Primary Gastric Synovial Sarcoma Diagnosed by Endoscopic Surveillance of a Gastric Ulcer

Primary gastric synovial sarcoma is rare and challenging to diagnose. We present a case of a gastric ulcer that was diagnosed as primary gastric synovial sarcoma only after surveillance endoscopy with repeat biopsies. The diagnosis was established with the identification of the pathognomonic chromosomal translocation t(X;18)(p11;q11). The patient was treated with wedge resection and has remained disease-free on surveillance imaging and endoscopy. This case demonstrates the difficulty in diagnosing primary gastric synovial sarcoma and the benefits of early disease detection.

Detection of the SYT-SSX Fusion Gene in Synovial Sarcoma

Objective: To investigate the feasibility and significance of detecting SYT-SSX fusion gene in paraffin-embedded tissues of synovial sarcoma (SS) by reverse-transcriptase polymerase chain reaction(RT-PCR) methods. Methods: Twenty cases of SS tumors from archival materials were collected and all samples were formalin-fixed and paraffin-embedded (FFPE). SYT-SSX fusion transcript was detected by RT-PCR. Home-keeping gene Porphobilinogen Deaminase (PBGD) was regarded as internal control.Results: PBGD mRNA was detected in all 20 tumor cases (100%). SYT-SSX fusion transcript was detected in 18 tumor cases (90%). In 18 SYT-SSX positive SS cases, there are 12 present SYT-SSX1 fusion transcript and 6 present SYT-SSX2 fusion transcript. SYT-SSX1 fusion transcript can be seen in 9 monophasic SS and 3 biphasic SS. In 6 SYT-SSX2 positive SS cases, 4 were monophasic SS and 2 were biphasic. Conclusion: Detection of SYT-SSX fusion transcripts in FFPE tissues for diagnosis of SS is feasible and sensitive. Subtypes of SYT-SSX fusion gene may provide prognosis information.

Solitary primary pulmonary synovial sarcoma:A case report

BACKGROUND Synovial sarcoma(SS)is an uncommon and highly malignant soft tissue sarcoma in the clinic,with primary pulmonary SS(PPSS)being extremely rare.Here,we describe the clinical characteristics,diagnosis,and treatment of a solitary PPSS case confirmed via surgical resection and fluorescence in situ hybridization(FISH).CASE SUMMARY A 33-year-old man was admitted because of intermittent coughing and hemoptysis for one month,with lung shadows observed for two years.Wholebody positron emission tomography-computed tomography(PET-CT)revealed a solitary mass in the upper lobe of the right lung,with uneven radioactivity uptake and a maximum standardized uptake value of 5.6.The greyish-yellow specimen obtained following thoracoscopic resection was covered with small multinodulated structures and consisted of soft tissue.Hematoxylin and eosin staining revealed spindle-shaped malignant tumor cells.Immunohistochemistry indicated these tumor cells were CD99 and BCL-2-positive.Furthermore,the FISH test revealed synovial sarcoma translocation genetic reassortment,which confirmed the diagnosis of SS.CONCLUSION PPSS is extremely rare and tends to be misdiagnosed as many primary pulmonary diseases.PET-CT,histologic analysis,and FISH tests can be used to differentiate PPSS from other diseases.Surgical resection is regularly recommended for the treatment of solitary PPSS and is helpful for improving the prognosis.

Imaging findings of primary pulmonary synovial sarcoma with secondary distant metastases:A case report

BACKGROUND Synovial sarcoma(SS)accounting for 6%-10%of primary soft tissue malignancies mainly occurs in deep soft tissue adjacent to joints of the limbs.Primary pulmonary SS(PPSS)is rare and has a poor prognosis.Cases of secondary distant metastases of PPSS occur rarely and there is a lack of corresponding imaging reports.We summarized the imaging findings of PPSS with multiple metastases confirmed by surgery and pathology,and shared valuable information on PPSS.CASE SUMMARY A 43-year-old female patient had a solid space occupying lesion in the right upper lobe of the lung.The results of a hemogram,erythrocyte sedimentation rate(ESR)and tumor markers were all within the normal range,tuberculin skin test(5 TU PPD)was negative(-).Chest computed tomography examination showed similar round soft tissue density in the posterior segment of the right upper lobe.Thoracoscopic-assisted wedge resection of the right upper lobe of the lung,right upper lobe resection and lymph node dissection were performed.Nine months after surgery,ultrasound examination showed multiple metastases on the chest wall and kidney.CONCLUSION PPSS is a rare malignant lung tumor with strong invasiveness,early distant metastasis and poor prognosis.There are very few imaging reports.PPSS is often manifested as irregular tumor and calcification,and the metastases have extremely low echo on ultrasonography.Contrast-enhanced ultrasound indicates that the arterial phase of tumor metastases shows rapid centripetal high enhancement,manifested as“fast forward and fast regression”.

Primary orbital monophasic synovial sarcoma with calcification:A case report

BACKGROUND Synovial sarcoma is a malignant mesenchymal neoplasm with variable epithelial differentiation.Most synovial sarcoma cases are reported in young adults and can arise in any body site.Notably,primary orbital synovial sarcoma is rare.CASE SUMMARY An 8-year-old east Asian girl with 1-month history of gradual painless proptosis and lacrimation of the right eye was admitted.The patient presented with painless proptosis,downward eyeball displacement,and upward movement disorders.According to clinical manifestations,imaging examinations and postoperative immunohistochemical examinations,the diagnosis was monophasic synovial sarcoma with calcification.The patient underwent anterior orbitotomy procedure for removal of the right orbital mass under general anesthesia.The diagnosis of monophasic synovial sarcoma with calcification was confirmed finally through histological and immunohistochemical exam.The follow-up period was 6 mo,and no recurrence was observed during this period.CONCLUSION Primary orbital monophasic synovial sarcoma with calcification is a rare sarcoma,and clinical manifestations and imaging results are not specific.The tumor may present similar features as a benign tumor.Comprehensive analysis of clinical,radiological,and pathological findings is critically important for making the right diagnosis.Conventional treatment approach for synovial sarcoma is surgical resection with adjuvant or neoadjuvant radiotherapy,which is highly effective for localized tumors.

Primary intracranial synovial sarcoma with hemorrhage:A case report

BACKGROUND Synovial sarcoma(SS)is a highly malignant tumor of unknown histological origin.This tumor can occur in various parts of the body,including those without synovial structures,but mainly in and around the joints,mostly in the lower extremities.Primary intracranial SSs are remarkably rare.This paper aims to report a case of primary intracranial SS with hemorrhage.CASE SUMMARY A 35-year-old male patient suffered a headache and slurred speech during manual labor and was sent to the emergency department.Through imaging examination,the patient was considered to have high-grade glioma complicated with hemorrhage and was treated with craniotomy.Postoperative pathology revealed SS.positron emission tomography/computed tomography was performed,which ruled out the possibility of metastasis to the intracranial from other parts of the body.Postoperative radiotherapy was given to the patient,during which radiation necrosis occurred.Sixteen months after craniotomy,cranial magnetic resonance imaging revealed recurrence of the tumor.CONCLUSION Primary intracranial SS is a rare malignant tumor.Primary intracranial SS with hemorrhage and radiation necrosis should be carefully monitored during postoperative radiotherapy.Surgical resection of the tumor combined with postoperative radiotherapy and chemotherapy is currently used,but the prognosis is poor.

Clinical Pathological Analysis of Synovial Sarcoma

OBJECTIVE To investigate the clinical diagnosis and differential diagno- sis of synovial sarcoma (SS). METHODS A total of 41 paraffin-embedded synovial sarcoma samples were examined by H&E staining, immunohistochemistry staining and the re- verse transcriptase polymerase chain reaction (RT-PCR), in order to provide a scientific bases for diagnosis and differential diagnosis. RESULTS Twelve cases were a biphasic type, 22 cases were a mono- phasic fibrous type, and 7 cases were a poorly differentiated type. Thirty-six cases were both CK (and/or EMA) and Vim positive. Five cases were only Vim positive. A SYT-SSX fusion gene was detected in 18 cases by RT-PCR. CONCLUSION By observation of the histomorphology, immunohisto- chemistry markers and detection of a SYT-SSX fusion gene, we can make a clinical pathological diagnosis of synovial sarcoma.

Massive recurrent synovial sarcoma of the neck:a case report

We report a rare case of massive recurrent synovial sarcoma of the neck in a 25-year-old woman.Physical exami- nation showed a 30×28 cm well-defined subcutaneous mass in the left cervical region,compressing the trachea to the right. Systemic examination revealed no metastasis to other organs.The diagnosis of sarcoma was established by percutaneous needle biopsy.Branches of the left subclavian artery and the left external carotid artery were found to supply the tumor,their embolization successfully shrank the tumor preoperatively allowing subsequent resection.Pathological analysis confirmed the diagnosis of synovial sarcoma.The patient was without recurrence 12 months after operation.

Commentary on"Primary orbital monophasic synovial sarcoma with calcification:A case report"

The present letter to the editor is related to the study titled“Primary orbital monophasic synovial sarcoma with calcification:A case report”.Orbital synovial sarcoma is one of the rare intraorbital masses seen in adult and pediatric populations.Some case reports in the literature revealed that synovial sarcoma may contain calcifications.Therefore,it is important to make differential diagnosis among calcified orbital masses in childhood.

Testicular Synovial Sarcoma:A Case Report

This paper reports a case of testicular synovial sarcoma with molecular genetic analysis. A 24-year-old male presented with painless scrotal mass. Ultrasonography showed a heterogeneous mass of 66 mm × 34 mm in size involving the inguinal region. Histological examination of a surgical biopsy showed a grade III monophasic growth pattern of spindle cell proliferation. Immunohistochemical analyses indicated positive staining for pancytokeratine and epithelial membrane antigen. Cytogenetic analysis showed the presence of CYT-SSX1 mutation, and CT scan showed non-specific pleural micro-nodules with a size of 7.5 mm. The patient had an extended left orchidectomy but was lost to follow-up for 1 year. A local recurrent scrotal mass of 32 mm ~ 25 ram, multiple inguinal lymph nodes, and increased pleural nodules, which were confirmed by histological examination, were treated with three cycles of adriamycine and ifosfamide chemotherapy, surgical resection, and radiotherapy with complete response. After 3 months, the patient developed local recurrence and pulmonary metastases that did not respond to second-line chemotherapy based on gemcitabine and paclitaxeL The patient had dyspnea at the time of this writing and chest pain, and is under third-line chemotherapy based on Deticene after 30 months of following up. This patient died on November 16, 2012 after a resperatory failure and malignant plural effusion. Synovial sarcoma should be considered in the differential diagnosis of soft tissue tumor and it should be aggressively treated to improve prognosis. Although our patient has shown numerous factors of bad prognosis, he has had a relatively long survival time.

Primary synovial sarcoma of pericardium: A case report

Primary pericardial sarcomas are extremely rare. We report a case of 19 year old male who presented with cough, dyspnoea, and orthopnea. Investigations and exploratory thoracotomy revealed a large pericardial mass. Surgical debulking of the tumor was performed and the histopathological examination was compatible with synovial sarcoma. The tumor was unresectable due to its invasion and adhesion to mediastinal structures. Hence patient was started on palliative chemotherapy (adriamycin and ifosfamide based). Patient showed an initial symptomatic response but later on there was a clinical progres-sion and died within six months of his diagnosis.

Primary Synovial Sarcoma of the Kidney: A Case Report

Synovial sarcoma originating from the kidney is extremely rare. A 13-year-old girl presented with a mild left flank pain of one-week duration, with no associated history of hematuria or any other systemic symptoms. Computed tomography (CT) demonstrated a 6 × 13 × 9 cm mass in the left kidney. No soft tissue or extrarenal masses were identified. The patient received a combined of treatment with doxorubicin and ifosfamide. A radical nephrectomy was performed in the left kidney with no complications. Postoperative pathology revealed post-chemotherapy residue of monophasic spindle cell synovialosarcoma of the left kidney. She received a combined treatment with doxorubicin and ifosfamide in concomittance with external radiation therapy. The patient was re-examined 4 months after surgery. An abdominal and pulmonary CT found no recurrence or metastasis.

Improvement of histone deacetylase inhibitor efficacy by SN38 through TWIST1 suppression in synovial sarcoma

Background:Synovial sarcoma(SS)is an SS18-SSX fusion gene-driven soft tissue sarcoma with mesenchymal characteristics,associated with a poor prognosis due to frequent metastasis to a distant organ,such as the lung.Histone deacetylase(HDAC)inhibitors(HDACis)are arising as potent molecular targeted drugs,as HDACi treatment disrupts the SS oncoprotein complex,which includes HDACs,in addition to general HDACi effects.To provide further molecular evidence for the advantages of HDACi treatment and its limitations due to drug resistance induced by the microenvironment in SS cells,we examined cellular responses to HDACi treatment in combination with two-dimensional(2D)and 3D culture conditions.Methods:Using several SS cell lines,biochemical and cell biological assays were performed with romidepsin,an HDAC1/2 selective inhibitor.SN38 was concomitantly used as an ameliorant drug with romidepsin treatment.Cytostasis,apoptosis induction,and MHC class I polypeptide-related sequence A/B(MICA/B)induction were monitored to evaluate the drug efficacy.In addition to the conventional 2D culture condition,spheroid culture was adopted to evaluate the influence of cell-mass microenvironment on chemoresistance.Results:By monitoring the cellular behavior with romidepsin and/or SN38 in SS cells,we observed that responsiveness is diverse in each cell line.In the apoptotic inducible cells,co-treatment with SN38 enhanced cell death.In nonapoptotic inducible cells,cytostasis and MICA/B induction were observed,and SN38 improved MICA/B induction further.As a novel efficacy of SN38,we revealed TWIST1 suppression in SS cells.In the spheroid(3D)condition,romidepsin efficacy was severely restricted in TWIST1-positive cells.We demonstrated that TWIST1 downregulation restored romidepsin efficacy even in spheroid form,and concomitant SN38 treatment along with romidepsin reproduced the reaction.Conclusions:The current study demonstrated the benefits and concerns of using HDACi for SS treatment in 2D and 3D culture conditions and provided molecular evidence that concomitant treatment with SN38 can overcome drug resistance to HDACi by suppressing TWIST1 expression.

Detection of SYT-SSX fusion transcripts in paraffin-embedded tissues of synovial sarcoma by reverse transcription-polymerase chain reaction

OBJECTIVE: To assess the feasibility of detecting SYT-SSX fusion transcripts in paraffin-embedded tissues of synovial sarcoma by reverse transcription-polymerase chain reaction (RT-PCR). METHODS: RT-PCR was used to amplify the SYT-SSX fusion transcripts using archival formalin-fixed paraffin-embedded tumor specimens from a series of 37 synovial sarcoma cases. To investigate the specificity of the SYT-SSX fusion transcripts, a variety of non-synovial sarcoma tumors were included in the study as negative controls. The detected messages derived from fusion genes were confirmed by subsequent sequence analysis. RESULTS: SYT-SSX fusion transcripts were detected in 33 of 37 (89.2%) synovial sarcomas. None of the 34 cases of non-synovial sarcoma tumors showed amplified products of SYT-SSX fusion transcripts, although PBGD mRNA was detected in all specimens. Among 33 SYT-SSX-positive synovial sarcomas, 22 tumors had an SYT-SSX 1 fusion transcript, whereas 6 tumors had an SYT-SSX2 fusion transcript. Fusion types can not be distinguished in the remaining 5 cases. There was a significant relationship between SYT-SSX fusion type and histologic subtype. All 10 biphasic synovial sarcomas had the SYT-SSX1 fusion, whereas all tumors with SYT-SSX2 were of monophasic morphology (P

Human Leukocyte Antigen-A Allele Distribution in Nasopharyngeal Carcinoma Patients Showing Anti-Melanoma-Associated Antigen A or Synovial Sarcoma X-2 T Cell Response in Blood

Background： Development of innovative immunotherapy is imperative to improve the poor survival of the nasopharyngeal carcinoma （NPC） patients. In this study, we evaluated the T cell response to melanoma-associated antigen （MAGE）-A1, MAGE-A3, or synovial sarcoma X-2 （SSX-2） in the peripheral blood of treatment-naive NPC patients. The relationship of responses among the three proteins and the human leukocyte antigen （HLA）-A types were analyzed to provide evidence of designing novel therapy. Methods： Sixty-one NPC patients admitted into the Tumor Hospital affiliated to the Xinjiang Medical University between March 2015 and July 2016 were enrolled. Mononuclear cells were isolated from the peripheral blood before any treatment. HLA-A alleles were typed with Sanger sequence-based typing technique. The T cell response to the MAGE-A1, MAGE-A3, or SSX-2 was evaluated with the Enzyme-Linked ImmunoSpot assay. Mann-Whitney U-test was used to compare the T cell responses from different groups. Spearman＇s rank correlation was used to analyze the relationship of T cell responses. Results： HLA-A＊02：01, A＊02：07, and A＊24：02 were the three most frequent alleles （18.9%, 12.3%, and 11.5%, respectively） among the 22 detected alleles. 31.1%, 19.7%, and 16.4% of the patients displayed MAGE-A1, MAGE-A3, or SSX-2-specific T cell response, respectively. The magnitudes of response to the three proteins were 32.5, 38.0, and 28.7 SFC/106 peripheral blood mononuclear cells, respectively. The T cell response against the three proteins correlated with each other to different extent. The percentage of A＊02：01 and A＊24：02 carriers were significantly higher in patients responding to any of the three proteins compared to the nonresponders. Conclusion： MAGE-A1, MAGE-A3, or SSX-2-specific T cell responses were detectable in a subgroup of NPC patients, the frequency and magnitude of which were correlated.

SYNOVIAL SARCOMA IN CHILDHOOD:CLINICAL AND RADIOLOGICAL FINDINGS

Objective: To study the clinical characteristics and radiological features of synovial sarcoma in childhood and its relation to the diagnosis and treatment. Methods: The clinical radiological features of 15 children with synovial sarcoma proved surgically and pathologically were analyzed. Results: In children, the tumor boundaries are poorly defined due to paucity of fat, and metastasis usually occurs early. Eight patients in this series had bone involvement, including: direct erosion by tumor causing cortical destruction, indirect pressure defect with sharp margin and reactive bone sclerosis and bone destruction of the primary intraosseous synovial sarcoma. Conclusion: The tumor is often misdiagnosed, the final confirmed diagnosis must be made by histological examination with imaging findings. It is emphasized that the patients should be treated with radiotherapy and chemotherapy preoperatively and postoperatively.

DNA CONTENT AND MORPHOLOGICAL PARAMETERS IN SYNOVIAL SARCOMA AND SYNOVIOMA

By using image analysis technique with 6 normal synovial tissue specimens as controls, nuclear DNA content and morphological parameters in specimens from 51 synovial sarcomas and 15 synovioma were examined quantitatively, The DNA content in synovial sarcoma and synovioma was significantly different (P < 0.01), and that of synovioma and normal synovial tissue was also different (P < 0.05), Morphological parameters including nuclear areas and perimeters differed significantly among the above three groups (P < 0.01). There was no correlation between the DNA content of synovial sarcoma and its histologic type (P > 0.05), but the DNA content greatly differed between its higher and lower differentiated grade in each type (P < 0.01). The DNA content in synovial sarcoma IIA stage, according to Enneking's staging system, was lower than that in IIB, IIIA and IIIB (P < 0.05), The 5-year survival rate of synovial sarcoma with diploid / nearly diploid (D / ND) pattern was higher than that of aneuploid (AN) pattern (P = 0.028), The study suggests that analysis of the DNA content and morphological parameters of tumor cells is helpful in the diagnosis and pathologic grading of synovial sarcoma, It also provides a guide to clinical operation and estimation of the prognosis, Although synovioma is usually a well-differentiated and benign lesion, this investigation also suggested that increased DNA content may be an indication of poor prognosis for this neoplasm.

上颌窦原发性滑膜肉瘤1例

滑膜肉瘤常发生于四肢及大关节附近,原发于上颌窦部位的极为罕见,目前国外偶有报道[1]。本病发病率低,且临床表现不特异,常造成误诊。本文对1例发生于上颌窦的滑膜肉瘤进行报道。1临床资料患者,女,55岁,因左侧面部疼痛伴右侧鼻塞2月余于2012年12月入院。患者2月前无明显诱因出现左侧面部疼痛,伴有左侧面部麻木,未服用药物,症状逐渐加重,出现黄涕、异味及涕中带血,

复发性咽部原发滑膜肉瘤1例

1 临床资料 患者,女,37岁,因“确诊口咽滑膜肉瘤5年,吞咽异物感4个月余”于2021-11-14日入院。患者5年前于海军军医大学第二附属医院耳鼻咽喉科在全麻下行“左侧扁桃体新生物切除术”,术后病理:梭形细胞滑膜肉瘤。后在我院肿瘤科行“吡柔比星+异环磷酰胺”方案化疗共6次,定期复查无明显异常。2021年7月患者开始出现吞咽异物感,伴咽部不适,无发热、声嘶、咽痛、咳血、呼吸困难等症状,就诊我科。

Glycogen synthase kinase 3b biology in bone and soft tissue sarcomas

Bone and soft tissue sarcomas are malignant neoplasms probably originating from musculoskeletal and mesenchymal progenitor cells.More than 80 different histopathological subtypes are encountered in orthopedics.The standard of care for sarcoma patients involves a multidisciplinary combination of surgery,anthracycline-based multiagent chemotherapy and radiation.Unfortunately,these are associated with adverse events and occasionally disappointing outcomes.Various genomic-,biologically-,and immunologically-based therapies are still under evaluation in early-phase clinical trials.However,there are strong barriers to the development and clinical translation of new therapeutic modalities.This is due to the rarity of these diseases,the broad spectrum of tumor subtypes with genetic and biological heterogeneity,and the wide variability in clinical manifestation,response to treatment and prognosis.A potential approach toward overcoming this barrier is to identify therapeutic targets that cover multiple sarcoma types.Glycogen synthase kinase 3b(GSK3b)has emerged as a common therapeutic target in more than 25 different cancer types.Here we review the evidence for tumor-promoting roles of GSK3b in the major types of bone and soft tissue sarcomas including osteosarcoma,rhabdomyosarcoma,synovial sarcoma,and fibrosarcoma.In this review,we describe the therapeutic effects of inhibiting GSK3b in these sarcoma types,while also protecting healthy cells and tissues from detrimental effects associated with conventional therapies,such as doxorubicin-induced cardiotoxicity.Consequently,we highlight GSK3b as a potential therapeutic target spanning multiple sarcoma types.