación diagnóstica por RM y postratamiento mediante ADC: Presentación de un caso / Monophasic Fibroblastic Synovial Sarcoma. Diagnostic and Post-Treatment Assessment by MRI and ADC: Case Report

El sarcoma sinovial representa entre el 2 al 10 % de todas las malignidades primarias de tejidos blandos, situándose en el cuarto lugar en frecuencia de los sarcomas de tejidos blandos. Es una neoplasia clasificada por la WHO (World Health Organization) bajo la categoría de tumores de diferenciación incierta, considerándose una malignidad entre intermedio y alto grado. El tratamiento estándar es la escisión quirúrgica, sin embargo, por su alta recurrencia y en los casos en que se asocia a metástasis o márgenes positivos después de la resección, se han planteado otros tratamientos como la quimio y radioterapia. La imaginología juega un papel importante en el diagnóstico, la estadificación y evaluación de la respuesta al tratamiento. Nuevas técnicas como el uso de difusión y mapas de ADC (Apparent Diffusion Coefficient, o coeficiente de difusión aparente) en resonancia magnética (RM) son útiles para evaluar las lesiones tumorales y la respuesta al tratamiento. En este artículo se presenta el caso de un paciente con un sarcoma sinovial monofásico, el diagnóstico por imágenes y evaluación del tratamiento mediante técnicas avanzadas de resonancia magnética como la difusión

Synovial sarcomas represent 2 to 10% of all the primary tissue malignancies and occupy the fourth place in the list of most common soft tissue sarcomas. According to the World Health Organization (WHO), this neoplasm is classified under the category of tumors of uncertain differentiation and is considered an intermediate to high-grade malignancy. Although the standard treatment is surgical excision, alternative treatments such as radiotherapy and chemotherapy have been proposed due to its high rate of recurrence in cases when it is associated with metastasis or positive resection margins. Imaging plays a key role in the diagnosis, staging and assessment of treatment of this disease. New techniques in Magnetic Resonance Imaging such as diffusion and Apparent Diffusion Coefficient (ADC) mapping are useful to further characterize these neoplastic lesions and to assess treatment response. In this article we present a patient with monophasic synovial sarcoma in which the use of these new imaging techniques was essential for the diagnosis and evaluation post-treatment.

Evolución infrecuente de sinoviosarcoma de muslo / Uncommon evolution after excision of a thigh synovial sarcoma

Background: Synovial sarcoma is a soft tissue tumor with a high degree of local and metastatic invasion. Case report: We report a 26 years old smoker woman with a history of a synovial tumor excised from the right thigh, who required a hip prosthesis for a pathological fracture due to metastases. Two years after, a lung metastasis in the left upper lobe was excised and treated with chemo and radiotherapy. Two years later a new lung nodule was found and excised. Pathology confirmed that they were metastases of the primary tumor. Three months after this last excision, the patient is asymptomatic.

Introducción: El sinoviosarcoma es una neoplasia de partes blandas con alto grado de invasión local y producción de metástasis. Caso clínico: Mujer de 26 años con 2 episodios secuenciales de metástasis pulmonar a lo largo de 6 años posterior al tratamiento del tumor primario. Patología de tratamiento muy complejo.

Sarcomas de partes blandas en pacientes pediátricos: análisis de una serie de casos del subtipo no rabdomiosarcoma / Pediatric soft tissue sarcomas: analysis of non rhabdomyosarcoma group

Se evaluó una serie de 29 pacientes con diagnóstico de sarcomas de partes blandas variante no rabdomiosarcomas tratados quirúrgicamente entre2000 y 2010, y se analizó la supervivencia global y los factores que influyen en el pronóstico. La edad media fue de 11,6 años (rango de 3 meses-17 años); 16 pacientes eran de sexo masculino y el tiempo promedio de seguimiento fue de 56 meses (de 8 a 132 meses). Se documentaron 8 variedades histológicas diferentes de tumores malignos, y el sarcoma sinovial resultó ser el más frecuente (14 pacientes). La cirugía de conservación de miembro fue posible en 28 pacientes y se asoció tratamiento adyuvante en 26. La supervivencia global de la serie estudiada fue de 72% a los 5 años y, en 9 pacientes, se diagnosticó una recurrencia local. La presencia de metástasis (p < 0,0001) y la recurrencia local (p < 0,007) resultaron ser factores de pronóstico negativo para la supervivencia global.

We evaluated 29 patients with non rhabdomyosarcoma soft tissue sarcomas treated with surgery between 2000 and 2010; we analyzed overall survival and which factors affect the prognosis. The mean age was 11.6 years (range 3 months-17 years); 16 patients were males and the median follow-up was 56 months (8 to 132 months). Eight different histological malignant tumors were identified, being synovial sarcoma the most prevalent one (14 patients). Twenty-eight patients were treated with limb salvage surgery and in 26 cases, adjuvant therapy was used. Five years overall survival was 72%. Nine of the 29 patients presented a local recurrence. The presence of metastases (p <0.0001) and local recurrence (p <0.007) were negative prognostic factors for overall survival.

Poorly differentiated synovial sarcoma in the wrist - Case report

Synovial sarcomas are rare malignant tumors affecting mainly young adults, presenting as a slow growth mass located in deep soft tissues of extremities, near the joints. In this report a 34-year-old male patient, presented an ulcerovegetative lesion on the right wrist which was completely excised. Histopathology and immunohistochemistry confirmed synovial sarcomas with poorly differentiated cells. This patient presented 11 months later with ipsilateral axillary lymph node metastasis, which emphasizes the unfavorable prognosis of this synovial sarcoma variant. The indolent growth pattern of this sarcoma justifies the well circumscribed initial stages, which progressively infiltrate adjacent structures with lung metastasis (80%) and lymph node involvement (20%) and thus corroborates the importance of early diagnosis and proper treatment.

Synovial Sarcoma in Head and Neck: A Case Report

Introduction: Synovial sarcoma is a malignant tumor of mesenchymal pluripotent cells. Objectives: We present a case of synovial sarcoma in the posterolateral wall of the oropharynx. Resumed report: The patient, a 23-year-old woman, was admitted with a history of dysphagia and difficulty in breathing for 8 months, resulting in progressive deterioration and onset of snoring, muffled voice, and local pain. An oropharyngeal tumor in the left posterolateral wall touched the base of the ipsilateral tongue. The patient underwent endoscopic pharyngectomy to remove the lesion. Pathologic examination revealed synovial sarcoma with positive margins, and Mohs technique was proposed for margin control. The margins were disease-free, without the need for total laryngectomy. The pharynx was reconstructed with a microvascular forearm flap. The patient developed postoperative stability. Conclusion:  Despite its name, synovial sarcoma is rarely sourced directly from synovial membranes. It is most commonly found in the vicinity of large joints. The location at the head and neck, a location poor in synovial tissue, is unusual. Synovial sarcoma in the head and neck has an aggressive nature and poor prognosis. Resection with negative margins remains the foundation of therapy, which is not so easily achieved in the head and neck. It is important for the otorhinolaryngologist and head and neck surgeon to be familiar with this aggressive tumor, which carries high mortality and morbidity. The appropriate diagnosis and treatment can improve prognosis and patient survival...

Sarcoma sinovial primário do esôfago / Primary synovial sarcoma of the esophagus

Synovial sarcomas are uncommon malignant mesenchymal tumors occurring mainly near the joints of the extremities of young adults. Synovial sarcomas are exceedingly rare neoplasms of the digestive tract. We report the first diagnosed case of esophageal synovial sarcoma, highlighting its diagnostic features surgical management and follow-up.

Sarcoma sinovial bifásico pulmonar: presentación de un caso y revisión de la literatura / Biphasic synovial sarcoma of the lung: report of a case and review of the literature

Introduction: Synovial sarcoma is an aggressive neoplasm described in several locations but is uncommon in the lung. Method: We report a case of biphasic synovial sarcoma of the lung stressing the histologic and immunohistochemical characteristics, genetics and differential diagnosis. Results: a biphasic pattern and SYT-SSX translocation were demostrated. Comments: histology and immuno-histochemistry lead to diagnosis most of the times but the typical translocation is definitive.

Introducción: El sarcoma sinovial es una neoplasia agresiva que aun habiendo sido descrita en múltiples localizaciones, resulta sumamente infrecuente en localización pulmonar. Método: Presentamos un caso de sarcoma sinovial bifásico de localización pulmonar haciendo hincapié en sus características histológicas inmunohistoquímicas y genéticas así como en su diagnóstico diferencial. Resultados: La neoplasia mostraba un patrón bifásico bien caracterizado. El estudio genético demostró la translocación SYT-SSX. Comentarios: Si bien la histología y la inmunohistoquímica permiten en la mayoría de los casos el diagnóstico del sarcoma sinovial, es la translocación genética la que define verdaderamente esta entidad.

Sinoviosarcoma de tornozelo: quarta recidiva / Ankle's synoviosarcoma: fourth relapse

Esse relato apresenta o caso de uma paciente com quarta recidiva de sinoviosarcoma, um sarcoma de partes moles que possui estreita relação com bainhas tendinosas, bursas e cápsulas articulares. A paciente apresentou uma quarta recidiva, sendo necessária a realização da amputação de perna, visto que as cirurgias conservadoras já não aliviam o sofrimento da paciente. O principal objetivo deste relato de caso é apresentar um caso clássico de neoplasia rara, expondo a doença e fazer uma breve revisão da literatura e mostrar sua importância, visto que corresponde a apenas 5% das neoplasias de partes moles.

This report presents the case of a female patient with fourth synoviosarcoma recurrence, a sarcoma of soft parts that have close relationship with tendom sheaths, bursas and articular capsule.The patient present her fourth recurrence, in wich the perfomance of amputation was required, since the conservative surgeriesno longer relieved the patient suffering. The main objective of this case report is to present a classic case of a rare cancer, exposing the disease, and to make a brief review of the literature to show its importance, since it corresponds to only five percent of the cancers of soft parts.

Sarcoma sinovial digital / Digital Synovial Sarcoma

El sarcoma sinovial es una entidad bien definida clínica y morfológicamente y, a pesar de su nombre, es muy raro en las cavidades articulares. Este sarcoma afecta zonas sin relación aparente con las estructuras sinoviales y representa entre el 5% y el 10% de todos los sarcomas de los tejidos blandos. Se encuentra con mayor frecuencia en las extremidades, especialmente en las inferiores, donde tiende a localizarse en la vecindad de las grandes articulaciones; se han informado muy pocos casos de sarcomas sinoviales en las manos o los pies, con una evolución clínica favorable. Así mismo, es más frecuente de 15 a 40 años de edad (con una edad media de 34 años). Este artículo presenta el caso de una mujer adulta a quien se le realizó el diagnóstico histopatológico e inmunohistoquímico de un sarcoma sinovial en el quinto dedo de la mano derecha.

Synovial sarcoma is a clinically and morphologically well defined entity which, in spite of its name, is rare in joint cavities. This sarcoma affects zones without any apparent relation to synovial structures and represents from 5% to 10% of all sarcomas in soft tissues. It is most frequently found in the extremities, especially the lower ones, where it tends to be located in the vicinity of the major articulations; there are very few favorable reports on the clinical evolution of synovial sarcomas in the hands or feet. It is most frequent between the ages of 15 to 40 (median age, 34). This article presents the case of an adult woman who underwent histopathologic and immunohistochemical diagnosis of a synovial sarcoma on the little finger of her right hand.

Compressão da medula espinhal torácica por metástase secundária de sarcoma sinovial: relato de caso / Compresión de la medula espinal torácica por metástasis secundaria de sarcoma sinovial: relato de caso / Thoracic spinal cord compression secondary to metastatic synovial sarcoma: case report

Synovial sarcoma is an uncommon malignant soft tissue neoplasm, occurring primarily in adolescents and young adults. It is prevalent in the periarticular soft tissues near large joints of the extremities and rarely involves the trunk. Metastases are not uncommon and usually involve the lungs; metastasis to the thoracic spine is rare. We report the case of a 47-year-old man with a history of synovial sarcoma of the lower back, with subsequent metastases to the lung, penis, and perineum (all previously resected), presenting with a 3-month history of low back pain and lower extremity paresthesias. Magnetic resonance imaging (MRI) demonstrated multiple lesions involving multiple contiguous vertebral bodies, with the mass at T12 compressing the spinal cord. The patient underwent T11-T12 laminectomy, transpedicular decompression, tumor debulking, and posterior fixation and fusion. The patient died six months later due to disease progression. Although not curative, decompression and stabilization of the spine are often necessary in patients who present spinal cord compression.

O sarcoma sinovial é uma neoplasia rara dos tecidos moles que afeta adolescentes e adultos jovens. A sua maior prevalência é nas grandes articulações das extremidades e raramente acomete o tronco. As lesões metastáticas são raras e geralmente acometem os pulmões, e as metástases para a coluna torácica são raras. Relata-se o caso de um paciente de 47 anos de idade com 3 meses de história de dor lombar e que apresentava metástase de sarcoma sinovial na coluna lombar. A ressonância magnética demonstrava lesões contíguas do corpo vertebral e compressão do canal vertebral ao nível de T12. O paciente foi submetido à laminectomia de T11-T12, descompressão transpedicular, remoção tecido tumoral e artrodese e fixação posterior. O paciente foi a óbito após seis meses devido à progressão da doença. Embora a descompressão e estabilização cirúrgica do canal vertebral não sejam curativas, esses procedimentos podem ser necessários em pacientes que apresentam compressão da medula espinhal.

El sarcoma sinovial es una neoplasia rara de los tejidos blandos que afecta adolescentes y adultos jóvenes. Su mayor prevalencia es en las grandes articulaciones de las extremidades y raramente ataca el tronco. Las lesiones metastásicas son raras y generalmente atacan los pulmones, siendo que las metástasis de columna torácica son raras. Será relatado el cuadro clínico de un paciente de 47 años de edad con tres meses de historia de dolor lumbar y presentando metástasis de sarcoma sinovial en la columna lumbar. La resonancia magnética demostraba lesiones contiguas del cuerpo vertebral y compresión del canal vertebral al nivel de T12. El paciente fue sometido a la laminectomía de T11-T12, descompresión transpedicular, remoción de tejido tumoral y artrodesis con fijación posterior. El paciente fue a óbito después de seis meses debido a la progresión de la enfermedad. Aunque la descompresión y estabilización quirúrgica del canal vertebral no sea curativa, ese procedimiento puede ser necesario en los pacientes que presentan compresión de la médula espinal.

Sarcoma sinovial monofásico primario de pulmón: reporte de un caso y revisión de la literatura / Single primary synovial sarcoma of the lung: a case report and review of the literature

El sarcoma sinovial es una entidad morfológica, clínica y genéticamente distinta y se puede apreciar en cualquier ubicación. Es un tumor mesenquimal de células fusiformes con una diferenciación epitelial variable. No existe una terapia estandarizada, generalmente el tratamiento es quirúrgico, con una resección total de la lesión, aplicándose en algunos casos radioterapia adyuvante. Los sarcomas sinoviales han mostrado quimiosensibilidad a la ifosfamida y a la doxorrubicina con una respuesta aproximada del 24 por ciento. Se presenta caso de una paciente de 32 años de edad quien presenta tumor en lóbulo inferior de pulmón izquierdo. Se le realizan estudios de extensión, paraclínicos y biopsia por punción dirigida por fluoroscopia que reportó neoplasia epitelial maligna poco diferenciada. Se diagnostica como cáncer de pulmón estadío I realizándose lobectomía inferior izquierda cuya biopsia y estudios inmunohistoquímicos diagnostica un sarcoma sinovial monofásico. Evoluciona satisfactoriamente refiriéndose a los servicios de oncología médica y radioterapia para tratamiento adyuvante.

Synovial Sarcoma is morphologic, clinical organization and genetically different and is possible to be appreciated in any location. Is mesenquimal tumor fusiform cell with variable epithelial differentiation. Treatment, standardized therapy does not exist; the treatment is generally surgical, with total resection of the injury, being applied in some cases adjuvant radiotherapy. Synovial sarcomas has shown chemo sensibility to ifosfamide one and doxorrubicin with an approximated answer of 24 %. A case of a patient of 32 years of age appears that presents displays tumor in inferior lobe of left lung. Studies of extension, paraclinics are made to him and biopsy by punction directed by fluoroscopy that reported neoplasia epithelial vitiates little differentiated. It is diagnosed as lung cancer stage I being made left inferior lobotomy whose inmunohystochemestry biopsy and studies diagnose Synovial sarcoma single-phase. It evolves satisfactorily talking about to the services of medical oncology and radiotherapy for adjuvant treatment.

Sarcoma sinovial intraoral primario monofásico / Monophasic synovial sarcoma of the oral cavity: report of one case

El sarcoma sinovial es un tumor maligno de partes blandas, bien diferenciado y que representa entre 5.6 por ciento a 10 por ciento de todos los sarcomas. Su localización en cabeza y cuello no es común, y representa cerca de un 9 por ciento, con menos de 100 casos reportados en la literatura. La localización intraoral es muy peculiar, existiendo 32 casos previamente descritos en el mundo, de los cuales 3 corresponden al tipo monofásico. Se presenta un caso de un paciente varón de 16 años con un caso de sarcoma sinovial intraoral monofásico y una revisión de la literatura disponible.

Synovial sarcoma is a well-differentiated malignant tumor of soft tissues, that corresponds approximately 10 percent of all soft tissue sarcomas. Its location in the head and neck uncommon and the location in the oral cavity is really peculiar, with only 32 previously described cases, of which only three corresponded to the monophasic type. We report a 16 years old male patient consulting for a lump in the right oral mucosa. The patient was operated in two occasions excising the tumor with radical lymph node dissection and parotid gland excision. The pathological diagnosis was a monophasic intraoral synovial sarcoma. Postoperative radiotherapy was recommended but not done. After 16 months of follow up the patient is without evidence of relapse.