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.

Human Leukocyte Antigen-A Allele Distribution in Nasopharyngeal Carcinoma Patients Showing Anti-Melanoma-Associated Antigen A or Synovial Sarcoma X-2 T Cell Response in Blood / 中华医学杂志(英文版)

<p><b>Background</b>Development of innovative immunotherapy is imperative to improve the poor survival of the nasopharyngeal carcinoma (NPC) patients. In this study, we evaluated the T cell response to melanoma-associated antigen (MAGE)-A1, MAGE-A3, or synovial sarcoma X-2 (SSX-2) in the peripheral blood of treatment-naive NPC patients. The relationship of responses among the three proteins and the human leukocyte antigen (HLA)-A types were analyzed to provide evidence of designing novel therapy.</p><p><b>Methods</b>Sixty-one NPC patients admitted into the Tumor Hospital affiliated to the Xinjiang Medical University between March 2015 and July 2016 were enrolled. Mononuclear cells were isolated from the peripheral blood before any treatment. HLA-A alleles were typed with Sanger sequence-based typing technique. The T cell response to the MAGE-A1, MAGE-A3, or SSX-2 was evaluated with the Enzyme-Linked ImmunoSpot assay. Mann-Whitney U-test was used to compare the T cell responses from different groups. Spearman's rank correlation was used to analyze the relationship of T cell responses.</p><p><b>Results</b>HLA-A*02:01, A*02:07, and A*24:02 were the three most frequent alleles (18.9%, 12.3%, and 11.5%, respectively) among the 22 detected alleles. 31.1%, 19.7%, and 16.4% of the patients displayed MAGE-A1, MAGE-A3, or SSX-2-specific T cell response, respectively. The magnitudes of response to the three proteins were 32.5, 38.0, and 28.7 SFC/10 peripheral blood mononuclear cells, respectively. The T cell response against the three proteins correlated with each other to different extent. The percentage of A*02:01 and A*24:02 carriers were significantly higher in patients responding to any of the three proteins compared to the nonresponders.</p><p><b>Conclusion</b>MAGE-A1, MAGE-A3, or SSX-2-specific T cell responses were detectable in a subgroup of NPC patients, the frequency and magnitude of which were correlated.</p>

A Case Report of Primary Pulmonary Synovial Sarcoma with Postoperative Multiple Metastases Treated with Apatinib / 中国肺癌杂志

Primary pulmonary synovial sarcoma is a rare pulmonary malignant tumor originated from primitive mesenchymal, which has short overall survival and poor prognosis. Related case reports are lacked at home and abroad. In recent years, the development of targeted therapy has brought remarkable benefits to cancer patients. Apatinib (Hengrui Pharmaceutical Co. Ltd, Jiangsu, People's Republic of China) is a small molecule vascular endothelial growth factor receptor 2 (VEGFR-2) inhibitor, which selectively inhibits VEGFR-2 and blocks the VEGF signal pathway, then strongly inhibiting the tumor angiogenesis. Apatinib has shown favorable therapeutic effect and acceptable toxicity on various tumors. Here we report a case of primary pulmonary synovial sarcoma with postoperative multiple metastases treated with apatinib, in order to provide a new considerable treatment.
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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.

Synovial Sarcoma Complicating Total Knee Arthroplasty

Synovial sarcoma is a relatively common periarticular soft tissue malignancy, occurring mostly in the extremities of younger patients. Occasionally, diversity in its clinical features may lead to misdiagnosis and inappropriate management. The authors report herein a unique case of a patient who underwent a primary total knee arthroplasty to treat osteoarthritis. During the operation, a mass was discovered but was attributed to synovitis. Biopsy revealed a rare intra-articular synovial sarcoma. The patient underwent reoperation with wide excision and endoprosthesis placement and is disease-free at the 8-year follow-up.

Primary pharynx synovial sarcoma: a case report / 华西口腔医学杂志

A case of primary pharynx synovial sarcoma was reported in this paper. A 15-year-old male patient experienced painless pharyngeal swelling that gradually proliferated for 1 month. Special examination showed an 8 cm × 4 cm × 3 cm tumor located in the left pharynx and the supratonsillar crypt. Imaging tests revealed an irregular mass on the left side of the oropharynx and an unclear boundary. Immunohistochemical examination yielded the following results: epithelial membrane antigen (+), cytokeratin (CK)19 (+), CD7(+), vimentin (+), CK10(-), E-cadherin (+), B-cell lymphoma-2 (-), CD2 (-), CD10 (-), CD138 (+), CD99 (+), leukocyte common antigen (+), and Ki-67 (20%+). This condition was pathologically diagnosed as primary pharynx synovial sarcoma.

One cases of nasal synovial sarcoma / 临床耳鼻咽喉头颈外科杂志

Synovial sarcoma is a rare tumour found in soft tissue; it is a mesenchymal spindle cell tumour that is not related to the synovial membrane. This tumour has a low incidence, and the most frequent place of occurrence is the lower extremities in young adults. Synovial sarcoma of the head and neck accounts for 3%-5% of sarcomas in this anatomical region. The tumor in the nasal cavity is less than 1%. The treatment of choice for synovial sarcoma of the head and neck is complete surgical excision of the tumour mass followed by adjuvant radiotherapy.

Synovial Sarcoma Mimicking Myositis Ossificans

A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis.

Synovial Sarcoma of the Extremities / 대한정형외과학회잡지

PURPOSE: Synovial sarcoma is an uncommon malignant soft tissue tumor mostly prevalent in young adults. Previous studies analyzing the prognostic factors have been limited due to the inclusion of heterogenous cohorts of patients with nonextremity and recurrent tumors. The purpose of this study was to determine the independent prognostic factors for the treatment methods associated with local recurrence and metastasis of primary synovial sarcoma localized to the extremities. MATERIALS AND METHODS: Between April 1999 and June 2014, a total of 79 patients were diagnosed with synovial sarcoma, of which 73 underwent wide excision at Kosin University Gospel Hospital and were followed-up for 60 months (24-72 months). The analyzed prognostic factors were treatment methods for local recurrence and metastasis during the postoperative follow-up period. The Cox regression model was used for multivariate analysis. RESULTS: For local recurrence according to the treatment methods, 9 recurrences occurred in 9 surgical treatments, 8 in 20 chemotherapy added surgical treatments, 13 in 20 radiotherapy added surgical treatments, and 4 in 24 chemotherapy with radiotherapy added surgical treatments. For metastasis according to the treatment methods, 9, 5, 12, and 2 metastases occurred respectively. Chemotherapy had statistical significance in multivariate analysis (p<0.0001). CONCLUSION: Adjuvant chemotherapy can be useful for the treatment of synovial sarcoma.

A Case of Transoral Endoscopic Excision of Recurred Synovial Sarcoma in Pyriform Sinus / 대한이비인후과학회지

Synovial sarcoma in head and neck is an extremely rare cancer. Symptoms may occur depending on the location of tumor. Synovial sarcoma is divided into two main phenotypes-biphasic and monophasic. Surgical excision including optimal resection margin is generally recommended as a standard treatment of synovial sarcoma, if the tumor is resectable. Standard adjuvant therapy of synovial sarcoma has not been established yet. Recently, we have experienced a case of recurrent synovial sarcoma in pyriform sinus without regional or distant metastasis after initial resection of tumor. We successfully performed endoscopic excision of recurred tumor and obtained clear resection margin. We followed up the patient without any adjuvant therapy for 2 years. We report this rare case with literature review.

Pulmonary Metastasectomy in Adult Patients with Synovial Sarcoma: A Single-Center Experience

BACKGROUND: This study assessed the efficacy of pulmonary metastasectomy for synovial sarcoma in adult patients. METHODS: Fifty patients, diagnosed with pulmonary metastasis from June 1990 to August 2010, were reviewed retrospectively. Twenty-eight patients underwent complete pulmonary metastasectomy, and their survival was evaluated. Age, sex, time to metastatic progression, laterality, number of tumors, size of largest nodule, and number of metastasectomies were analyzed as potential prognostic factors. RESULTS: In all, 29 patients underwent at least one pulmonary metastasectomy, and 51 resections were performed. One intraoperative mortality occurred, and the 5-year survival rate was 58.4%. Bilateral metastases and early metastatic progression were associated with poor survival in multivariate analyses. CONCLUSION: Surgical resection can be a good option for treating pulmonary metastasis in patients with synovial sarcoma. Repeated resection was feasible with low mortality and morbidity.

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.

Synovial Sarcoma Vulva: A Case Report.

Introduction: Contrary to its name, synovial sarcoma does not arise from the synovial membrane but from multi-potent stem cells and can present in any part of the body. Very few cases of vulval synovial sarcoma have been reported in the literature; we report on such a presentation. Th ese tumors can present as painless lumps, which must be completely excised to give the best prognosis. Th erefore the diagnosis of synovial sarcoma should always be kept in mind in the management of vulval masses, especially in young patients.

Solitary pulmonary nodule a rare case

A middle aged man presented with a solitary pulmonary nodule. He had a diagnostic dilemma as the CT guided FNAC was inconclusive. The frozen section exam reported it to be malignant so a lobectomy was performed for this lesion. The final histopathology report confirmed it to be a very rare tumor of extra skeletal synovial sarcoma