RESUMEN
INTRODUCTION: Synovial sarcoma is an unusual tumor with an incidence of 1-3 cases per million. It is more frequent in teenagers and young adults under 30. It develops anywhere, but the extremities are the most frequent place of appearance (80% extremities, 20% other locations: 8% trunk, 7% retroperitoneal/abdominal, 5% head and neck). Oncological results are different depending on the study. Survival rate free of local recurrence, survival rate free of events and global survival rate vary upon published studies. The same happens with the disease's prognostic factors. METHODS: The objective was to analyze a group of 43 patients with diagnosis of synovial sarcoma of the extremities treated surgically and determine (1) global survival rate, (2) survival rate free of events, (3) local recurrence rate and (4) oncological risk factors. RESULTS: The global survival rate at 2 years was 90% (IC95%: 76 - 96%) and 67% (IC95%: 49-80%) at 5 years. The survival rate free of events at 2 years was 68% (IC95% 51-80%) and 48% (IC95% 32-52%) at 5 years. The recurrence rate at 2 years was 9% (IC95% 3-25%) and 25% (IC95% 13-46%) at 5 years. The histological grade and metastatic presence were bad prognostic factors. DISCUSSION: We can conclude that our oncological results are in line with those published in previous series and that there were two factors associated with poor prognosis.
Introducción: El sarcoma sinovial es un tumor raro (incidencia de 1-3 casos por millón). Es más frecuente en adolescentes y adultos menores de 30 años. Se desarrolla en cualquier parte del cuerpo, siendo, las extremidades el lugar más frecuente de aparición (80% extremidades y 20% otras localizaciones: 8% tronco, retroperitoneal/ abdominal 7%, cabeza y cuello 5%). Los resultados oncológicos de los pacientes con sarcoma sinovial son disímiles. La tasa de supervivencia libre de recurrencia local, la supervivencia libre de eventos y la supervivencia global varían entre las series publicadas. Lo mismo sucede con los factores pronósticos de la enfermedad. Métodos: El objetivo fue analizar un grupo de 43 pacientes con diagnóstico de sarcoma sinovial de las extremidades tratados quirúrgicamente, y determinar (1) tasa de supervivencia global, (2) tasa de supervivencia libre de eventos, (3) tasa de recurrencia local y (4) factores de riesgo oncológicos. Resultados: La supervivencia global a los 2 años fue 90% (IC95%: 76-96%), y 67% (IC95%: 49-80%) a los 5 años. La supervivencia libre de eventos a los 2 años fue 68% (IC95% 51-80%) y a los 5 años 48% (IC95% 32-52%). El riesgo de recurrencia local a 2 años fue 9% (IC95% 3-25%) y a los 5 años 25% (IC95% 13-46%). Los factores de mal pronóstico oncológico fueron el grado histológico y la presencia de metástasis. Discusión: Podemos concluir que nuestros resultados oncológicos se asemejan a las series publicadas y que en nuestra serie hubo dos factores de mal pronóstico.
Asunto(s)
Sarcoma Sinovial , Adolescente , Adulto Joven , Humanos , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patología , Pronóstico , Extremidades/patología , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/patología , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
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.
Asunto(s)
Humanos , Masculino , Niño , Neumotórax/complicaciones , Neumotórax/etiología , Sarcoma Sinovial/complicaciones , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patología , Neoplasias Pulmonares/complicaciones , Neoplasias Pulmonares/diagnóstico , Tos , Pulmón/patologíaRESUMEN
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.
Asunto(s)
Neoplasias Pulmonares , Neumotórax , Sarcoma Sinovial , Masculino , Adolescente , Adulto Joven , Humanos , Niño , Neumotórax/etiología , Neumotórax/complicaciones , Sarcoma Sinovial/complicaciones , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patología , Neoplasias Pulmonares/complicaciones , Neoplasias Pulmonares/diagnóstico , Tos , Pulmón/patologíaRESUMEN
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.
Asunto(s)
Neoplasias Renales/diagnóstico , Neoplasias Renales/terapia , Recurrencia Local de Neoplasia , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/terapia , Hematuria/etiología , Humanos , Neoplasias Renales/epidemiología , Neoplasias Renales/patología , Recurrencia Local de Neoplasia/patología , Proteínas de Fusión Oncogénica/genética , Dolor/etiología , Sarcoma Sinovial/epidemiología , Sarcoma Sinovial/secundario , Tasa de SupervivenciaRESUMEN
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.
Asunto(s)
Humanos , Masculino , Niño , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/diagnóstico , Neoplasias Pulmonares/cirugía , Neoplasias Pulmonares/diagnóstico , Broncoscopía , Radiografía Torácica , Sarcoma Sinovial/patología , Neoplasias Pulmonares/patologíaAsunto(s)
Humanos , Masculino , Adulto , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/diagnóstico , Neoplasias Renales/cirugía , Neoplasias Renales/diagnóstico , Inmunohistoquímica/métodos , Tomografía Computarizada por Rayos X , Ultrasonografía , Resultado del Tratamiento , Laparoscopía , Nefrectomía/métodosAsunto(s)
Neoplasias Maxilares/diagnóstico , Sarcoma Sinovial/diagnóstico , Biopsia , Terapia Combinada , Diagnóstico Diferencial , Diagnóstico por Imagen , Humanos , Inmunohistoquímica , Masculino , Neoplasias Maxilares/patología , Neoplasias Maxilares/terapia , Sarcoma Sinovial/patología , Sarcoma Sinovial/terapia , Adulto JovenRESUMEN
Pediatric tumors are heterogenous and can be quite varied in appearance. However, those in the infamous "small round blue-cell tumor" group, with their hyperchromatic nuclei and small amount of cytoplasm can be challenging, and their diagnosis and prognostication require cost-efficient and focused immunohistochemistry and ancillary testing. Ideally, ample material should be obtained for routine histology and ancillary testing, including immunohistochemistry, fluorescent in situ hybridization, fresh tissue for cytogenetic studies, and snap-frozen tumor for DNA/RNA extraction both for routine molecular testing (ie, reverse-transcription PCR studies), as well as future research study protocols (genome wide studies, targeted gene sequencing). This review focuses on the main pediatric tumors with emphasis on immunophenotype, keeping in mind that a directed panel approach yields the highest yield with combination of clinical history, histologic features, and ancillary molecular testing.
Asunto(s)
Biomarcadores de Tumor/genética , Inmunofenotipificación , Fenotipo , Niño , Tumor Desmoplásico de Células Pequeñas Redondas/diagnóstico , Tumor Desmoplásico de Células Pequeñas Redondas/genética , Tumor Desmoplásico de Células Pequeñas Redondas/patología , Fibrosarcoma/diagnóstico , Fibrosarcoma/genética , Fibrosarcoma/patología , Hepatoblastoma/diagnóstico , Hepatoblastoma/genética , Hepatoblastoma/patología , Humanos , Inmunohistoquímica , Hibridación Fluorescente in Situ , Neuroblastoma/diagnóstico , Neuroblastoma/genética , Neuroblastoma/patología , Blastoma Pulmonar/diagnóstico , Blastoma Pulmonar/genética , Blastoma Pulmonar/patología , Rabdomiosarcoma/diagnóstico , Rabdomiosarcoma/genética , Rabdomiosarcoma/patología , Sarcoma de Parte Blanda Alveolar/diagnóstico , Sarcoma de Parte Blanda Alveolar/genética , Sarcoma de Parte Blanda Alveolar/patología , Sarcoma de Ewing/diagnóstico , Sarcoma de Ewing/genética , Sarcoma de Ewing/patología , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/genética , Sarcoma Sinovial/patología , Tumor de Wilms/diagnóstico , Tumor de Wilms/genética , Tumor de Wilms/patologíaAsunto(s)
Imagen por Resonancia Magnética/métodos , Neurilemoma/patología , Neoplasias de los Tejidos Blandos/patología , Bolsa Sinovial/patología , Bursitis/diagnóstico , Diagnóstico Diferencial , Femenino , Articulación de la Cadera/patología , Histiocitoma Fibroso Maligno/diagnóstico , Humanos , Persona de Mediana Edad , Neoplasias de la Vaina del Nervio/diagnóstico , Sarcoma Sinovial/diagnósticoRESUMEN
Synovial sarcomas of the hypopharynx are a rare form of soft tissue sarcomas. In the head and neck, they are most commonly found on the pharyngeal wall in areas that do not have synovial cells. We review a case of a 23 year-old pregnant female who presented to our hospital with acute airway obstruction due to a large hypopharynx lesion. She underwent emergency tracheotomy and tumor debulking. Pathology revealed synovial sarcoma. After the procedure the patient went into labor delivering a 29 weeks premature child. Six weeks after being discharged from the hospital the patient had a partial pharyngectomy performed to excise the remnant base of the tumor. She has had no recurrence at the 30-month post-operative period.
Asunto(s)
Neoplasias Hipofaríngeas , Complicaciones Neoplásicas del Embarazo , Sarcoma Sinovial , Femenino , Humanos , Neoplasias Hipofaríngeas/diagnóstico , Neoplasias Hipofaríngeas/cirugía , Embarazo , Complicaciones Neoplásicas del Embarazo/diagnóstico , Complicaciones Neoplásicas del Embarazo/cirugía , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/cirugía , Adulto JovenRESUMEN
BACKGROUND: Primary synovial sarcomas of the lung are extremely rare, constituting 0.1% to 0.5% of lung cancers. The first case was reported by Gaertner in 1996. CLINICAL CASE: We present the case of a 15-year-old female who presented with cough, white-colored secretions, right-sided chest pain and progressive dyspnea. Physical examination revealed increased exertion during breathing with no cyanosis. The presence of right scapular pulmonary condensation syndrome was observed. Chest x-ray demonstrated homogeneous opacity occupying the lower two thirds of the right hemithorax. Posterolateral thoracotomy with right lower and medium lobectomy was performed. Because there was evidence of upper lobe tumor, it was decided to carry out a right pneumonectomy. Histopathological diagnosis was biphasic synovial sarcoma. CONCLUSIONS: The presentation of pulmonary synovial sarcoma generally shows a peripheral location with a nonencapsulated and well-circumscribed tumor. Size ranges from 0.6 to 17 cm (mean: 5 cm). Histology is often characterized by a monophasic pattern. Diagnosis is difficult except for a uniform spindle cell pattern. Most synovial sarcomas show immunoreactivity for cytokeratin and/or epithelial membrane antigen. Cytogenetic characteristic of synovial sarcoma are t(X; 18)(p11, q11). Patient prognosis for pulmonary synovial sarcoma is poor with an overall 5-year survival rate of 50%.
Asunto(s)
Neoplasias Pulmonares/patología , Sarcoma Sinovial/patología , Adolescente , Biomarcadores de Tumor/análisis , Tos/etiología , Disnea/etiología , Femenino , Humanos , Neoplasias Pulmonares/química , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/cirugía , Neumonectomía , Pronóstico , Sarcoma Sinovial/química , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/diagnóstico por imagen , Sarcoma Sinovial/cirugía , Tomografía Computarizada por Rayos XRESUMEN
Involvement of the tongue by a synovial sarcoma (SS) is an extremely rare event; there have only been 13 cases previously reported. The authors present herein a case of monophasic SS arising in the tongue in a 32-year-old woman. The neoplasm expressed cytokeratins AE1-3, OSCAR, and EMA as well as Bcl-2 and TLE1. Molecular analysis indicated that the patient tested positive for the SYT/SS2 fusion transcript.
Asunto(s)
Proteínas de Fusión Oncogénica/genética , Patología Molecular/métodos , Sarcoma Sinovial/diagnóstico , Neoplasias de la Lengua/diagnóstico , Adulto , Biomarcadores de Tumor/metabolismo , Proliferación Celular , Proteínas Co-Represoras , Femenino , Humanos , Queratina-3/metabolismo , Mucina-1/metabolismo , Proteínas de Fusión Oncogénica/metabolismo , Proteínas Proto-Oncogénicas c-bcl-2/metabolismo , Receptores de Superficie Celular/metabolismo , Proteínas Represoras/metabolismo , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa/métodos , Sarcoma Sinovial/genética , Sarcoma Sinovial/metabolismo , Lengua/patología , Lengua/cirugía , Neoplasias de la Lengua/genética , Neoplasias de la Lengua/metabolismoRESUMEN
Pleuropulmonary synovial sarcoma (PPSS) is a rare entity, similar to synovial sarcoma of soft tissue (STSS). There are 120 published cases of PPSS, but no studies have explored the expression of TLE1. In soft tissues, it has been proven a useful marker, but in tumors of other sites, its expression has not been explored. The main objective was to study the expression and diagnostic sensitivity and specificity of TLE1 in a group of PPSS, of which the diagnosis was corroborated by fluorescence in situ hybridization confirming t(X;18) in a tissue microarray. Immunohistochemistry including TLE1, vimentin, CD99, CD56, bcl-2, AE1-AE3, EMA, CD34, CK7, CK19, calponin, and S-100 was performed on all PPSS and on 25 control cases (five carcinomas, ten mesotheliomas, and ten thoracic sarcomas). TLE1 was positive in 11 cases (73.3%); bcl-2 and vimentin in 100%; calponin and CD56 in 26.6%; CD99, CK AE1-AE3, CK19, CK7, and EMA in 80%; and S100 negative in all. The only biphasic PPSS was positive for epithelial markers only in the epithelial component. TLE1 was negative in all control cases. TLE1 is expressed in 73% of PPSS, a value inferior to that reported in STSS, but is highly specific for PPSS. TLE1 may therefore be of value in the differential diagnosis of PPSS, but should be used in a panel of antibodies.
Asunto(s)
Biomarcadores de Tumor/metabolismo , Neoplasias Pulmonares/metabolismo , Proteínas Represoras/metabolismo , Sarcoma Sinovial/metabolismo , Adulto , Anciano , Antígenos CD34/metabolismo , Proteínas Co-Represoras , Femenino , Humanos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/patología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Proteínas S100/metabolismo , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patología , Sensibilidad y Especificidad , Vimentina/metabolismoRESUMEN
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.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/patología , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/genética , Sarcoma Sinovial/patología , Diagnóstico Diferencial , Inmunohistoquímica , Proteínas de Fusión Oncogénica , Reacción en Cadena de la Polimerasa , Translocación GenéticaRESUMEN
We report a case of a 63 years-old-male with repeated bouts of confusion, aggressive behavior, dizziness and loss of consciousness occurring over a year. Such episodes increased in frequency and severity over time and were treated at the Emergency Room with intravenous dextrose solutions. Admitted at San Juan Bautista Medical Center due to severe hypoglycemia. Laboratory evaluation and prolong fasting ruled out an insulinoma or surreptitious administration of insulin or sulfonylurea. A CT scan of the abdomen and pelvis with oral contrast demonstrated a huge intra-abdominal mass invading the mesentery with herniated components laterally in the right lower quadrant. Patient underwent surgical resection of the abdominal mass. Histological description demonstrated a neoplastic pattern. Immunohistochemistry was compatible with an undifferentiated Synovial Sarcoma.
Asunto(s)
Mesenterio , Neoplasias Peritoneales/diagnóstico , Sarcoma Sinovial/diagnóstico , Agresión , Confusión/etiología , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Peritoneales/complicaciones , Recurrencia , Sarcoma Sinovial/complicaciones , Inconsciencia/etiologíaRESUMEN
Renal synovial sarcoma is a recently reported neoplasm rarely seen. We report a case of primary renal synovial sarcoma. The signs and symptoms are similar to any primary renal tumour diagnosis is clinically difficult through general survey or multiple imaging modalities and requires specific molecular and genetic testing. Surgical resection and ifosfamide-based chemotherapy are the mainstay of management. The prognosis can be poor. Physicians should be aware that synovial sarcoma is one of the possibilities of malignancy in the kidney.
Asunto(s)
Neoplasias Renales/diagnóstico , Sarcoma Sinovial/diagnóstico , Adulto , Carcinoma de Células Renales/diagnóstico , Diagnóstico Diferencial , Humanos , MasculinoAsunto(s)
Neoplasias Faciales/diagnóstico , Sarcoma Sinovial/diagnóstico , Neoplasias de los Tejidos Blandos/diagnóstico , Niño , Fosa Craneal Media/patología , Diagnóstico Diferencial , Neoplasias Faciales/patología , Femenino , Estudios de Seguimiento , Humanos , Queratina-1/análisis , Queratina-3/análisis , Mucina-1/análisis , Proteínas Proto-Oncogénicas c-bcl-2/análisis , Rabdomiosarcoma Embrionario/diagnóstico , Sarcoma Sinovial/patología , Neoplasias de los Tejidos Blandos/patología , Vimentina/análisisRESUMEN
OBJECTIVE: We report a primary renal Synovial Sarcoma (SS) case and analyze its features. METHOD: A 15 year old male presented with left abdominal mass and weight loss. CT scan images showed a 13 cm mass located in the lower pole of the left kidney. Renal biopsy recognized an undifferentiated neoplasm, the immunohistochemistry suggesting the probability of neuroectodermic primitive tumor versus SS. Chemotherapy and radical nephrectomy were carried out. Pathological study showed a big multilobulated necrotic tumor 22 x 13 x 12.5 cm. Histopathological study demonstrated a neoplasm composed by immature cells. Currently, patient has survived 1,8 years. A structured bibliographical search was performed in the Medline, Imbiomed and Scielo databases. RESULTS: The final immunohistochemistry studies gave the diagnosis of poorly differentiated renal SS small cell variety. CONCLUSION: The renal SS is extremely infrequent, with less than 40 cases reported, of which this case reports the earlier age. These tumors, when located in the kidney, represent a great diagnostic challenge that requires adequate clinical, radiological, surgical, and pathological correlation for appropriate diagnosis and treatment.
Asunto(s)
Neoplasias Renales , Sarcoma Sinovial , Adolescente , Factores de Edad , Humanos , Neoplasias Renales/diagnóstico , Masculino , Sarcoma Sinovial/diagnósticoRESUMEN
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.