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.

Evolución infrecuente de sinoviosarcoma de muslo / Uncommon evolution after excision of a thigh synovial sarcoma

Background: Synovial sarcoma is a soft tissue tumor with a high degree of local and metastatic invasion. Case report: We report a 26 years old smoker woman with a history of a synovial tumor excised from the right thigh, who required a hip prosthesis for a pathological fracture due to metastases. Two years after, a lung metastasis in the left upper lobe was excised and treated with chemo and radiotherapy. Two years later a new lung nodule was found and excised. Pathology confirmed that they were metastases of the primary tumor. Three months after this last excision, the patient is asymptomatic.

Introducción: El sinoviosarcoma es una neoplasia de partes blandas con alto grado de invasión local y producción de metástasis. Caso clínico: Mujer de 26 años con 2 episodios secuenciales de metástasis pulmonar a lo largo de 6 años posterior al tratamiento del tumor primario. Patología de tratamiento muy complejo.

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.

Primary intrathoracic biphasic synovial sarcoma

Synovial sarcomas are most frequently observed in the extremities. A lthough synovial sarcomas are the third most common histological type of soft-tissue sarcomas of the extremities, primary mediastinal synovial sarcoma is extremely rare. Monophasic synovial sarcoma is the most commonly observed subtype. whereas the biphasic subtype is less common. We present our case which was diagnosed as biphasic synovial sarcoma located in the anterior mediastinum, which is considered to be a rare entity. The patient underwent surgical resection together with multimodal adjuvant radiotherapy and chemotherapy

Primary renal synovial sarcoma

Primary synovial sarcoma [SS] of kidney is very rare and difficult to diagnose. Here, we present a case of a 21-year-old female clinically diagnosed as renal cell carcinoma. Right nephrectomy specimen showed a cystic tumor in the upper pole of kidney with areas of hemorrhage and solid growth. Histologically, it showed poorly differentiated cells with hemangiopericytoma-like vascular pattern. Morphologic and immunohistochemical features were compatible with the diagnosis of poorly differentiated SS of kidney. Primary renal SS is a recently described entity. To the best of our knowledge, approximately 34 cases have been reported till date and this is the eighth documented case of poorly differentiated variant. Most of the time, poorly differentiated SS of kidney exhibits hemangiopericytoma like histology. Reverse transcriptase-polymerase chain reaction analysis to demonstrate SYT-SSX fusion gene transcript helps to confirm the diagnosis

Sarcoma sinovial intraoral primario monofásico / Monophasic synovial sarcoma of the oral cavity: report of one case

El sarcoma sinovial es un tumor maligno de partes blandas, bien diferenciado y que representa entre 5.6 por ciento a 10 por ciento de todos los sarcomas. Su localización en cabeza y cuello no es común, y representa cerca de un 9 por ciento, con menos de 100 casos reportados en la literatura. La localización intraoral es muy peculiar, existiendo 32 casos previamente descritos en el mundo, de los cuales 3 corresponden al tipo monofásico. Se presenta un caso de un paciente varón de 16 años con un caso de sarcoma sinovial intraoral monofásico y una revisión de la literatura disponible.

Synovial sarcoma is a well-differentiated malignant tumor of soft tissues, that corresponds approximately 10 percent of all soft tissue sarcomas. Its location in the head and neck uncommon and the location in the oral cavity is really peculiar, with only 32 previously described cases, of which only three corresponded to the monophasic type. We report a 16 years old male patient consulting for a lump in the right oral mucosa. The patient was operated in two occasions excising the tumor with radical lymph node dissection and parotid gland excision. The pathological diagnosis was a monophasic intraoral synovial sarcoma. Postoperative radiotherapy was recommended but not done. After 16 months of follow up the patient is without evidence of relapse.

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 synovial sarcoma of the abdominal wall: a case report and review of the literature

Synovial sarcoma is a malignant mesenchymal neoplasm which commonly occurs in the extremities of adults, in close association with joint capsules, tendon sheaths, bursae and fascial structures. Only a few cases of synovial sarcoma occurring in the abdominal wall have been reported. A case of a primary synovial sarcoma arising from the anterior abdominal wall fascial aponeurosis is presented

Sarcoma sinovial de cabeza y cuello: reporte de un caso / Synovial sarcoma of the head and neck: case report

El sarcoma sinovial es un tumor mesenquimático de tejidos blandos que se encuentra raramente ubicado en la región de cabeza y cuello. Estos tumores se presentan predominantemente en pacientes adultos jóvenes de sexo masculino, ubicándose generalmente en el espacio parafaríngeo. Reportamos el caso de un paciente con sarcoma sinovial bifásico de alto grado de la pared posterior de la faringe, al que se efectuó una resección en dos etapas, seguidas de radioterapia post operatoria.

Synovial sarcoma is a mesenchymal soft-tissue tumor rarely found in the head and neck region. These tumors occur most commonly in young-adult male patients and generally localize in the parapharyngeal space. We report the case of a patient with a high grade biphasic synovial sarcoma of the posterior wall of the pharynx, to whom a two-stages resection was effected, following by post surgical radiotherapy sessions.

Surgical Treatment of Malignant and Aggressive Bone Tumors Around the Knee by Segmental Resection and Rotationplasty

In patients having malignant or aggressive bone tumors around the knee joint and requiring amputation, segmental resection and rotationplasty was performed and the clinical results were analyzed. Twenty-six patients underwent segmental resection and rotationplasty between February 1988 and June 1994. The mean follow-up period was 57 (6 - 120) months and the average age of patients was 21.4 (5 - 37) years. Out of 26 patients, there were 18 osteosarcomas (> or = stage IIB), 2 synovial sarcomas, and 6 giant cell tumors. Clinical results were evaluated by the Shriner's rating system. Four patients were excluded due to death or amputation and the remaining twenty-two patients were included for assessment. Eighteen patients had excellent results, 3 good, and 1 fair. Range of motion of the ankle joint was -11 (dorsiflexion) - 80 (plantarflexion) degrees and daily walking activity was possible. Local recurrence developed in 2 patients and distant metastasis in 10. Early complications include 3 thromboses and 1 sepsis, and late complications were 6 nonunion, 2 malrotation and 1 stiffness of the ankle joint. Rotationplasty, which is functionally excellent, may serve as an effective partial limb salvage procedure, especially in patients younger than 10 years old who are expected to have leg length discrepancy or loosening of the tumor prosthesis due to the growth of the medullary cavity or when amputation is inevitable for a wide resection margin.

Margen quirurgico en los sarcomas musculoesqueleticos y de partes blandas de bajo y alto grado de malignidad / Surgical margin in the treatment of muscle-skeletal and soft-tissue sarcomas of high and low grade malignancy

El motivo del presente trabajo es presentar los resultados de la planificacion, la clasificacion y el tipo de margen quirurgico en el tratamiento de los sarcomas musculoesqueleticos y de partes blandas, de bajo y alto grado de malignidad, para elevar la casuistica presentada el 14 de junio de 1991 a 18 pacientes, tratados y controlados en el Hospital Angel C. Padilla de la ciudad de San Miguel de Tucuman, de 1985 a 1998. Durante este periodo, fueron evaluados 11 sarcomas musculoesqueleticos y 7 sarcomas de partes blandas ; 16 ubicados en las extremidades inferiores y 2 en las extremidades superiores. Ocho pacientes eran de sexo masculino y 10 de sexo femenino. Las edades oscilaban entre los 15 y 69 años (resumen truncado)

Tumores cutáneos dolorosos: segunda parte / Painful cutaneous tumors: second part

Se describe una serie clínico-patológica de tumores cutáneos habitualmente dolorosos y se revisan las características de cada uno de ellos, lipomatosis dolorosa, neurilemoma, neuroma, nevo azul en tetina de goma, sinovialoma, tumor de células granulosas y tumor glómico

Hallazgos en resonancia magnética de sarcoma sinovial primario y recurrente / Findings in magnetic resonance of primary and recurrence synovial sarcoma

Object: To evaluate MRI findings of primary synovial sarcoma and to differentiate recurrent tumor from post-treatment changes. Material and methods: We retrospectively analyzed pre and post-treatment MRI findings of proven primary and recurrent synovial sarcomas in 22 patients. MRI findings were correlated with histological diagnoses and clinical follow-ups for 1-5 years. Results: Ninety-one percent of the studied synovial sarcomas were in the extremities (68 percent) in the lower extremities), and sizes ranged 3-12 cm. Aproximately 73 percent of the primary tumors were located less than 5 cm. from a synovial joint. Most common MRI findings were nodular masses with heterogeneous intermediate signal intensity (SI) on T1 weighted images (WI), high SI on T2-WI and heterogenous contrast enhancement. A cystic component was recognized in 17 cases (77 percent). Ninety percent of the sarcomas were oval and well-defined. MRI findings consistent with intratumoral hemorrhage was noted in 73 percent. Lung and nodal metastases were found in 27 percent and 5 percent respectively. Lung metastases were found more commonly in biphasic than monophasic histologic types (50 percent vs 41 percent). Involvement of adjacent bones was seen in 23 percent (3 biphasic abc 2 monophasic sarcomas). Calcification was only noted in three monophasic sarcomas on plain films and CT. Two recurrent sarcomas showed focal nodular masses with high SI on T2-WI and homogenous contrast enhancement. Post-treatment changes revealed diffusely increased SI on T2-WI and slight diffuse contrast enhancement with feathery appearence. Conclusion: Detection of primary and recurrent synovial sarcoma can be made by recognizing both morphology and MR signal characteristics including contrast enhancement pattern. Contrary to other reports, sarcomas with biphasic histologic type showed more metastases and bone involvement. Association between calcifications and monophasic type was not previously reported

Sarcoma sinovial intraoral: reporte de un caso / Cheek synovial sarcoma: report an case

Se presenta el caso de un paciente masculino quien consulta al Servicio de Cirugía Reconstructiva y Maxilofacial, presentando un tumor de 2 años de evolución, a nivel de la mucosa del carrillo izquierdo de crecimiento progresivo, que limita los movimientos masticatorios y la apertura bucal, de difícil diagnóstico y de rara localización. Estudios histológicos preoperatorios reportaron 1) Neurofiboma celular, 2) Rabdomiosarcoma, y 3) Dermatofibrosarcoma protuberante. No siendo sino al examinar exhaustivamente la pieza operatoria cuando se puede obtener un diagnóstico definitivo de Sarcoma

Pneumotórax espontâneo bilateral com metástases pulmonares de sarcoma sinovial

A associaçäo de pneumotórax expontâneo bilateral com tumor pulmonar é pouco freqüente, e com metástases pulmonares é rara. As características clíncias e radiológicas de pneumotórax expontâneo com metástases de sarcoma sinovial estäo descritas neste caso, de um adolescente de 14 anos de idade