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OBJECTIVE: In this study, we examined the reason and prognosis of unplanned excision on synovial sarcoma. METHODS: We retrospectively analyzed 54 patients diagnosed with synovial sarcoma between March 2013 and February 2021, including 26 cases of unplanned excision surgery. Patients were divided into two groups based on whether they underwent unplanned excision. Then, factors such as gender, age, tumor size, tumor location, American Joint Committee on Cancer (AJCC) staging, unplanned excision, time of onset, duration of disease, radiotherapy, chemotherapy, amputation, local recurrence factors, and death were statistically evaluated. RESULTS: The results of a multivariate analysis revealed that the AJCC staging is an independent factor for patient prognosis. When patients were divided into two groups, those who had undergone unplanned excision and those who had not, statistical analysis revealed that there was no difference of survival between two groups, but tumor size and AJCC staging had statistical difference. To further explore the influences of unplanned excision, we performed propensity score analysis with 1:1 matching using the nearest neighbor matching method to balance the covariates between the two groups. There was no difference of survival between two groups after propensity score matching. CONCLUSION: Unplanned excision is commonly performed in synovial sarcoma and do not impact the prognosis after extensive resection.
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Synovial sarcoma (SS) and solitary fibrous tumor (SFT) are entities with considerable morphological and immunohistochemical similarities that sometimes show a non-confirmatory profile (TLE1 negative, CD34 and focal or negative STAT6 and lack of specific fusion IHC markers), in which the utility ultrastructure is unknown. A cross-sectional, retrospective, analytical, nonexperimental study was carried out by the Department of Pathology of the National Cancer Institute of Mexico (INCan) e from January 1, 2009 to December 31, 2018. With 17 SFT cases with diffuse or focal CD34 and STAT6 positivity and 18 cases of SS with positive FISH molecular test t(X:18) breakapart were studied by electron microscopy of fresh glutaraldehyde fixed or paraffin-embedded tissue. The ultrastructural findings with a significant difference present in the SS were tandem tight junctions, desmosomes and abundance of dilated rough endoplasmic reticulum (RER) cisternae (p < 0.001, 0.003, and 0.001, respectively); while in the (SFT) the presence of abundant glycogen, basal lamina, long and slender cytoplasmic processes, pinocytic vesicles, hemidesmosomes, and/or dense plaques, collagen skein, and microvilli-like buds (p = 0.028, 0.005, and <0.001 for the last five). We then infer that the five distinctive markers of the SFT are the collagen skeins intermingled with cellular processes in a shape of "squid can," and the pinocytic vesicles as they were not observed in any case of SS. Conversely, tandem junctions were not found in any SFT case. Although the presence of multivesicular buds in the SFT was not significant, it had not been previously described.
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Sarcoma Sinovial , Tumores Fibrosos Solitários , Humanos , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/ultraestrutura , Sarcoma Sinovial/ultraestrutura , Sarcoma Sinovial/patologia , Adulto , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , México , Estudos Transversais , Biomarcadores Tumorais , Idoso , Adulto Jovem , Diagnóstico DiferencialRESUMO
Resumen El sarcoma sinovial primario del pericardio es un tumor muy raro y de mal pronóstico y se sabe poco en cuanto al manejo terapéutico. Presentamos el caso de una paciente de 51 años a quien se le realizó resección quirúrgica incompleta, quimioterapia y radioterapia. Hasta donde sabemos, este es el primer caso de un sarcoma sinovial primario de pericardio que luego de operado se mantuvo asintomático durante 5 años hasta que en una TAC de control se le detectaron metástasis cardiacas que comprometían las cavidades derechas y con quimioterapia, la ecocardiografía demostró la reso lución completa de las mismas.
Abstract Primary pericardial synovial sarcoma is an extraor dinarily very rare tumor with a poor prognosis, and little is known about its therapeutic management. We describe the case of a 51-year-old woman patient who underwent incomplete surgical resection, chemotherapy, and radiotherapy. To the best of our knowledge, no pri mary pericardial synovial sarcoma has been described which, after surgery, remains asymptomatic for 5 years, and until a control CT scan detects cardiac metastases that compromised the lumen of the right cavities and with chemotherapy, echocardiography demonstrated complete resolution of cardiac metastases.
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Primary pericardial synovial sarcoma is an extraordinarily very rare tumor with a poor prognosis, and little is known about its therapeutic management. We describe the case of a 51-year-old woman patient who underwent incomplete surgical resection, chemotherapy, and radiotherapy. To the best of our knowledge, no primary pericardial synovial sarcoma has been described which, after surgery, remains asymptomatic for 5 years, and until a control CT scan detects cardiac metastases that compromised the lumen of the right cavities and with chemotherapy, echocardiography demonstrated complete resolution of cardiac metastases.
El sarcoma sinovial primario del pericardio es un tumor muy raro y de mal pronóstico y se sabe poco en cuanto al manejo terapéutico. Presentamos el caso de una paciente de 51 años a quien se le realizó resección quirúrgica incompleta, quimioterapia y radioterapia. Hasta donde sabemos, este es el primer caso de un sarcoma sinovial primario de pericardio que luego de operado se mantuvo asintomático durante 5 años hasta que en una TAC de control se le detectaron metástasis cardiacas que comprometían las cavidades derechas y con quimioterapia, la ecocardiografía demostró la resolución completa de las mismas.
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Neoplasias Cardíacas , Sarcoma Sinovial , Neoplasias do Timo , Feminino , Humanos , Pessoa de Meia-Idade , Sarcoma Sinovial/diagnóstico por imagem , Sarcoma Sinovial/cirurgia , Ecocardiografia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/terapiaRESUMO
Background: Soft-tissue sarcomas are a rare and diverse group of neoplastic lesions. They represent only 1% of malignant tumors in adults and 15% in children. Synovial sarcoma (SS) is a type of soft-tissue sarcoma, accounting for 5-10% of cases, and commonly affecting extremities. Diagnosis, treatment, and prognosis remain challenging especially when localized in uncommon areas, such as intracranial lesions. Case Description: A 13-year-old male patient with a clinical history of neurofibromatosis Type I (NF1) presenting holocranial headache with jet vomiting and apathy 2 days before admission, without neurological deficits and/or focal findings. On magnetic resonance imaging: an extra-axial infiltrative lesion with contrast uptake at the base of the skull in the olfactory groove topography. After total tumor resection, the anatomopathological examination showed monophasic SS. The patient returned after 6 months with similar symptoms, and the lesion recurred and was reoperated. Unfortunately, 7 months after the second surgery, the patient died. Conclusion: SS can occur extraarticulously and with a variable clinical presentation and poor prognosis despite adjuvant therapies with radiotherapy and chemotherapy. In individuals with clinical history of NF1, there is still no direct correlation between the two manifestations, although current descriptions are suggestive of a possible interaction.
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Background: Synovial sarcoma (SS) is part of soft tissue sarcomas (STS). An incidence between 5% to 10% is estimated. The origin is mesenchymal mainly affecting the extremities. Being even rarer at the chest level and vertebral body, representing around 1%. Histologically, it consists of 3 variants: monophasic, biphasic, and poorly differentiated. Surgical resection is a priority when it comes to multidisciplinary management. The prognosis of patients with SS over the years has improved markedly. Purpose: Understand and evaluate the multidisciplinary management of SS considering that the SS has a lowe prevalence and highly malignancy. Study Design: We present a case of a 31-year-old male who has a history of monophasic synovial sarcoma diagnosed in 2019 and underwent surgery. Patient came back after two years without symptoms and posterior to a control MRI we observed a local recurrence of SS. Methods: The literature was reviewed with a focus on best clinical and surgical strategy for recurrence of SS. Results: The patient recovered well with return to his normal daily activities. The review of the literature shows us the importance of the multidisciplinary management for the optimal clinical and surgical approach of SS recurrence. Conclusions: SS represents a unique variant of STS, with malignant and metastatic potential. Being a rare pathology, an adequate multidisciplinary management is essential when providing optimal care for the patient.
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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.
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Humanos , Masculino , Criança , Pneumotórax/complicações , Pneumotórax/etiologia , Sarcoma Sinovial/complicações , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patologia , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/diagnóstico , Tosse , Pulmão/patologiaRESUMO
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.
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Neoplasias Pulmonares , Pneumotórax , Sarcoma Sinovial , Masculino , Adolescente , Adulto Jovem , Humanos , Criança , Pneumotórax/etiologia , Pneumotórax/complicações , Sarcoma Sinovial/complicações , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patologia , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/diagnóstico , Tosse , Pulmão/patologiaRESUMO
INTRODUCTION: Synovial Sarcoma is a rare malignancy that accounts between 8 and 10% of soft tissue neoplasms, with the highest presentation rate in extremities, an extremely uncommon condition in head and neck. CLINICAL FINDINGS: We present a case of an 18-year-old male with synovial sarcoma situated at hypopharynx who underwent surgical resection and postoperative radiotherapy. CONCLUSION: Synovial Sarcoma represents a rare head and neck malignancy with challenging diagnostic approach due to its frequency and nonspecific clinical manifestations. Surgical treatment must assure good free margins. Adjuvant radiotherapy has a positive impact in local recurrence and survival.
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PURPOSE OF REVIEW: To update epidemiological, diagnostic, and therapeutic information on primary synovial sarcoma of the kidney. RECENT FINDINGS: A total of 96 studies were analyzed; age at presentation was 38.6±14.2 years, predominant location of tumor was right kidney; frequent reported symptoms at diagnosis were hematuria and pain. For definitive diagnosis, cytogenetic technique was used. Detected oncogene was available in 37.8% cases with fusion of SS18-SSX in most patients. Surgery is treatment of choice, with adjuvant chemotherapy; most frequently ifosfamide-based associated with doxorubicin or epirubicin. Overall median survival was 34 months. Mortality was 29% of the cases which reported death and the recurrence rate was 39.8%. Risk of death was increased in patients with metastases at diagnosis Primary RSS occurs more often in young men. RSS often presents with symptoms and in an advanced stage. Surgical treatment is the most commonly used and chemotherapy for advanced or recurrent treatment.
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Neoplasias Renais/diagnóstico , Neoplasias Renais/terapia , Recidiva Local de Neoplasia , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/terapia , Hematúria/etiologia , Humanos , Neoplasias Renais/epidemiologia , Neoplasias Renais/patologia , Recidiva Local de Neoplasia/patologia , Proteínas de Fusão Oncogênica/genética , Dor/etiologia , Sarcoma Sinovial/epidemiologia , Sarcoma Sinovial/secundário , Taxa de SobrevidaRESUMO
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.
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Humanos , Masculino , Criança , Sarcoma Sinovial/cirurgia , Sarcoma Sinovial/diagnóstico , Neoplasias Pulmonares/cirurgia , Neoplasias Pulmonares/diagnóstico , Broncoscopia , Radiografia Torácica , Sarcoma Sinovial/patologia , Neoplasias Pulmonares/patologiaRESUMO
BACKGROUND: Synovial sarcoma (SS) is an aggressive soft-tissue sarcoma with a poor prognosis owing to its resistance to radiation and chemotherapy. Thus, novel therapeutic strategies for SS are urgently required. Anlotinib, a new oral tyrosine kinase inhibitor, is designed to primarily inhibit multi-targets in vasculogenesis and angiogenesis. This study was designed to characterize its antitumor efficacy and possible mechanism in patients with advanced refractory synovial sarcoma. METHODS: Anlotinib's antitumor effect was evaluated in vivo and vitro. Downstream targets of anlotinib in treating synovial sarcoma were analyzed through microarray assay. Cell proliferation and apoptosis analyses were performed to evaluate the impact of candidate downstream gene depletion in synovial sarcoma cells. Microarray assay were carried out to investigate potential signal network related with candidate downstream gene. RESULTS: Anlotinib significantly suppresses synovial sarcoma proliferation in PDTX model and cell lines. Additionally, GINS1 (also named as PSF1, Partner of SLD Five 1), rather than other conventional gene target, was demonstrated to be a vital target of anlotinib's antitumor effect in synovial sarcoma through microarray assay. Expression of GINS1 was remarkably higher in synovial sarcoma tumor samples and related with poor outcome. Knockdown of GINS1 expression could remarkably inhibit proliferation and promote apoptosis in vitro. Meanwhile, through microarray assay, CITED2, EGR1, SGK1 and SPP1 were identified and further validated by qPCR/WB as downstream targets of GINS1. CONCLUSION: Anlotinib might suppress proliferation of SS through a novel downstream GINS1-regulated network which plays a vital function in SS proliferation and also demonstrated that targeting the GINS1-regulated signal pathway could be a potential strategy for management of SS.
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Neoplasias Ósseas/tratamento farmacológico , Proteínas de Ligação a DNA/efeitos dos fármacos , Indóis/uso terapêutico , Proteínas de Neoplasias/efeitos dos fármacos , Inibidores de Proteínas Quinases/uso terapêutico , Quinolinas/uso terapêutico , Sarcoma Sinovial/tratamento farmacológico , Apoptose/efeitos dos fármacos , Neoplasias Ósseas/genética , Proliferação de Células/efeitos dos fármacos , Proteínas de Ligação a DNA/genética , Proteínas de Ligação a DNA/metabolismo , Progressão da Doença , Proteína 1 de Resposta de Crescimento Precoce/efeitos dos fármacos , Proteína 1 de Resposta de Crescimento Precoce/genética , Proteína 1 de Resposta de Crescimento Precoce/metabolismo , Técnicas de Silenciamento de Genes , Humanos , Proteínas Imediatamente Precoces/efeitos dos fármacos , Proteínas Imediatamente Precoces/genética , Proteínas Imediatamente Precoces/metabolismo , Proteínas de Neoplasias/genética , Proteínas de Neoplasias/metabolismo , Osteopontina/efeitos dos fármacos , Osteopontina/genética , Osteopontina/metabolismo , Análise Serial de Proteínas , Proteínas Serina-Treonina Quinases/efeitos dos fármacos , Proteínas Serina-Treonina Quinases/genética , Proteínas Serina-Treonina Quinases/metabolismo , RNA Mensageiro/análise , Proteínas Repressoras/efeitos dos fármacos , Proteínas Repressoras/genética , Proteínas Repressoras/metabolismo , Sarcoma Sinovial/genética , Transativadores/efeitos dos fármacos , Transativadores/genética , Transativadores/metabolismoRESUMO
ABSTRACT Introduction: Synovial sarcomas are rare and aggressive neoplasms located in the head and neck region and usually occurs in young adults. Presentation of case: This report presents a case of synovial sarcoma in a 15-year-old male patient who sought medical treatment for painful symptoms and associated dysphagia. The lesion was nodular, extensive, localized in the parotid region, and extended to the left cervical region. The patient was treated in a referral hospital with a treatment protocol that initially included chemotherapy for six months and surgery to attempt to excise the lesion, but the surgery was ineffective because removal could have damaged important vital structures. The Computed Tomography scan showed a hypodense area with diffuse growth and no involvement of the facial bones and the histopathological analysis revealed pleomorphic and oval spindle cells with rounded epithelial cells that formed nests surrounded by fibrous tissue. The Immunohistochemistry analysis was conclusive for the diagnosis of a high-grade SS in the parotid and left cervical regions. The medical team opted for palliative treatment with cervical radiotherapy. The patient remained hospitalized for four months after the surgery and died after 15 months since the diagnosis for compromise of airway by fast tumor growth. Conclusion: The synovial sarcoma, when diagnosed late may reduce the survival of patients because of the complications that tumor growth can bring to the prognosis and quality of life.
RESUMO Sarcomas sinoviais são neoplasias raras e agressivas, localizadas na região da cabeça e pescoço e geralmente ocorrem em adultos jovens. Relato do caso: este relato apresenta um caso de sarcoma sinovial em um paciente de 15 anos que procurou tratamento médico para sintomas dolorosos e associados à disfagia. A lesão era nodular, extensa, localizada na região da parótida e estendida à região cervical esquerda. O paciente foi tratado em um hospital de referência com um protocolo de tratamento que inicialmente incluiu quimioterapia por seis meses e cirurgia para tentar excisar a lesão, mas a cirurgia foi ineficaz porque a total remoção do tumor poderia comprometer estruturas vitais importantes. A tomografia computadorizada mostrou uma área hipodensa com crescimento difuso, sem envolvimento dos ossos faciais, e a análise histopatológica revelou células fusiformes pleomórficas e ovais, com células epiteliais arredondadas formando ninhos rodeados por tecido fibroso. A análise imunohistoquímica foi conclusiva para o diagnóstico de um sarcoma sinovial de alto grau na região cervical parotídea esquerda. A equipe médica optou pelo tratamento paliativo com radioterapia cervical. O paciente permaneceu hospitalizado por quatro meses após a cirurgia e faleceu 15 meses após o diagnóstico, devido à obstrução das vias aéreas pelo rápido crescimento tumoral residual. O sarcoma sinovial, quando diagnosticado tardiamente pode reduzir a sobrevida dos pacientes por causa de complicações que o crescimento do tumor pode trazer para o prognóstico e qualidade de vida.
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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.
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Humanos , Adulto , Feminino , Neoplasias Pulmonares/cirurgia , Neoplasias Pulmonares/secundário , Neoplasias de Tecidos Moles/patologia , Sarcoma Sinovial/cirurgia , Sarcoma Sinovial/secundário , Coxa da PernaRESUMO
El sarcoma sinovial es una entidad morfológica, clínica y genéticamente distinta y puede tener cualquier otra ubicación. No existe una terapia estandarizada. Generalmente el tratamiento es quirúrgico, con resección radical de la lesión, aplicándose en algunos casos radioterapia adyuvante. Los sarcomas sinoviales han mostrado quimiosensibilidad a la ifosfamida con una respuesta aproximada del 24%. Se encuentran presentes más frecuentemente en las extremidades, siendo su diseminación metastásica a ganglios linfáticos regionales y/o pulmón, hígado, hueso y cerebro. Se trata de un caso clínico: paciente femenino de 40 años de edad, portadora de tumor en miembro inferior derecho, en el tercio medio del muslo, de 15x10 cm de diámetro, que compromete el vasto anterior, lateral y posterior del cuádriceps derecho, con irrupción de la aponeurosis y extensión al tejido celular subcutáneo. Por medio de la inmunohistoquímica se observó inmunomarcaje de las células neoplásicas con EMA, QAE1/AE3, vimentina, CD99, CD34 y Bcl2, como diagnóstico de sarcoma sinovial monofásico. Para el tratamiento se procedió a desarticulación de cadera, postoperatorio sin complicaciones y evolución satisfactoria. Se refiere al servicio de oncología médica para tratamiento coadyuvante. Se concluye que los procedimientos quirúrgicos en el tratamiento de tumores en extremidades se divide en resección amplia del tumor con preservación del miembro mediante endoprotesis, y la desarticulación, no existiendo evidencias clínicas que soporten la ventaja sobre cualquier otra opción terapéutica, sin embargo, el objetivo principal del procedimiento oncológico es controlar el tumor para disminuir la recurrencia y mejorar la supervivencia del paciente.
Synovial sarcoma is a clinically and genetically distinct morphological entity, and can be found in any location. There is no standard therapy. Treatment is usually surgical; with radical resection of the lesion, applying, in some cases, adjuvant radiotherapy. Synovial sarcomas have shown chemosensitivity to ifosfamide with an approximate 24% response. They are most often present in the extremities causing metastatic spread to regional lymph nodes and/or lung, liver, bone and brain. It is a clinical case: a female 40 year old patient with a tumor in right leg, on the middle third of the thigh, 15x10 cm in diameter, that involves the anterior, lateral and posterior vastus of the right quadriceps, with emergence of aponeurosis and extension to the subcutaneous tissue. Through immunohistochemical test, immuno-labeling in neoplastic cells with EMA, QAE1/AE3, vimentin, CD99, CD34 and Bcl2, as diagnosis of monophasic synovial sarcoma, was observed. For treatment, hip disarticulation was carried out, no postoperative complications and satisfactory outcome. The patient is referred to medical oncology service for adjuvant treatment. We conclude that surgical procedure in the treatment of tumors in extremities is divided into wide resection of the tumor with preservation of the member by endoprosthesis, and disarticulation amputees, with no clinical evidence to support the benefit of any other treatment option, however, the main purpose of the oncologic procedure is to control the tumor recurrence and improve patient survival.
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Primary synovial sarcoma (SS) of the kidney is a rare neoplasm and its presenting features are similar to other common renal tumors, making early diagnosis difficult. To date, few cases have been reported in the literature. Primary renal SSs can exist in either a monophasic or a biphasic pattern, the former being more common and tending to have a better prognosis than the biphasic variant. Herein we describe a case of primary renal SS that was diagnosed based on histopathology and immunohistochemistry after radical nephrectomy. Fusion gene product analysis was also done by FISH and RT-PCR. Patient follow-up and literature review are presented, focused on systemic therapy. We highlight that these tumors should be correctly diagnosed as clinical results and specific treatment are distinct from primary epithelial renal cell carcinoma. Adjuvant chemotherapy should be tailored for each patient in the management of disease, although its role still remains unclear.
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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.
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Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/patologia , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/genética , Sarcoma Sinovial/patologia , Diagnóstico Diferencial , Imuno-Histoquímica , Proteínas de Fusão Oncogênica , Reação em Cadeia da Polimerase , Translocação GenéticaRESUMO
We describe a rare case of primary pleural synovial sarcoma in a 27-year-old man with a 4-month history of dry cough and left-sided chest pain. A CT scan showed a large cystic mass in the left pleural cavity. The patient underwent two video-assisted thoracoscopic biopsies and the diagnosis of synovial sarcoma of the pleura was established. After neoadjuvant chemotherapy, which resulted in a partial response, the tumor was completely resected with extrapleural pneumonectomy. Pathological findings showed less than 5% of viable cancer and free surgical margins. The patient is clinically well 24 months after surgery, with no evidence of recurrent disease.