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....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.展开更多
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 trea...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.展开更多
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. Met...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.展开更多
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.Case...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”.展开更多
Synovial sarcoma(SS) is genetically characterized by chromosomal translocation, which generates SYT-SSX fusion transcripts. Although SS can occur in any body part, primary gastric SS is substantially rare. Here we des...Synovial sarcoma(SS) is genetically characterized by chromosomal translocation, which generates SYT-SSX fusion transcripts. Although SS can occur in any body part, primary gastric SS is substantially rare. Here we describe a detection of the fusion gene sequence of gastric SS in plasma cell-free DNA(cf DNA). A gastric submucosal tumor was detected in the stomach of a 27-year-old woman and diagnosed as SS. Candidate intronic primers were designed to detect the intronic fusion breakpoint and this fusion sequence was confirmed in intron 10 of SYT and intron 5 of SSX2 by genomic polymerase chain reaction(PCR) and direct sequencing. A locked nucleic acid(LNA) probe specificto the fusion sequence was designed for detecting the fusion sequence in plasma and the fusion sequence was detected in preoperative plasma cfD NA, while not detected in postoperative plasma cfD NA. This technique will be useful for monitoring translocation-derived diseases such as SS.展开更多
BACKGROUND Synovial sarcoma,a rare mesenchymal tumor type with unclear histological origin and direction of differentiation,accounts for 6%–10%of soft tissue tumors.It is mainly located near the joints and tendons of...BACKGROUND Synovial sarcoma,a rare mesenchymal tumor type with unclear histological origin and direction of differentiation,accounts for 6%–10%of soft tissue tumors.It is mainly located near the joints and tendons of the limbs,and occurs primarily in children or young adults.Primary renal synovial sarcoma(PRSS)is very rare,accounting for approximately 1%of synovial sarcomas.It is a spindle cell tumor type affecting mesenchymal tissue,and has morphological,genetic,and clinical characteristics,and a certain degree of epithelial differentiation.It is highly malignant and has the fourth highest incidence among soft tissue sarcomas.Here,we report a case of PRSS and share some valuable information about the disease.CASE SUMMARY A 54-year-old male patient was admitted to the hospital for a space-occupying lesion in the right kidney for 2 d upon ultrasound examination.The patient had no cold or fever;no frequency,urgency or pain of urination;and no other discomfort.The results of a hemogram,blood biochemistry,and tumor markers were in the normal range.The patient was examined by computed tomography(CT),which indicated the presence of a soft tissue density shadow with a diameter of approximately 6.8 cm in the right renal pelvis area,showing uneven enhancement.Ultrasound indicated a cystic solid mass of approximately 6.8 cm×6.5 cm in the right kidney,with an unclear boundary and irregular shape.Meanwhile,color Doppler flow imaging showed dotted blood flow signals in the periphery and interior.Contrast-enhanced ultrasound(CEUS)showed"slow in and fast out"hyperenhancement of the right renal mass after contrast agent injection.The postoperative pathological diagnosis was(right kidney)synovial sarcoma.Despite postoperative adjuvant chemotherapy,tumor recurrence was detected two years later.CONCLUSION PRSS is a rare malignant tumor.To date,no characteristic imaging findings have been observed.The diagnosis is confirmed primarily through postoperative pathological immunohistochemistry and SS18(SYT)gene detection.In this case,CEUS was used preoperatively.We found that PRSS has the characteristic of"slow in and fast out"hyperenhancement,and its particular characteristics have diagnostic value.Postoperative adjuvant chemotherapy is not very effective.展开更多
BACKGROUND Synovial sarcoma(SS) is a rare type of soft tissue sarcoma that is usually developed from areas where synovial tissue exists, especially at the extremities.Nevertheless, several cases of retroperitoneal SS(...BACKGROUND Synovial sarcoma(SS) is a rare type of soft tissue sarcoma that is usually developed from areas where synovial tissue exists, especially at the extremities.Nevertheless, several cases of retroperitoneal SS(RSS) have been described. We herein report a case of RSS presented in our institution.CASE SUMMARY A 69-year-old female patient was admitted with a large, palpable, firm mass in the right abdominal space SS. Computerized tomography scan depicted a concentric, sharply marinated retro-peritoneal lesion which was displacing the right kidney and the lower edge of the liver. Subsequently, the patient underwent surgical excision of the mass with additional right nephrectomy and resection of the right adrenal gland and a part of the diaphragm. The final histological diagnosis of the tumour was grade II monophasic RSS.CONCLUSION RSS is encountered in the biphasic type, the monophasic fibrous, and the monophasic epithelial category as well. Relevant clinical manifestations are not always documented at early stages. Therefore, the final diagnosis is posed after complete histological examination taking into consideration the results of immunochemistry and genetic analysis. Therapeutic approach happens often late when metastases at the lungs and the liver are apparent. Thus, 5-year survival rates remain low.展开更多
We describe a case of primary pulmonary synovial sarcoma arising in the lung of a 35-year-old woman.The tumor was a single mass and had a well-defined pleural-based with homogeneous density in the edge of the middle l...We describe a case of primary pulmonary synovial sarcoma arising in the lung of a 35-year-old woman.The tumor was a single mass and had a well-defined pleural-based with homogeneous density in the edge of the middle lobe,with no calcification and a fat component on plain CT scan.On the contrast-enhanced CT scan,the tumor showed moderately enhancement and circular enhancement with no enlargement of mediastinal,hilar lymph nodes.Skeleton single photon emission CT(ECT) was unremarkable.Gross and histological findings by postoperative pathology showed the tumor had pseudo-capsule and was well-circumscribed with foci of necrosis,hemorrhage and cystic change,under microscope tumor was typically mitotically active,spindle cells growing in intersecting fascicles or in solid sheets with epithelial differentiation.The immunohistochemical examination of CD99,Vim,PCK and EMA were positive.The tumor recurred with multiple pleural nodules and mass at 5 months after surgery,chest CT revealed the tumor was in the ipsilateral costophrenic angle for chest pain of the patient.After dynamic contrast-enhanced,the solid parts and the capsule of the lesion showed progressive enhancement and the liquefied area of the lesion without enhancement.This case indicates that a primary pulmonary synovial sarcoma is extremely rare and malignant,its prognosis is poor.There are some relatively specific imaging findings.展开更多
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 ...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.展开更多
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 join...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.展开更多
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 t...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.展开更多
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, immunohistoche...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.展开更多
BACKGROUND In patients with metastatic colorectal cancer(mCRC),the treatment options are limited and have been proved to be affected by rat sarcoma virus(RAS)mutational status.In RAS wild-type(wt)patients,the combinat...BACKGROUND In patients with metastatic colorectal cancer(mCRC),the treatment options are limited and have been proved to be affected by rat sarcoma virus(RAS)mutational status.In RAS wild-type(wt)patients,the combination of antiepidermal growth factor receptor(EGFR)monoclonal antibodies with chemotherapy(CT)is more effective than CT alone.On the other hand,RAS-mutated patients are not eligible for treatment with anti-EGFR antibodies.CASE SUMMARY Eleven patients with initially RAS-mutated mCRC were followed from diagnosis to May 2022.At the time of cell-free DNA determination,five patients had undergone one CT line,five patients had undergone two CT lines,and one patient had undergone three CT lines(all in combination with bevacizumab).At the second and third treatment lines[second line(2L),third line(3L)],patients with neo-RAS wt received a combination of CT and cetuximab.In neo-RAS wt patients treated with anti-EGFR,our findings indicated an increase in progression-free survival for both 2L and 3L(14.5 mo,P=0.119 and 3.9 mo,P=0.882,respectively).Regarding 2L overall survival,we registered a slight increase in neo-RAS wt patients treated with anti-EGFR(33.6 mo vs 32.4 mo,P=0.385).At data cut-off,two patients were still alive:A RAS-mutated patient undergoing 3L treatment and a neo-RAS wt patient who received 2L treatment with anti-EGFR(ongoing).CONCLUSION Our case series demonstrated that monitoring RAS mutations in mCRC by liquid biopsy may provide an additional treatment line for neo-RAS wt patients.展开更多
BACKGROUND Primary renal synovial sarcoma (PRSS) is an extremely rare tumor with a poor prognosis. Its imaging and immunohistochemical characteristics may overlap with other renal tumors, which renders its early diagn...BACKGROUND Primary renal synovial sarcoma (PRSS) is an extremely rare tumor with a poor prognosis. Its imaging and immunohistochemical characteristics may overlap with other renal tumors, which renders its early diagnosis in a dilemma. The diagnosis of primary renal synovial sarcoma requires histopathology and the confirmation of SYT-SSX gene fusion using molecular techniques. Cases of primary renal synovial sarcoma have been previously reported in the literature. However, to our knowledge, primary renal allograft synovial sarcoma was never described. CASE SUMMARY A 43-year-old male patient who underwent kidney transplantation 9 months ago came to our hospital for regular follow-up. Traditional ultrasonography revealed multiple hypo-echo neoplasms in the renal allograft. Contrast-enhanced computed tomography (CECT) showed slightly hyper-density masses with slow homogeneous enhancement. Ultrasound-guided biopsy was conducted for accurate pathological diagnosis. The neoplasms were diagnosed as synovial sarcoma by pathological, immunohistochemical, and genetic analyses. Positron emission tomography/CT showed no evidence of metastasis. At approximately one week post biopsy, contrast-enhanced ultrasound was conducted to eliminate active hemorrhage. One month later, CECT showed that the biggest neoplasm grew from 3.3 cm to 5.7 cm in diameter. Parametric imaging was conducted with SonoLiver CAP to conduct further quantitative analysis, which showed that the enhancement pattern was heterogeneous hyper-vascular enhancement. Radical surgical resection of the whole renal allograft and ureter was conducted without additional adjuvant chemotherapy or external radiotherapy. Anlotinib was chosen for targeted therapy with a good response. CONCLUSION We propose multimodality imaging for accurate diagnosis of renal allograft synovial sarcoma especially when it is formed by spindle-shaped cells.展开更多
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 a...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.展开更多
Synovial sarcomas are rare tumors which most often present in the distal extremities of young adults and children. There are unique clinicopathologic features of this tumor which lend themselves to multidiscipli- nary...Synovial sarcomas are rare tumors which most often present in the distal extremities of young adults and children. There are unique clinicopathologic features of this tumor which lend themselves to multidiscipli- nary treatment and translational research. We present a case of a primarily cystic synovial sarcoma and pro- vide a thorough review of the available literature.展开更多
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 s...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.展开更多
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. Su...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.展开更多
Synovial sarcoma originating from the kidney is extremely rare. A 13-year-old</span><span style="font-family:Verdana;"> girl presented with a mild left flank pain of one-week duration, with no as...Synovial sarcoma originating from the kidney is extremely rare. A 13-year-old</span><span style="font-family:Verdana;"> 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.展开更多
Myeloid sarcoma(MS)is a rare neoplasm characterized by the proliferation of immature myeloid precursor cells outside the bone marrow.The pathogenesis of MS is complex and not completely understood.Moreover,it develops...Myeloid sarcoma(MS)is a rare neoplasm characterized by the proliferation of immature myeloid precursor cells outside the bone marrow.The pathogenesis of MS is complex and not completely understood.Moreover,it develops in any extramedullary site of the body.In this editorial,we discuss the article published by Li et al,which presents a clinical case involving a 32-year-old man who exhibited gingival inflammation in the maxillary region.It was initially diagnosed as periodontal disease.However,clinical evaluation revealed a firm,grayishwhite mass which underscored the need for comprehensive diagnostics to distinguish MS from other oral conditions.This article emphasizes the different clinical presentations of similar case studies in the literature,and highlights the difficulty in diagnosing oral MS due to its rarity and variability in clinical manifestation.The treatment of MS depends on the clinical presentation,tumor location,and the patient's response to conventional therapies.The various therapeutic options currently available are analyzed and discussed.Early intervention and multidisciplinary management are crucial for improving treatment outcomes.Increased awareness and education about the various clinical presentations of MS lead to earlier diagnosis and timely treatment,thereby enhancing patients'survival and quality of life.Continued research is essential for optimizing therapeutic strategies and addressing the challenges presented by this rare neoplasm.展开更多
文摘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.
文摘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.
文摘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.
文摘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”.
文摘Synovial sarcoma(SS) is genetically characterized by chromosomal translocation, which generates SYT-SSX fusion transcripts. Although SS can occur in any body part, primary gastric SS is substantially rare. Here we describe a detection of the fusion gene sequence of gastric SS in plasma cell-free DNA(cf DNA). A gastric submucosal tumor was detected in the stomach of a 27-year-old woman and diagnosed as SS. Candidate intronic primers were designed to detect the intronic fusion breakpoint and this fusion sequence was confirmed in intron 10 of SYT and intron 5 of SSX2 by genomic polymerase chain reaction(PCR) and direct sequencing. A locked nucleic acid(LNA) probe specificto the fusion sequence was designed for detecting the fusion sequence in plasma and the fusion sequence was detected in preoperative plasma cfD NA, while not detected in postoperative plasma cfD NA. This technique will be useful for monitoring translocation-derived diseases such as SS.
文摘BACKGROUND Synovial sarcoma,a rare mesenchymal tumor type with unclear histological origin and direction of differentiation,accounts for 6%–10%of soft tissue tumors.It is mainly located near the joints and tendons of the limbs,and occurs primarily in children or young adults.Primary renal synovial sarcoma(PRSS)is very rare,accounting for approximately 1%of synovial sarcomas.It is a spindle cell tumor type affecting mesenchymal tissue,and has morphological,genetic,and clinical characteristics,and a certain degree of epithelial differentiation.It is highly malignant and has the fourth highest incidence among soft tissue sarcomas.Here,we report a case of PRSS and share some valuable information about the disease.CASE SUMMARY A 54-year-old male patient was admitted to the hospital for a space-occupying lesion in the right kidney for 2 d upon ultrasound examination.The patient had no cold or fever;no frequency,urgency or pain of urination;and no other discomfort.The results of a hemogram,blood biochemistry,and tumor markers were in the normal range.The patient was examined by computed tomography(CT),which indicated the presence of a soft tissue density shadow with a diameter of approximately 6.8 cm in the right renal pelvis area,showing uneven enhancement.Ultrasound indicated a cystic solid mass of approximately 6.8 cm×6.5 cm in the right kidney,with an unclear boundary and irregular shape.Meanwhile,color Doppler flow imaging showed dotted blood flow signals in the periphery and interior.Contrast-enhanced ultrasound(CEUS)showed"slow in and fast out"hyperenhancement of the right renal mass after contrast agent injection.The postoperative pathological diagnosis was(right kidney)synovial sarcoma.Despite postoperative adjuvant chemotherapy,tumor recurrence was detected two years later.CONCLUSION PRSS is a rare malignant tumor.To date,no characteristic imaging findings have been observed.The diagnosis is confirmed primarily through postoperative pathological immunohistochemistry and SS18(SYT)gene detection.In this case,CEUS was used preoperatively.We found that PRSS has the characteristic of"slow in and fast out"hyperenhancement,and its particular characteristics have diagnostic value.Postoperative adjuvant chemotherapy is not very effective.
文摘BACKGROUND Synovial sarcoma(SS) is a rare type of soft tissue sarcoma that is usually developed from areas where synovial tissue exists, especially at the extremities.Nevertheless, several cases of retroperitoneal SS(RSS) have been described. We herein report a case of RSS presented in our institution.CASE SUMMARY A 69-year-old female patient was admitted with a large, palpable, firm mass in the right abdominal space SS. Computerized tomography scan depicted a concentric, sharply marinated retro-peritoneal lesion which was displacing the right kidney and the lower edge of the liver. Subsequently, the patient underwent surgical excision of the mass with additional right nephrectomy and resection of the right adrenal gland and a part of the diaphragm. The final histological diagnosis of the tumour was grade II monophasic RSS.CONCLUSION RSS is encountered in the biphasic type, the monophasic fibrous, and the monophasic epithelial category as well. Relevant clinical manifestations are not always documented at early stages. Therefore, the final diagnosis is posed after complete histological examination taking into consideration the results of immunochemistry and genetic analysis. Therapeutic approach happens often late when metastases at the lungs and the liver are apparent. Thus, 5-year survival rates remain low.
文摘We describe a case of primary pulmonary synovial sarcoma arising in the lung of a 35-year-old woman.The tumor was a single mass and had a well-defined pleural-based with homogeneous density in the edge of the middle lobe,with no calcification and a fat component on plain CT scan.On the contrast-enhanced CT scan,the tumor showed moderately enhancement and circular enhancement with no enlargement of mediastinal,hilar lymph nodes.Skeleton single photon emission CT(ECT) was unremarkable.Gross and histological findings by postoperative pathology showed the tumor had pseudo-capsule and was well-circumscribed with foci of necrosis,hemorrhage and cystic change,under microscope tumor was typically mitotically active,spindle cells growing in intersecting fascicles or in solid sheets with epithelial differentiation.The immunohistochemical examination of CD99,Vim,PCK and EMA were positive.The tumor recurred with multiple pleural nodules and mass at 5 months after surgery,chest CT revealed the tumor was in the ipsilateral costophrenic angle for chest pain of the patient.After dynamic contrast-enhanced,the solid parts and the capsule of the lesion showed progressive enhancement and the liquefied area of the lesion without enhancement.This case indicates that a primary pulmonary synovial sarcoma is extremely rare and malignant,its prognosis is poor.There are some relatively specific imaging findings.
基金Supported by the Science and Technology Planning Project of Xingtai,No.2019ZC246.
文摘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.
基金the National Natural Science Foundation of China,No.81971085.
文摘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.
文摘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.
文摘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.
文摘BACKGROUND In patients with metastatic colorectal cancer(mCRC),the treatment options are limited and have been proved to be affected by rat sarcoma virus(RAS)mutational status.In RAS wild-type(wt)patients,the combination of antiepidermal growth factor receptor(EGFR)monoclonal antibodies with chemotherapy(CT)is more effective than CT alone.On the other hand,RAS-mutated patients are not eligible for treatment with anti-EGFR antibodies.CASE SUMMARY Eleven patients with initially RAS-mutated mCRC were followed from diagnosis to May 2022.At the time of cell-free DNA determination,five patients had undergone one CT line,five patients had undergone two CT lines,and one patient had undergone three CT lines(all in combination with bevacizumab).At the second and third treatment lines[second line(2L),third line(3L)],patients with neo-RAS wt received a combination of CT and cetuximab.In neo-RAS wt patients treated with anti-EGFR,our findings indicated an increase in progression-free survival for both 2L and 3L(14.5 mo,P=0.119 and 3.9 mo,P=0.882,respectively).Regarding 2L overall survival,we registered a slight increase in neo-RAS wt patients treated with anti-EGFR(33.6 mo vs 32.4 mo,P=0.385).At data cut-off,two patients were still alive:A RAS-mutated patient undergoing 3L treatment and a neo-RAS wt patient who received 2L treatment with anti-EGFR(ongoing).CONCLUSION Our case series demonstrated that monitoring RAS mutations in mCRC by liquid biopsy may provide an additional treatment line for neo-RAS wt patients.
基金Supported by Funding from the Beijing Municipal Administration of Hospitals’ Ascent Plan,No.DFL20180102
文摘BACKGROUND Primary renal synovial sarcoma (PRSS) is an extremely rare tumor with a poor prognosis. Its imaging and immunohistochemical characteristics may overlap with other renal tumors, which renders its early diagnosis in a dilemma. The diagnosis of primary renal synovial sarcoma requires histopathology and the confirmation of SYT-SSX gene fusion using molecular techniques. Cases of primary renal synovial sarcoma have been previously reported in the literature. However, to our knowledge, primary renal allograft synovial sarcoma was never described. CASE SUMMARY A 43-year-old male patient who underwent kidney transplantation 9 months ago came to our hospital for regular follow-up. Traditional ultrasonography revealed multiple hypo-echo neoplasms in the renal allograft. Contrast-enhanced computed tomography (CECT) showed slightly hyper-density masses with slow homogeneous enhancement. Ultrasound-guided biopsy was conducted for accurate pathological diagnosis. The neoplasms were diagnosed as synovial sarcoma by pathological, immunohistochemical, and genetic analyses. Positron emission tomography/CT showed no evidence of metastasis. At approximately one week post biopsy, contrast-enhanced ultrasound was conducted to eliminate active hemorrhage. One month later, CECT showed that the biggest neoplasm grew from 3.3 cm to 5.7 cm in diameter. Parametric imaging was conducted with SonoLiver CAP to conduct further quantitative analysis, which showed that the enhancement pattern was heterogeneous hyper-vascular enhancement. Radical surgical resection of the whole renal allograft and ureter was conducted without additional adjuvant chemotherapy or external radiotherapy. Anlotinib was chosen for targeted therapy with a good response. CONCLUSION We propose multimodality imaging for accurate diagnosis of renal allograft synovial sarcoma especially when it is formed by spindle-shaped cells.
文摘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.
文摘Synovial sarcomas are rare tumors which most often present in the distal extremities of young adults and children. There are unique clinicopathologic features of this tumor which lend themselves to multidiscipli- nary treatment and translational research. We present a case of a primarily cystic synovial sarcoma and pro- vide a thorough review of the available literature.
文摘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.
文摘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.
文摘Synovial sarcoma originating from the kidney is extremely rare. A 13-year-old</span><span style="font-family:Verdana;"> 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.
文摘Myeloid sarcoma(MS)is a rare neoplasm characterized by the proliferation of immature myeloid precursor cells outside the bone marrow.The pathogenesis of MS is complex and not completely understood.Moreover,it develops in any extramedullary site of the body.In this editorial,we discuss the article published by Li et al,which presents a clinical case involving a 32-year-old man who exhibited gingival inflammation in the maxillary region.It was initially diagnosed as periodontal disease.However,clinical evaluation revealed a firm,grayishwhite mass which underscored the need for comprehensive diagnostics to distinguish MS from other oral conditions.This article emphasizes the different clinical presentations of similar case studies in the literature,and highlights the difficulty in diagnosing oral MS due to its rarity and variability in clinical manifestation.The treatment of MS depends on the clinical presentation,tumor location,and the patient's response to conventional therapies.The various therapeutic options currently available are analyzed and discussed.Early intervention and multidisciplinary management are crucial for improving treatment outcomes.Increased awareness and education about the various clinical presentations of MS lead to earlier diagnosis and timely treatment,thereby enhancing patients'survival and quality of life.Continued research is essential for optimizing therapeutic strategies and addressing the challenges presented by this rare neoplasm.