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Objective:To understand the clinical features and computed tomography (CT) imaging performance of mediastinal giant malignant synovial sarcoma (SS) and to improve the clinicians′ diagnosis and treatment of this disease.Methods:We report the clinical data of 2 cases of primary mediastinal giant malignant SS, and reviews the literature for CT presentation and differential diagnosis.Results:Primary giant malignant SS of mediastinum is rare in clinic. CT showed irregular cystic and solid mass with mural nodules, adjacent osteolytic destruction, unclear boundary with adjacent tissues. The enhancement showed that the solid components of the tumor and mural nodules were enhanced, while the cystic necrosis area had no enhanced uneven enhancement. The two cases in this paper showed " borehole-like" growth above the clavicle and growth into the left thoracic cavity, with pulmonary metastasis and lymphatic metastasis respectively.Conclusions:Mediastinal malignant SS is a rare disease with high malignancy and easy to metastasize, which emphasizes " early detection, early diagnosis and early treatment" . It should be taken into account in the differential diagnosis of mediastinal tumors, but the confirmation of diagnosis needs to be combined with pathological and immunohistochemical findings. Clinicians need to take advantage of imaging examinations to provide auxiliary guidance on the diagnosis, metastasis assessment and surgical treatment options of SS.
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Objective@#To investigate histopathological characteristics, and differential diagnoses of childhood synovial sarcoma.@*Methods@#HE staining, immunohistochemical staining and fusion gene detection by FISH were performed in 12 cases of synovial sarcoma in childhood at Beijing Children′s Hospital from 2016 to 2018.@*Results@#There were 6 cases of biphasic type, 1 case of monophasic epithelial type, 3 cases of monophasic spindle cell type and 2 cases of poorly differentiated synovial sarcomas. EMA, CKpan, bcl-2, CD99, TLE1 and CD34 immunostain positivities were observed in 10/12, 9/12, 12/12, 10/12, 10/12 and 0/12 cases respectively. Unique INI1 immunohistochemical staining was observed in 9/12 cases. SS18-SSX gene fusion was detected in 8 of 11 cases by FISH.@*Conclusions@#Synovial sarcoma is rare in children. Histological morphology combined with immunohistochemistry and FISH SS18-SSX fusion gene detection are important for the diagnosis and differential diagnosis of synovial sarcoma in children.
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El sarcoma sinovial representa entre el 2 al 10 % de todas las malignidades primarias de tejidos blandos, situándose en el cuarto lugar en frecuencia de los sarcomas de tejidos blandos. Es una neoplasia clasificada por la WHO (World Health Organization) bajo la categoría de tumores de diferenciación incierta, considerándose una malignidad entre intermedio y alto grado. El tratamiento estándar es la escisión quirúrgica, sin embargo, por su alta recurrencia y en los casos en que se asocia a metástasis o márgenes positivos después de la resección, se han planteado otros tratamientos como la quimio y radioterapia. La imaginología juega un papel importante en el diagnóstico, la estadificación y evaluación de la respuesta al tratamiento. Nuevas técnicas como el uso de difusión y mapas de ADC (Apparent Diffusion Coefficient, o coeficiente de difusión aparente) en resonancia magnética (RM) son útiles para evaluar las lesiones tumorales y la respuesta al tratamiento. En este artículo se presenta el caso de un paciente con un sarcoma sinovial monofásico, el diagnóstico por imágenes y evaluación del tratamiento mediante técnicas avanzadas de resonancia magnética como la difusión
Synovial sarcomas represent 2 to 10% of all the primary tissue malignancies and occupy the fourth place in the list of most common soft tissue sarcomas. According to the World Health Organization (WHO), this neoplasm is classified under the category of tumors of uncertain differentiation and is considered an intermediate to high-grade malignancy. Although the standard treatment is surgical excision, alternative treatments such as radiotherapy and chemotherapy have been proposed due to its high rate of recurrence in cases when it is associated with metastasis or positive resection margins. Imaging plays a key role in the diagnosis, staging and assessment of treatment of this disease. New techniques in Magnetic Resonance Imaging such as diffusion and Apparent Diffusion Coefficient (ADC) mapping are useful to further characterize these neoplastic lesions and to assess treatment response. In this article we present a patient with monophasic synovial sarcoma in which the use of these new imaging techniques was essential for the diagnosis and evaluation post-treatment.
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Humans , Diagnostic Imaging , Magnetic Resonance Imaging , Sarcoma, SynovialABSTRACT
Objective To analyse the clinicopathological characteristics of synovial sarcoma (SS) from histomorphology,immunohistochemical (IHC) and fluorescence in situ hybridization (FISH),and to investigate the related factors influencing the prognosis of SS patients.Methods 94 cases were collected,including 60 SS cases and 34 in dined to SS cases.The expressions of common antibodies related to the diagnosis of SS Vimentin,CD99,AE1/AE3,EMA,CD34,Calponin,Ki-67 were detected by IHC,and the SYT-SSX fusion gene was detected by FISH.The clinical pathologic factors that affecting the prognosis of patients were analyzed through the statistical method.Results The positive rates of Vimentin,CD99,AE1/AE3,EMA,CD34,Calponin,Ki-67 were 100.00 ％,74.47 ％,36.17 ％,28.72 ％,17.02 ％,90.43 ％ and 60.64 ％ respectively.FISH results showed that 81 patients were tested with SYT gene translocation,including 54 cases who were considered as SS,and 27 cases who were suspected/inclined to SS,and the number of positive cells surpassed 80 ％.The gene translocation was not related with the patient' s age,sex,tumor position,histological type,differentiation of the cancer (all P ＞ 0.05).Single factor survival analysis showed that the degree of differentiation,tumor diameter,the recurrence and metastasis had certain influences on patients' survival time (all P ＜ 0.05).Multiple factors regression analysis showed that the degree of differentiation and tumor diameter were the risk factors influencing the prognosis of SS (risk coefficients ＞ 1,P ＜ 0.05).Conclusions IHC combined with FISH SYT-SSX fusion gene detection have the vital significance in the diagnosis of SS.The degree of differentiation and tumor size are the risk factors influencing the prognosis of SS.
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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.
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Adult , Humans , Male , Sarcoma, Synovial/pathology , Skin/pathology , Soft Tissue Neoplasms/pathology , Wrist , Biopsy , ImmunohistochemistryABSTRACT
Introdução: O sarcoma sinovial (SS) é uma neoplasia rara e agressiva, sendo a região da cabeça e pescoço envolvida em 5% a 10% dos casos. A apresentação clínica é uma massa indolor de crescimento progressivo. As metástases ocorrem em 10% a 15% dos casos, principalmente por via hematogênica, para pulmões, linfonodos e medula óssea. O tratamento inclui exérese cirúrgica ampla e radioterapia. Ainda não existem dados que comprovem a eficácia da quimioterapia neste tipo de tumor; seu principal benefício consistiria na prevenção de metástases à distancia. A sobrevida em 5 anos varia de 40% a 50%. Objetivo: Relatar um caso de sarcoma sinovial cervical em mulher de 21 anos. Resultados: Paciente com história de tumor cervical de crescimento rápido há 05 meses, inicialmente indolor. Ao exame a lesão apresentava consistência fibroelástica, superfície lisa, aderida a planos profundos, comprometendo os níveis II, III, IV e V à direita e dor à palpação. Tomografia Computadorizada evidenciando grande tumor homogêneo com efeito de massa nos níveis II a V à direita e ocupando espaço parafaríngeo. Punção aspirativa (PAAF) sugestivo de sarcoma. Foi submetida à ressecção do tumor cervical e quimioterapia adjuvante. O anatomopatológico da lesão, com estudo imunohistoquímico, foi compatível com sarcoma sinovial cervical. Está no o 3º ano de seguimento pós-operatório e encontra-se sem sinais de lesão residual ou recidiva. Conclusão: o sarcoma sinovial cervical é uma neoplasia rara e agressiva que demanda ressecção cirúrgica ampla.
Introduction: The synovial sarcoma (SS) is a rare and aggressive neoplasm, with the head and neck involved in 5% to 10% of cases. The clinical presentation is a painless mass with progressive growth. Metastases occur in 10% to 15% of cases, mainly hematogenic to lungs, lymph nodes and bone marrow. Treatment includes wide surgical excision and radiotherapy. There are no data to prove the effectiveness of chemotherapy in this tumor type, but its main benefit would be the prevention of distant metastases. The 5-year survival ranges from 40% to 50%. Objective: To report a case of synovial sarcoma of the neck in a 21 years old female. Results: female with history of cervical tumor of rapid growth for 05 months, initially painless. On examination the lesion presented fibroelastic consistency, smooth surface, adhered to deep planes, compromising levels II, III, IV and V to the right. Computed tomography revealed a large homogenous tumor with mass effect on levels II to V and occupying the right parapharyngeal space. Needle aspiration were suggestive of sarcoma. Underwent resection of the cervical tumor and adjuvant chemotherapy. Histopathological examination of the lesion with immunohistochemical study was consistent with cervical synovial sarcoma. She is in the 3rd year of postoperative follow-up and found no signs of residual lesion or recurrence. Conclusion: synovial sarcoma of the neck is a rare and aggressive neoplasm that requires wide surgical resection.
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Objective To investigate the way of improving the diagnosis and cure rate of pulmonary synovial sarcoma.Methods One case of pulmonary synovial sarcoma was selected,at the same time accompanied with 80 cases of pulmonary synovial sarcoma with complete data referenced from China Journal Full-text Database on October 2012 were retrospectively studied.The main clinical manifestations,imaging characteristics were analyszed.According to the pathology,immunohisochemistry and molecular biology for diagnosis and discrimination,in order to reduce the misdiagnosis.Results All of 81 patients,40 male cases and 41 female cases,the main clinical manifestations were chest pain,difficult breathing,hemoptysis and cough.In individual cases without any discomfort.All cases had not been accurately diagnosed before surgery.Ultimately diagnosis of patients depend on histopathology and immunohistochemistry.Conclusions Pulmonary synovial sarcoma shows different clinical symptoms but specific imaging characteristic and maybe confused with other pulmonary tumors or inflammation.Histopathological,immunophenotypic and fusion gene detection is the mainly technics for diagnosis.Surgery is the primary therapy,patients undergoing uncompletely resection have a poor prognosis.
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Objective To investigate the expressions and functions of the fusion gene SYT-SSX1 and SYT-SSX2 in synovial sarcoma.Methods The synovial sarcoma tissue samples and clinical data of 22 synovial sarcoma patients were collected.The expressions of fusion genes were detected by RT-PCR.The relationships between fusion gene and clinicopathologic factors were statistically analyzed.The abilities of proliferation,migration and invasion of 3T3 cells transfected with tusion gene plasmids were detected by MTT,migration and invasion assays.Results There were 11 cases with expression of SYT-SSX1 gene and 9 cases with that of SYT-SSX2 genes.The ratio of SYT-SSX1 and SYT-SSX2 was about 1:1.The SYT-SSX1 positive tumors were most biphasic SS and the tumor volumes of patients with SYT-SSX1 positive were larger than that of patients with SYT-SSX2 positive (P =0.028).The SYT-SSX1 positive NIH3T3 cells exhibited higher abilities of proliferation,migration and invasion than SYT-SSX2 positive.Conclusions The ratio of expression of SYT-SSX1 and SYT-SSX2 in synovial sarcoma was about 1∶1.Both SYT-SSX1 and SYT-SSX2 can promote the growth and migration in NIH3T3 cell.The abilities of proliferation and migration of SYT-SS1 were more potent than that of SYT-SSX2.
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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.
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Adolescent , Female , Humans , Esophageal Neoplasms , Sarcoma, Synovial , Esophageal Neoplasms/pathology , Esophageal Neoplasms/surgery , Sarcoma, Synovial/pathology , Sarcoma, Synovial/surgeryABSTRACT
We experienced a case of primary pulmonary biphasic synovial sarcoma, which was confirmed by immunohistochemistry and molecular testing of SYT-SSX2 fusion transcripts. The patient was a 61-year-old man who presented with a well-defined mass in the left upper lung field on chest radiography. Left upper lobectomy with lymph node dissection was performed. Histological and immunophenotypic features were consistent with biphasic synovial sarcoma. Reverse transcriptase polymerase chain reaction, performed using RNA extracted from frozen tumor samples for the detection of SYT-SSX fusion gene, amplified a single 331-bp fragment that was characteristic of the SYT-SSX2 fusion transcripts. We report a case of primary pulmonary biphasic synovial sarcoma, which was confirmed by SYT-SSX2 fusion transcripts, and present a brief review of the literature on Korean cases.
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Humans , Male , Middle Aged , Base Sequence , DNA Primers/genetics , Korea , Lung Neoplasms/diagnosis , Oncogene Proteins, Fusion/genetics , Oncogenes , Sarcoma, Synovial/diagnosisABSTRACT
Objective To discuss the presentations, pathologic features, diagnosis and treatment of primary synovial sarcoma of the kidney. Methods One case of primary synovial sarcoma of the kidney was reported and the relevant literature was reviewed. A 55-year-old man was admitted with complaint of right abdomen and flank pain for 5 h. Computerized tomography revealed a 12.5 cm × 11.0 cm × 9. 0 cm mass located at the middle and lower pole of the right kidney. The patient was taken radical nephrectomy. Results The diagnosis of primary synovial sarcoma of the kidney in the patient was confirmed by postoperative pathology. Under microscope, tumor was typically mitotically active, monomorphic spindle cells growing in intersecting fascicles or in solid sheets with epithelial differentiation. In some areas a haemangiopericytoma-like pattern was found. Immunohistochemical staining showed that the tumor cells were positive for the markers Vimentin, CD99 and Bcl-2, but CK was negative. The patient died of local recurrence and multi-metastasis at 8 months after surgery. Conclusions Primary synovial sarcoma of the kidney is extremely rare with a high grade of malignancy,and its prognosis is poor. The diagnosis depends on pathological features, Immunohistochemical studies and RT-PCR detection. Radical resection combined with chemicaltherapy is considered to be the most reliable treatment so far.
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Synovial sarcoma is an uncommon malignant soft tissue neoplasm, occurring primarily in adolescents and young adults. It is prevalent in the periarticular soft tissues near large joints of the extremities and rarely involves the trunk. Metastases are not uncommon and usually involve the lungs; metastasis to the thoracic spine is rare. We report the case of a 47-year-old man with a history of synovial sarcoma of the lower back, with subsequent metastases to the lung, penis, and perineum (all previously resected), presenting with a 3-month history of low back pain and lower extremity paresthesias. Magnetic resonance imaging (MRI) demonstrated multiple lesions involving multiple contiguous vertebral bodies, with the mass at T12 compressing the spinal cord. The patient underwent T11-T12 laminectomy, transpedicular decompression, tumor debulking, and posterior fixation and fusion. The patient died six months later due to disease progression. Although not curative, decompression and stabilization of the spine are often necessary in patients who present spinal cord compression.
O sarcoma sinovial é uma neoplasia rara dos tecidos moles que afeta adolescentes e adultos jovens. A sua maior prevalência é nas grandes articulações das extremidades e raramente acomete o tronco. As lesões metastáticas são raras e geralmente acometem os pulmões, e as metástases para a coluna torácica são raras. Relata-se o caso de um paciente de 47 anos de idade com 3 meses de história de dor lombar e que apresentava metástase de sarcoma sinovial na coluna lombar. A ressonância magnética demonstrava lesões contíguas do corpo vertebral e compressão do canal vertebral ao nível de T12. O paciente foi submetido à laminectomia de T11-T12, descompressão transpedicular, remoção tecido tumoral e artrodese e fixação posterior. O paciente foi a óbito após seis meses devido à progressão da doença. Embora a descompressão e estabilização cirúrgica do canal vertebral não sejam curativas, esses procedimentos podem ser necessários em pacientes que apresentam compressão da medula espinhal.
El sarcoma sinovial es una neoplasia rara de los tejidos blandos que afecta adolescentes y adultos jóvenes. Su mayor prevalencia es en las grandes articulaciones de las extremidades y raramente ataca el tronco. Las lesiones metastásicas son raras y generalmente atacan los pulmones, siendo que las metástasis de columna torácica son raras. Será relatado el cuadro clínico de un paciente de 47 años de edad con tres meses de historia de dolor lumbar y presentando metástasis de sarcoma sinovial en la columna lumbar. La resonancia magnética demostraba lesiones contiguas del cuerpo vertebral y compresión del canal vertebral al nivel de T12. El paciente fue sometido a la laminectomía de T11-T12, descompresión transpedicular, remoción de tejido tumoral y artrodesis con fijación posterior. El paciente fue a óbito después de seis meses debido a la progresión de la enfermedad. Aunque la descompresión y estabilización quirúrgica del canal vertebral no sea curativa, ese procedimiento puede ser necesario en los pacientes que presentan compresión de la médula espinal.
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Humans , Adult , Laminectomy , Neoplasm Metastasis , Spinal Neoplasms/secondary , Sarcoma, Synovial , Spinal Cord Compression , Spinal Fusion , Thoracic Vertebrae/pathologyABSTRACT
Synovial sarcoma (SS) is defined as a malignant biphasic tumor of soft tissue of uncertain histogenesis characterized by epithelial glands situated within a spindled fibrosarcomatous stroma. SS comprising 5.6-10% of primary adult soft tissue sarcomas are more prevalent in adolescents and young adults aged 15-40 years. About 85-95% of SS arise in the extremities, only 3-10% arise in the head and neck region and rarely presents in the tongue. Males are affected twice as often as females. The hypopharynx and retropharynx are the most common sites of involvement in the head and neck. Fewer than 100 cases of SS have been reported in the head and neck area. We experienced a 21-year-old male patient who first complained of oral bleeding and dysphagia. Initial transoral biopsy specimen of the tongue base lesion was consistent with sarcoma. Surgical excision through left modified neck dissection with tongue base tumor excision and postoperative radiotherapy were performed. We will introduce this rare case report of SS with a literature review.
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Adolescent , Adult , Female , Humans , Male , Young Adult , Biopsy , Deglutition Disorders , Extremities , Head , Hemorrhage , Hypopharynx , Neck , Neck Dissection , Radiotherapy , Sarcoma , Sarcoma, Synovial , TongueABSTRACT
Synovial sarcoma is a malignant soft tissue tumor that primarily occurs in the paraarticular regions, especially in the knee. They are composed of keratin-positive epithelial cells and vimentin-positive spindle cells. We report a 76 year old woman with a primary synovial sarcoma in the mediastinum that had severe adhesion to the right side of pericardium. Primary synovial sarcoma in the mediastinum is extremely rare and this is the first case reported in the Korean literature. The mass including the pericardium was resected and the defect was closed with Gore-Tex patch. The patient is well and free of disease 6 months after the operation.