[Repeated Pericardial Effusion Leading to the Diagnosis of Synovial Sarcoma:Report of a Case].

The patient is a 76-year-old man. His chief complaint of chest pain led to a diagnosis of pericardial effusion of unknown cause, and pericardial drainage was performed. On the 30th day, chest pain appeared again. Echocardiography revealed a pericardial fluid reaccumulation and a substantial mass in the pericardial space. Surgical drainage was performed to find the cause. A hematoma/mass was present on the epicardium. The pericardial sac was filled with hematoma. The hematoma was removed, but part of the mass infiltrated close to the anterior descending branch of the left coronary artery, and removal of that part was abandoned. The intrapericardial hematoma and epicardium were submitted to pathology leading to the diagnosis of synovial sarcoma. The patient was discharged home 14 days after surgery.

Cardiogenic and obstructive shock: primary laryngeal synovial sarcoma with cardiac metastasis.

A man in his 20s with a history of laryngeal synovial sarcoma presented with dyspnoea. Imaging revealed a large right ventricular (RV) mass, which was resected, and histological analysis indicated synovial sarcoma recurrence. Within 1 month of RV mass resection, the tumour progressed with paratracheal metastasis. The lumen was nearly obliterated, and right ventricular outflow tract (RVOT) obstruction led to rapid deterioration with mixed cardiogenic and obstructive shock. We present a rare case of primary laryngeal synovial sarcoma metastasising to the heart.

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.

Association of SMC4 with prognosis and immune infiltration of sarcoma.

OBJECTIVE: This study was performed to explore the prognostic relevance of structural maintenance of chromosomes 4 (SMC4) in pan-cancer and explore the association between SMC4 and immune infiltration of sarcoma. RESULTS: Elevated expression of SMC4 was detected in cancer tissues compared to normal tissue, which was confirmed in synovial sarcoma tissues with immunohistochemistry (IHC). Additionally, higher expression of SMC4 was connected to worse outcomes of sarcoma, gastric cancer, breast cancer, liver cancer or ovarian cancer. Moreover, SMC4 was positively connected to immune cell infiltrates in sarcoma. In addition, infiltrating immune cell markers including monocyte, TAM, M1 and M2 presented different SMC4-associated immune infiltration patterns. CONCLUSION: The results from our study showed that SMC4 was positively related to the prognosis and immunological status of sarcoma. SMC4 could be a potential biomarker for prognosis and immune cell infiltrates in sarcoma. METHODS: Several databases including ONCOMINE, GEPIA, and Kaplan-Meier Plotter were adopted to explore the expression pattern of SMC4 in sarcoma, which was confirmed by IHC. The GEPIA and TIMER datasets were adopted to investigate the associations between SMC4 and prognosis in various cancers, especially in sarcoma.

Synovial sarcoma with intra-abdominal metastasis causing hemoperitoneum: a case-report.

Synovial sarcoma is a rare soft tissue sarcoma which frequently involves the upper or lower extremities. Soft tissue sarcomas including synovial sarcoma have a propensity to metastasize to the lungs, and there are very few reports of metastatic lesions in other locations.Here, we report a case of a 49-year-old patient who underwent neoadjuvant chemoradiation for an upper extremity synovial sarcoma and presented approximately 4 years later with abdominal pain and hemoperitoneum and was ultimately found to have metastatic synovial sarcoma involving the greater curvature of the stomach and surrounding peri-gastric soft tissue. We describe the multidisciplinary management of this complex patient presentation and propose that expanded surveillance imaging beyond that of the local tumor resection bed and the chest may be beneficial especially in tumors with high-risk features.

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

Pleuropulmonary synovial sarcoma (PPSS) is a primary malignancy of the lung, uncommon in pediatrics (prevalence: 0.1-0.5%) 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.

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.

Primary Pulmonary Synovial Sarcoma with Hemothorax: a Case Report.

Background: Synovial sarcoma is a rare and aggressive soft tissue malignancy most commonly arises from periarticular tissue of the extremities. Although several cases in the literature have reported different origins, primary pulmonary synovial sarcoma (PPSS) is an exceedingly rare and underrecognized entity, accounting for 0.5% of all lung malignancies. Clinical presentation includes chest pain, dyspnea, cough, and hemoptysis. The finding of hemothorax is a rare presentation and was barely reported in the literature. Due to its rarity and aggressive nature, the optimal treatment is unclear, while the mainstay remains surgical resection with chemo- and/or radiation therapy. Objective: To report a case of hemorrhagic effusion subsequently diagnosed with primary pulmonary synovial sarcoma with the main objective of enriching the literature regarding this rare malignancy. Case report: A 52-year-old male smoker with a background of coronary artery disease, hypertension, and diabetes mellitus was referred to our hospital. The patient presented with a history of chest pain, dyspnea, and massive right-sided pleural effusion. Laboratory investigations were unremarkable except for anemia. Chest x-ray showed a complete opacity on the right lower zone with right-sided pleural effusion. Thoracentesis was done and revealed hemorrhagic exudative effusion. Computed tomography (CT) scan showed a right heterogeneous lung mass compressing the medial segment of the middle lobe. Subsequently, the patient underwent bronchoscopy, which showed compression and edema on the right middle lobe bronchus with traces of blood coming from the right lower lobe. The patient underwent a right posterolateral thoracotomy, a fungating mass eroding the medial segment of the middle lobe was resected that was diagnosed as high-grade primary pulmonary synovial sarcoma. Radiotherapy was instituted. The patient died after two years due to recurrence. Conclusion: PPSS is an aggressive disease with poor prognostic outcomes, and Its presentation is almost similar to other lung malignancies. Meanwhile, there is no definitive management guideline, and most management depends on surgical resection if feasible with adjuvant chemo-radiation therapy.

Pain as Initial Presenting Symptom Is Associated With Delay to Seeking Medical Attention, Higher Risk of Relapse, and Shorter Survival in Patients With Early-Stage Extremity or Trunk Synovial Sarcoma.

BackgroundWhether the presenting symptom of pain vs mass impacts survival of early-stage synovial sarcoma is not known. Patients and MethodsThe authors investigated patients with early-stage extremity/trunk synovial sarcoma diagnosed from 2005 to 2017 at Kaiser Permanente Northern California for associations between the presenting symptom and survival. ResultsAmong 56 patients with early-stage extremity/trunk synovial sarcoma, median disease-free survival (DFS) was 20.3 months for the pain-only group (n = 19) vs 50.5 months for the mass ± pain group (n = 37) (p = 0.004), and median overall survival (OS) was 35.7 months vs 53.9 months (p = 0.009), respectively. Median DFS was 26.9 months for the pain ± mass group (n = 32) vs 48.6 months for the mass-only group (n = 24) (p = 0.047), whereas OS was not significantly different (49.6 vs. 53.6 months, p = 0.282). Pain at presentation was associated with a higher incidence of deep tumors and a higher risk of relapse. Cox regression model adjusting for age, sex, race, tumor location, tumor size, and wait-time to seek medical attention showed that pain at presentation was associated with 3-fold worse DFS and OS. ConclusionPain at presentation was an adverse risk factor for patients with early-stage extremity/trunk synovial sarcoma.

Synovial Sarcoma of the Foot as an Atypical Cause of Persistent Metatarsalgia: A Report of 3 Cases.

CASE: Three cases of atypical metatarsalgia are presented, all diagnosed with foot synovial sarcomas (SSs) at different stages of evolution, after a year of medical consultations. One case was treated with marginal excision without requiring bone excision; the second patient required amputation of the first ray; and the third patient, with advanced disease, required amputation through Chopart's joint. CONCLUSION: Metatarsalgia is a recurrent reason for consultation in orthopaedics. Even so, patients with persistent symptoms should be studied further in depth. Computed tomography or magnetic resonance imaging can detect tumor pathology, such as SS, of insidious development.

Right atrial synovial sarcoma with thrombocytopenia: A deceptive presentation.

INTRODUCTION: Primary cardiac synovial sarcoma is an exceedingly rare soft tissue malignant tumor, involving either the pericardium or chambers, with a striking male predominance, prevalently seen in the fourth decade. These tumours most commonly present as breathlessness on exertion. These patients readily exhibit gastrointestinal and systemic symptoms but sometimes the presentation can be fallacious. METHODS: A 37-year old male presented with fever, breathlessness on exertion and hematuria who was managed for dengue feverat another hospital. Patient also had features of right heart failure and congestive hepatomegaly. Patient was evaluated thoroughlyand diagnosed as right atrial tumour mass for which surgical resection was done. RESULTS: The tumour mass was diagnosed as right atrial biphasic synovial sarcoma after immunohistochemistry. Patient also had thrombocytopenia which was due to increased consumption of platelets. CONCLUSIONS: Right heart biphasic synovial sarcoma, arising from the tricuspid valve in association with thrombocytopenia and right heart failure is an extremely rare entity and that can have a deceptive presentation.

Synovial sarcoma with overwhelming glandular (adenocarcinoma-like) component: A case report and review of the literature.

Synovial sarcoma is an uncommon tumor of soft tissue, characterized by a specific SS18-SSX1/2/4 fusion gene. It is generally a lesion composed of monomorphic spindle cells, and can sometimes show variable epithelial differentiation. Here, we present the case of a young woman with a synovial sarcoma of the abdominal wall that showed an overwhelming (>90 %) epithelial glandular component mimicking adenocarcinoma, and only rare spindled areas. The diagnosis was confirmed by detection of targeted fusion transcripts associated with synovial sarcoma. We review the literature pertaining to synovial sarcoma, and we show that this case is only the sixth molecularly proven epithelial predominant synovial sarcoma in the literature. This report emphasizes the importance of molecular approaches in modern soft tissue pathology. Recognition of synovial sarcoma with predominant glandular component is imperative in order to avoid misdiagnosis of the tumor as metastatic adenocarcinoma, another type of sarcoma with epithelial differentiation, or a carcinoma with a sarcomatous component (sarcomatoid carcinoma), all of which have markedly different clinical management.

Primary renal synovial sarcoma presenting with a retroperitoneal bleed.

Synovial sarcomas are most commonly localised in extremities, especially in the lower thigh and knee areas. Comprising less than 1% of all malignancies, retroperitoneal synovial sarcoma is very rare with primary synovial sarcoma of the kidney being even more infrequent and difficult to diagnose. We describe a case report of a renal synovial sarcoma in a young adult who was initially managed as a case of Wunderlich's syndrome secondary to what was believed to be a ruptured renal angiomyolipoma. After biopsy confirmation, the patient was eventually managed with neo-adjuvant chemotherapy followed by a right radical nephrectomy and right hepatectomy. Despite its rarity, synovial sarcoma should be considered as differential diagnosis of a bleeding retroperitoneal soft tissue mass detected in young adults.

Synovial sarcoma of the spine in the lumbar vertebral body: A rare case report.

RATIONALE: Synovial sarcoma (SS) is a soft tissue neoplasm that rarely occurs in the vertebral body and should be considered in the differential diagnosis in patients with SS and vertebral lesions. SS often presents as a painless mass in the spine, which may undergo slow enlargement, resulting in sustained symptoms of neurologic deficit and pain. Due to the difficulty in differentiating between SS from other soft tissue tumors and metastatic tumors, careful histological confirmation is required for definite diagnosis. Furthermore, due to its malignancy, the appropriate treatment procedure for SS should be carefully considered. PATIENT CONCERNS: A 56-year-old female patient had low back pain. Radiological examination revealed bony erosion of the L-2 vertebral body, and no soft tissue mass around the lumbar spine. DIAGNOSIS: Histopathological and immunohistochemical examination revealed SS. INTERVENTIONS: The initial treatment of posterior laminectomy decompression and percutaneous vertebro plasty (PVP) was performed, however, this initial treatment course was inappropriate, but she eventually underwent L-2 complete resection and internal fixation. After the second surgery, she was treated by external beam radiation therapy. OUTCOMES: operation radiotherapy was finally performed. No local recurrence in L-2 vertebral body or distant metastasis was found at 1-year follow up postoperation; the neurologic symptom gradually relieved, and no other symptom was noted. And no local recurrence in L-2 vertebral body and distant metastasis was found in 1 year follow up postoperation. LESSONS: Solitary spinal SS is extremely rare. Early surgery for total resection and adjuvant radiotherapy/chemotherapy should be emphasized.

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.