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Ultrastructural Pathology May 2024Synovial sarcoma (SS) and solitary fibrous tumor (SFT) are entities with considerable morphological and immunohistochemical similarities that sometimes show a... (Comparative Study)
Comparative Study
Synovial sarcoma (SS) and solitary fibrous tumor (SFT) are entities with considerable morphological and immunohistochemical similarities that sometimes show a non-confirmatory profile (TLE1 negative, CD34 and focal or negative STAT6 and lack of specific fusion IHC markers), in which the utility ultrastructure is unknown. A cross-sectional, retrospective, analytical, nonexperimental study was carried out by the Department of Pathology of the National Cancer Institute of Mexico (INCan) e from January 1, 2009 to December 31, 2018. With 17 SFT cases with diffuse or focal CD34 and STAT6 positivity and 18 cases of SS with positive FISH molecular test t(X:18) breakapart were studied by electron microscopy of fresh glutaraldehyde fixed or paraffin-embedded tissue. The ultrastructural findings with a significant difference present in the SS were tandem tight junctions, desmosomes and abundance of dilated rough endoplasmic reticulum (RER) cisternae ( < 0.001, 0.003, and 0.001, respectively); while in the (SFT) the presence of abundant glycogen, basal lamina, long and slender cytoplasmic processes, pinocytic vesicles, hemidesmosomes, and/or dense plaques, collagen skein, and microvilli-like buds ( = 0.028, 0.005, and <0.001 for the last five). We then infer that the five distinctive markers of the SFT are the collagen skeins intermingled with cellular processes in a shape of "squid can," and the pinocytic vesicles as they were not observed in any case of SS. Conversely, tandem junctions were not found in any SFT case. Although the presence of multivesicular buds in the SFT was not significant, it had not been previously described.
Topics: Humans; Solitary Fibrous Tumors; Sarcoma, Synovial; Adult; Male; Female; Retrospective Studies; Middle Aged; Mexico; Cross-Sectional Studies; Biomarkers, Tumor; Aged; Young Adult; Diagnosis, Differential
PubMed: 38348820
DOI: 10.1080/01913123.2024.2313742 -
BMC Musculoskeletal Disorders Feb 2024Malignant femoral soft tissue tumors are occasionally resected together with the femoral nerves, but this can cause loss of knee extensor muscle activity. To the best of...
BACKGROUND
Malignant femoral soft tissue tumors are occasionally resected together with the femoral nerves, but this can cause loss of knee extensor muscle activity. To the best of our knowledge, no previous reports have detailed the gait analysis of such cases in combination with electromyography. Herein, we report the gait analysis of a patient who underwent left groin synovial sarcoma and left femoral nerve resection 12 years ago.
CASE PRESENTATION
We analyzed the gait of a 38-year-old man who was able to walk unaided after the resection of a synovial sarcoma in the left groin together with the ipsilateral femoral nerve. The muscle activities of the affected medial (MH) and lateral hamstrings (LH), and lateral heads of the gastrocnemius (GL) were increased during 50-75% of the stance phase. The hip flexion angle of the affected limb was smaller, and the ankle plantar flexion angle of the affected limb was larger than that of the non-affected limb. This means that in the affected limb, the hip and ankle angles were adjusted to prevent knee collapse, and the MH, LH, and GL muscles contributed in the mid- and late-stance phases. Moreover, we found that the hamstring and gastrocnemius of the affected limb worked together to keep the ipsilateral knee extended in the mid-stance phase and slightly flexed in the late-stance phase.
CONCLUSIONS
Patients capable of walking after femoral nerve resection may control their hamstrings and gastrocnemius muscles collaboratively to prevent ipsilateral knee collapse in the mid- and late-stance phases.
Topics: Male; Humans; Adult; Femoral Nerve; Gait Analysis; Sarcoma, Synovial; Gait; Walking; Knee Joint; Muscle, Skeletal; Sarcoma; Biomechanical Phenomena
PubMed: 38347547
DOI: 10.1186/s12891-024-07258-8 -
Annales de Pathologie May 2024Synovialosarcoma is a malignant mesenchymal tumor of young adults that occurs in the deep soft tissues, particularly around large joints. When it occurs in more unusual...
Synovialosarcoma is a malignant mesenchymal tumor of young adults that occurs in the deep soft tissues, particularly around large joints. When it occurs in more unusual sites, it could present a significant diagnostic challenge. In this case, a 19-year-old girl was treated for a pyloric mass. A pyelic urine cytology performed simultaneously with a pyloric biopsy proved to be a significant element of orientation and perfectly concordant with the histopathological aspect of the pyelic mass after nephrectomy. We report here the first case of renal synovialosarcoma documented in pyelic urine.
Topics: Humans; Female; Sarcoma, Synovial; Kidney Neoplasms; Young Adult; Nephrectomy; Biopsy; Diagnosis, Differential; Urine; Cytology
PubMed: 38342653
DOI: 10.1016/j.annpat.2023.12.002 -
Asian Journal of Surgery May 2024
Topics: Humans; Gastrectomy; Sarcoma, Synovial; Stomach Neoplasms
PubMed: 38331620
DOI: 10.1016/j.asjsur.2024.01.159 -
Clinical Oncology (Royal College of... Apr 2024Synovial sarcoma is a rare but aggressive variant of soft-tissue sarcoma. Literature is sparse and reported mostly from the West. We analysed the clinical profiles and...
AIMS
Synovial sarcoma is a rare but aggressive variant of soft-tissue sarcoma. Literature is sparse and reported mostly from the West. We analysed the clinical profiles and prognostic factors of extremity synovial sarcoma patients in order to study their clinical journey.
MATERIALS AND METHODS
This was a retrospective analysis. All patients with extremity synovial sarcoma treated between 1992 and 2020 were included. Patients with metastases at presentation were excluded. A descriptive analysis of demographic and clinicopathological features of patients undergoing limb salvage surgery (LSS) or amputation was carried out. Overall survival and disease-free survival were calculated for the entire cohort as well as for the LSS and amputation groups. Factors prognostic for survival were identified.
RESULTS
In total, 157 patients had localised extremity synovial sarcoma. Predominantly, young adults (median 31 years) and males (61%) were affected. Over 70% of patients presented after recurrence or unplanned surgeries. Sixty-seven per cent of tumours were >5 cm, 69% were deep and 23% involved bone. The limb salvage rate was 64%. In the LSS group, adjuvant radiotherapy and chemotherapy were given to 72% and 68% of patients, respectively. In the amputation group, 72% of patients received adjuvant chemotherapy. In a median follow-up of 59 months, 39.4% of patients had recurrences, the majority (61.2%) were systemic. Five-year overall survival and disease-free survival were 53.4% and 49.8%, respectively. Overall survival was 63.9% and 29.7% in the LSS and amputation groups, respectively. On multivariate analysis, tumour size, depth, omission of radiotherapy and bone invasion were found to be the adverse prognostic factors.
CONCLUSION
This is one of the largest studies on extremity synovial sarcoma. Mostly males and young adults were affected. The limb salvage rate was 64%, despite most being referred after unplanned surgery. Almost 70% of patients received radiotherapy and chemotherapy. Overall survival was inferior in the amputation group. Tumour size >5 cm, depth and bone invasion were negative, whereas adjuvant radiotherapy was a positive prognostic factor for survival. Chemotherapy had no impact on survival.
Topics: Male; Young Adult; Humans; Female; Sarcoma, Synovial; Retrospective Studies; Sarcoma; Extremities; Prognosis; Soft Tissue Neoplasms; Neoplasm Recurrence, Local
PubMed: 38326122
DOI: 10.1016/j.clon.2024.01.018 -
Thoracic Cancer Mar 2024Pericardial synovial sarcomas (PSS) have a low incidence rate and are highly invasive with a dismal prognosis. Standard treatment includes surgery, radiotherapy and...
Pericardial synovial sarcomas (PSS) have a low incidence rate and are highly invasive with a dismal prognosis. Standard treatment includes surgery, radiotherapy and chemotherapy but with limited response. Here, we report the case of a 15-year-old nonsmoking youngster diagnosed with PSS who developed disease relapsed from surgery after 1 month. Next-generation sequencing (NGS) using baseline tissue was performed, and BRCA2 c.968dupT was detected. Then pazopanib (a multitargeted inhibitor) plus nivolumab (an immune checkpoint inhibitor) was administered, with a partial response and progression-free survival of 14 months. BRCA2 c.968dupT has not previously been reported in PSS and its response to targeted combination immunotherapy are not well characterized. Here, we report the efficacy of pazopanib combined with nivolumab in a PSS patient harboring BRCA2 c.968dupT and also provide the clinical evidence of the utility of NGS in exploring actionable mutations for solid tumor. Combination therapy based on immunotherapy may be a potential treatment choice for PSS harboring BRCA2 mutation.
Topics: Humans; Adolescent; Sarcoma, Synovial; Nivolumab; Pyrimidines; Sulfonamides; Mediastinal Neoplasms; Thymus Neoplasms; Heart Neoplasms; Pleural Neoplasms; BRCA2 Protein; Indazoles
PubMed: 38323364
DOI: 10.1111/1759-7714.15237 -
Hinyokika Kiyo. Acta Urologica Japonica Jan 2024We experienced two cases of renal primary synovial sarcoma. Case 1: A 29-year-old man underwent laparoscopic radical nephrectomy and was originally diagnosed with renal...
We experienced two cases of renal primary synovial sarcoma. Case 1: A 29-year-old man underwent laparoscopic radical nephrectomy and was originally diagnosed with renal cell carcinoma. Case 2: A 25-year-old man was treated by open radical nephrectomy since radiographical findings indicated tumor invasion to the ureter causing hydronephrosis. Both cases were pathologically diagnosed as renal synovial sarcomas, and were followed using computed tomography. Recurrence was observed within a year in both cases.
Topics: Male; Humans; Adult; Sarcoma, Synovial; Kidney Neoplasms; Retroperitoneal Neoplasms; Carcinoma, Renal Cell; Kidney; Nephrectomy; Laparoscopy
PubMed: 38321742
DOI: 10.14989/ActaUrolJap_70_1_1 -
Journal of Maxillofacial and Oral... Feb 2024
PubMed: 38312967
DOI: 10.1007/s12663-023-01909-4 -
Mediterranean Journal of Rheumatology Dec 2023Soft tissue sarcomas are rare and often go undetected until a later stage, particularly when they present as intra-articular or tenosynovial lesions mimicking benign...
BACKGROUND
Soft tissue sarcomas are rare and often go undetected until a later stage, particularly when they present as intra-articular or tenosynovial lesions mimicking benign synovial pathologies. The failure to distinguish between malignant and benign synovial disease can have a significant impact on patient outcomes and limit alternatives for local control surgery and limb salvage.
CASE DESCRIPTION
In this case series, we present two cases of soft tissue sarcomas, one being an intraarticular synovial chondrosarcoma, and the other a pleomorphic spindle cell sarcoma centred along tendon sheaths. Radiologically, the initial clinical presentation of these cases resembled benign synovial pathologies, leading to a delay in diagnosis and treatment.
CONCLUSION
Our study underscores the importance of maintaining a low threshold of suspicion for surveillance, a multidisciplinary approach, and early histological diagnosis to ensure appropriate timely treatment and a favourable prognosis for patients with soft tissue sarcomas.
PubMed: 38282941
DOI: 10.31138/mjr.270823.sts -
Frontiers in Oncology 2023Synovial sarcoma is a malignant tumor of mesenchymal origin with a high degree of malignancy and easy metastasis. It mostly occurs in distal extremities or adjacent...
BACKGROUND
Synovial sarcoma is a malignant tumor of mesenchymal origin with a high degree of malignancy and easy metastasis. It mostly occurs in distal extremities or adjacent joints, and it is most common in deep knee joint. Primary sacral synovial sarcoma (PSSS) is extremely rare. The PET/CT imaging findings of a case of monophasic PSSS were reported. The clinical, imaging, and pathological data were summarized, and the literature was reviewed.
CASE DESCRIPTION
A 67-year-old female patient presented with sacrococcygeal pain without obvious causes on 16 September 2022, with occasional pain at night, as well as aggravated pain during hip flexion and long-distance walking, which could be slightly relieved with rest, without special treatment. For further treatment before 1 month to Jiangxi Provincial People's Hospital, after admission, laboratory tests were negative. Non-contrast CT scan showed expansive bone destruction in the S1-3 vertebrae with soft tissue density of about 58 mm × 46 mm × 52 mm. The boundary was clear, necrosis was visible within the vertebrae, and the boundary between the mass and the anterior sacral blood vessels and rectum was unclear. Non-contrast MRI scan showed mixed signals in lumbosacral masses, with equal signals in T1 and uneven and slightly higher signals in T2. Cystic degeneration and necrosis were visible, with multiple compartments in the lumbosacral masses. MRI enhancement showed uneven enhancement of lumbosacral mass with multiple compartments and no enhanced cystic lesion. The left sacral alar bone is destroyed, as shown by large flaky uneven strengthening. PET/CT showed that S1-3 vertebral body and left sacral alar bone were destroyed and soft tissue shadow formed, invading the sacral canal and the left foramina of S1-3. FDG metabolism was significantly increased, and malignant tumor was diagnosed by PET/CT. Pathological examination: The pathological diagnosis was monophasic PSSS. After systemic chemotherapy and local radiotherapy, no significant signs of recurrence and metastasis were found on CT so far. Follow-up treatment was continued.
CONCLUSION
The incidence of PSSS is very low, its clinical and imaging manifestations lack characteristics, and the final diagnosis still needs pathology. PET/CT imaging has a certain value in the diagnosis of PSSS and has great application value in the preoperative staging, postoperative efficacy evaluation, and follow-up.
PubMed: 38264744
DOI: 10.3389/fonc.2023.1309123