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.

Poorly differentiated synovial sarcoma in the wrist - Case report

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

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

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

Pericardium: An exceedingly rare site for a primary biphasic synovial sarcoma.

Pericardial neoplasms are rare. Among pericardial neoplasms, metastatic spread is more common and primary synovial sarcomas of the pericardium are exceedingly rare. A 61-year-old man was found dead in bed. Autopsy revealed the cause of death as atherosclerotic and hypertensive heart disease. There was a pericardial soft tissue mass lying predominantly over the left atrium. The cut surface was bulging out and grayish white in color with cystic changes. Microscopy showed spindle cells in densely cellular fascicles surrounding the epithelial cells. The epithelial cells were cuboidal and formed glandular structures and cleft-like spaces. Immunohistochemistry demonstrated cytokeratin and vimentin positivity in the spindled areas. Molecular analysis confirmed the diagnosis. Synovial sarcoma may be mistaken for other common neoplasms occurring in the pericardium due to its rarity. In all cases of pericardial tumors, a diligent search for synovial sarcoma by way of histological analysis including, immunohistochemistry and molecular analysis is indicated.

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

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

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

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

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

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

Synovial sarcoma of the foot: case report

Synovial sarcoma is a rare soft tissue sarcoma in the foot. It is commonly localized in the extremities, especially the lower thigh and knee areas. The histopathological immunohistochemical, and cytogenetic findings of a foot synovial sarcoma are described

Biphasic synovial sarcoma of oral cavity

Synovial sarcoma is a mesenchymal spindle cell tumour, which is unrelated to synovium and shows variable epithelial differentiation. Typically, synovial sarcoma arises in the soft tissues of the extremities but cases in the head and neck region are less common and oral cavity involvement is extremely rare. A 17-year-old girl presented with a gradually increasing swelling on the right cheek for 2 years, which on biopsy, revealed a biphasic tumour comprising fascicles of spindle shaped cells with gland formation by epithelial cells and scattered masts cells. Histological diagnosis of biphasic synovial sarcoma was confirmed on immunohistochemistry by strong positivity for EMA, S-100 and CD-99 in both epithelial as well as spindle cell areas

[Primary synovialosarcoma: a case of unusual location]

Report a new case of primary Synovi alosarcoma. We report a case of a 16 year old girl, who presented with a tumefaction on the right clavicle evolving or 3 months. Various explorations carried out, in particular thoracic scanner and imagery by magnetic resonance, showed a multilocular cystic formation of the seat and the clavicle reaching the mediastinum. A pathological examination of the part [piece] highlighted a biphasic synovialosarcoma of grade III. This diagnosis was validated by the immunohistochemistry study and the detection of transcribed specific fusion of the synovialosarcoma, SYT-SSXI. The unusual locations of the primitive SS must be known because the diversity of their microscopic aspects may cause confusion in the diagnosis. The immunohistochemistry, and more recently the cytogenetic studies, helps to solve these problems of differential diagnosis

Synovial sarcoma of the extremities: prognostic factors for 20 nonmetastatic cases and a new histologic grading system with prognostic significance

PURPOSE: To evaluate 20 cases of nonmetastatic synovial sarcoma of the extremities regarding prognostic factors, and to propose a histologic grading system with prognostic significance. METHODS: The cases of 20 patients (14 females and 6 males) with nonmetastatic synovial sarcomas of the extremities treated between 1985 and 1998, were retrospectively evaluated regarding prognostic factors. A histologic grading system with prognostic significance is proposed. RESULTS: The mean follow-up period was 48.4 months (range, 16-116 months). There was local recurrence in 3 cases (15 percent), microscopic surgical margin being the only prognostic factor identified. Seven patients (35 percent) died of the disease in a mean postoperative period of 31.7 months (range, 16-53 months), all with pulmonary or brain metastasis. The survival rate was 65 percent in 48.4 months of follow-up. CONCLUSION: The unfavorable prognostic factors identified regarding survival were high histologic grade, tumors proximal to the knee or elbow, and spontaneous tumor necrosis over 25 percent. Local recurrence did not have influence on survival in this study. The presence of mast cells appears to have a positive influence on survival, although statistical significance was not reached (P = 0.07). The oncologic and functional result was good in 6 cases (30 percent), regular in 7 (35 percent), and poor in 7 cases (35 percent).

OBJETIVO: Avaliar casos de sarcoma sinovial não-metastático das extremidades no que se refere a fatores prognósticos, e propor um sistema histológico de pontuação com significado prognóstico. MATERIAL E MÉTODO: Vinte casos (14 do sexo feminino e 6 do sexo masculino) de sarcomas sinoviais não-metastáticos das extremidades tratados entre 1985 e 1998 no departamento de Ortopedia foram avaliados retrospectivamente no que se refere a fatores prognósticos e está sendo proposto um sistema de pontuação histológico com significado prognóstico. RESULTADOS: A média dos períodos de acompanhamento foi 48,4 meses (mínimo 16 meses, máximo 116). Houve recorrência localizada em 3 casos (15 por cento), sendo a margem cirúrgica microscópica o único fator prognóstico identificado. Sete pacientes (35 por cento) morreram da doença, todos em período pós-operatório médio de 31,7 meses (mínimo 16 meses, máximo 53), todos com metástase pulmonar ou cerebral. A sobrevida foi de 65 por cento em 48,4 meses de acompanhamento. CONCLUSÃO: Os fatores prognósticos desfavoráveis identificados referentes à sobrevida foram: grau histológico alto, tumores proximais de joelho ou cotovelo e necrose espontânea de tumor acima de 25 por cento. Neste estudo, a recorrência localizada não influiu na sobrevida. Parece que a presença de mastócitos influi positivamente na sobrevida, porém não obtivemos significado estatístico (p=0,07). O resultado oncológico e funcional foi bom em seis casos (30 por cento), regular em sete (35 por cento) e insatisfatório em sete (35 por cento).

Regiões organizadoras nucleolares argirofílicas no sarcoma sinovial / Argyrophilic nucleolar organizer regions in synovial sarcoma

Regiões organizadoras nucleolares (NORs) correspondem a alças do DNA contendo genes responsáveis pela transcrição do RNA ribossômico, de 18S e 28S, situados no nucléolo da célula. Por possuírem reatividade com a prata são chamadas de AgNORs. O presente estudo investigou a correlação entre o número de AgNORs por núcleo e o prognóstico em dez sarcomas sinoviais diagnosticados no Hospital A. C. Camargo, entre 1972 e 1986, com informações confiáveis de seguimento clínico. A análise dos resultados não revelou correlação entre as variáveis estudadas, mostrando que o número de casos investigados e sua homogeneidade morfológica não permitem conclusões acerca do valor prognóstico dos AgNORs nos sarcomas sinoviais. Outrossim, o método se revelou barato, rápido e de aplicação simples para todo e qualquer serviço de patologia cirúrgica, podendo ser útil para avaliação da proliferação celular na rotina de laboratórios de pequeno porte.

Estudo do valor prognóstico de fatores clínico-patológicos e moleculares relacionados à regulação do ciclo celular, proliferação e angiogênese em portadores de sarcomas sinoviais de extremidades / Prognostic analysis of clinical, pathological and molecular factors related to cell regulation, proliferation and angiogenesis in patients with localized extremity synovial sarcoma

Para testar a hipótese de que pacientes portadores de sarcomas sinoviais de extremidades com características clínicas similares podem apresentar evoluções distintas em decorrência de alterações moleculares tumorais, reações imuno-histoquímicas para p53, Ki67, EGFR, c-erbB-2, e sintases do óxido nítrico 1,2 e 3 foram realizadas em tecidos de 57 pacientes para análise de sua positividade como valor prognóstico. Fatores prognósticos adversos para recidiva local, metástases e morte por tumor na análise multivariada foram apresentação como recidiva (p=0,014) e Ki67 score ≥5 por cento (p<0,001); Ki67 score ≥5 por cento (p<0,001) e negatividade para c-erb-B2 (p=0,01); e o tamanho do tumor >8 cm (p=0,047), respectivamente. Verificou-se que o Ki67 e c-erbB2 são marcadores de prognóstico independentes de fatores clínicos / To test the hypothesis that extremity synovial sarcoma patients with similar clinical scenarios can have distinct outcomes due to tumor molecular alterations, immunohistochemical reactions for p53, Ki67, EGFR, c-erbB-2, and nitric oxide sinthases 1,2 and 3 were performed in tissues of 57 patients to analyze their positivity as prognostic factors. Adverse prognostic factors for local recurrence, metastasis and tumor related death on multivariate analysis were presentation as local recurrence (p=0.014) and Ki67 score ≥5 per cent (p<0.001); Ki67 score ≥5 per cent (p<0.001) and negativity for c-erb-B2 (p=0.01); and tumor size >8 cm (p=0.047), respectively. It was verified that both Ki67 and c-erbB2 are prognostic factors independent of clinical variables...

Mesenchymal chondrosarcoma: a series of 23 cases.

A total of 23 cases of mesenchymal chondrosarcoma were studied from the histopathology records spanning 23 years. There were 16 men and 7 women afflicted with this tumor and their mean age was 28.1 years. The radiological features, histology and treatment outcomes have been studied. Osseous and extra-osseous mesenchymal chondrosarcomas are compared and the differential diagnosis discussed.

Primary renal sarcoma with morphologic and immunohistochemical aspects compatible with synovial sarcoma

Primary synovial sarcoma of the kidney is a rare tumor that is difficult to diagnose. We present one case that was not diagnosed through fine needle aspiration, requiring a morphologic and immunohistochemical analysis of the incision biopsy. Since the tumor was surgically unresectable, chemotherapy was employed previously to definitive radical surgery.

Primary Retroperitoneal Synovial Sarcoma: A Case Report

A case of a 36-yr-old woman with retroperitoneal synovial sarcoma is described. Her presenting symptom was epigastric pain that radiating to the back. On radiologic study, bulky retropancreatic soft tissue mass was detected which showed cystic and solid components. At operation, complete resection of the tumor was not possible because of the adhesion to the vena cava and the liver. During the follow-up, extensive tumor recurrence and liver metastasis were revealed. Primary retroperitoneal synovial sarcoma is a very rare malignant tumor with high mortality and recurrence rates. Retroperitoneal synovial sarcoma usually appears as a nonspecific soft tissue mass that do not have specific imaging features differentiating it from other mesenchymal tumors. However general radiologic findings and anatomic location of the tumor may help the diagnosis. In addition, synovial sarcoma should be included in the differential diagnosis of retroperitoneal soft tissue mass detected in young adults.