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Pediatric Emergency Care Mar 2024Synovial cell sarcoma is a rare mesenchymal tumor that typically originates from the soft tissues of the extremities of young adults. Only 3 cases of primary...
Synovial cell sarcoma is a rare mesenchymal tumor that typically originates from the soft tissues of the extremities of young adults. Only 3 cases of primary diaphragmatic synovial cell sarcoma have been described in the literature: 2 in adult males and 1 in a 12-year-old pediatric patient.1-3 When this tumor is found in the mediastinum or pericardial region, prognosis is historically poor because of the advanced disease stage at time of diagnosis. The surgical course and pathology have been described in this 12-year-old boy.3 This is the first case, to our knowledge, of the use of cardiac point-of-care ultrasound in the early identification and diagnosis of a primary diaphragmatic synovial sarcoma in a pediatric patient.
PubMed: 38471767
DOI: 10.1097/PEC.0000000000003149 -
EFORT Open Reviews Mar 2024Synovial sarcoma is a rare and highly malignant soft tissue sarcoma. The inconspicuous and diversity of its early symptoms make it a highly misdiagnosed disease. The... (Review)
Review
Synovial sarcoma is a rare and highly malignant soft tissue sarcoma. The inconspicuous and diversity of its early symptoms make it a highly misdiagnosed disease. The management of synovial sarcomas is challenging as they are rare and have a poor prognosis. Early and correct diagnosis and treatment are critical for clinical outcomes. Misdiagnosis or delayed diagnosis can have devastating consequences for the patient. The detection of SS18 gene rearrangement is considered a powerful tool in establishing the diagnosis of synovial sarcomas. Biopsies and testing for gene rearrangements are recommended for all patients in whom SS cannot be excluded. Surgery is the mainstay of treatment for synovial sarcomas. Neoadjuvant/adjuvant radiotherapy is recommended for patients with big tumors (>5 cm) or positive resection margins, and neoadjuvant/adjuvant chemotherapy is recommended for patients with high-risk tumors or advanced diseases. This article reviews synovial sarcomas from the perspectives of clinical and radiological presentation, histological and cytogenetic analysis, differential diagnosis, treatment, and prognosis.
PubMed: 38457918
DOI: 10.1530/EOR-23-0193 -
Journal of Cranio-maxillo-facial... Jan 2024The research purpose is to review the surgical approach and evaluate the results in adult patients with head and neck sarcomas. The histopathology varied, including two...
The research purpose is to review the surgical approach and evaluate the results in adult patients with head and neck sarcomas. The histopathology varied, including two leiomyosarcomas, six malignant fibrous histiocytomas, two malignant peripheral nerve sheath tumors, four dermatofibrosarcomas protuberans, three osteosarcomas, two angiosarcomas, one liposarcoma, one Ewing sarcoma, one synovial sarcoma, two unclassified/non-differentiated sarcomas and one solitary fibrous tumor. Surgical resection included maxillectomy, mandibulectomy, craniectomy, parotidectomy, scalp resection, face skin resection and laminectomy. The reconstruction was performed with one rectus abdominis flap, four radial forearm flaps, two latissimus dorsi flaps, two vascularized fibula flaps, two pectoralis major myocutaneous flaps, two trapezius flaps, two temporalis flaps, seven scalp flaps and two nasolabial flaps. The total patient number was 24. The hospitalization was uncomplicated, followed by postoperative radiotherapy in the majority of cases. In a mean 15-year follow-up period, 11 patients are still alive and disease-free. There were four recurrences treated with palliative radiotherapy. The surgical approach for head and neck sarcomas, including the achievement of a functionally acceptable result by organ sparing techniques, remains challenging. Wide resection combined with the appropriate reconstruction, particularly with microsurgical techniques, and followed by adjuvant radiotherapy or chemotherapy offer improved prognosis and quality of life.
PubMed: 38443189
DOI: 10.1016/j.jcms.2024.01.005 -
Cardiology Journal 2024
Topics: Humans; Mediastinum; Sarcoma, Synovial
PubMed: 38436487
DOI: 10.5603/cj.92292 -
Frontiers in Oncology 2024Synovial sarcomas (SSs) are a rare group of malignant tumors originating from pluripotential mesenchymal cells, which commonly occur as the primary tumor in the soft...
Synovial sarcomas (SSs) are a rare group of malignant tumors originating from pluripotential mesenchymal cells, which commonly occur as the primary tumor in the soft tissues near the articular surface, tendons, and articular synovium. Herein, we report a rare case of mediastinal SS in an 18-year-old teenager who initially presented with cough as the primary symptom. In this case, plain chest CT and contrast-enhanced CT clearly revealed the lesion presenting as a round-like and uneven density mass in the mediastinum with heterogeneous enhancement, which compressed the trachea and invaded the adjacent vessels. Based on the results of immunohistochemistry and fluorescence hybridization (FISH), combined with the differential diagnosis with other types of tumors in the mediastinum on imaging, we were able to diagnose the tumor as an SS located in the mediastinum. Subsequent resection of the lesion coupled with chemotherapy and immunotherapy led to an improvement in the patient's symptoms.
PubMed: 38434682
DOI: 10.3389/fonc.2024.1288213 -
Pediatric Blood & Cancer May 2024In complex pediatric surgical oncology, surgical planning is contingent upon data gathered from preoperative imaging. Three-dimensional (3D) modeling and printing has...
INTRODUCTION
In complex pediatric surgical oncology, surgical planning is contingent upon data gathered from preoperative imaging. Three-dimensional (3D) modeling and printing has been shown to be beneficial for adult presurgical planning, though pediatric literature is less robust. The study reviews our institutional experience with the use of 3D image segmentation and printed models in approaching resection of extracranial solid tumors in children.
METHODS
This is a single institutional series from 2021 to 2023. Models were based on computed tomography and magnetic resonance imaging studies, optimized for 3D imaging. The feasibility and creation of the models is reviewed, including specific techniques, software, and printing materials from our institution. Clinical implications for surgical planning are also described, along with detailed preoperative and intraoperative images.
RESULTS
3D modeling and printing was performed for four pediatric patients diagnosed with extracranial solid tumors. Diagnoses included Ewing sarcoma, hepatoblastoma, synovial sarcoma, and osteosarcoma. No intraoperative complications or discrepancies with the preoperative 3D-printed model were noted. No evidence of local recurrence was identified in any patient thus far.
CONCLUSION
Our institutional series demonstrates a wide spectrum of clinical application for 3D modeling and printing technology within pediatric surgical oncology. This technology may aid in surgical planning for both resection and reconstruction, can be applied to a diverse breadth of diagnoses, and may potentially augment patient and/or family education about their condition.
Topics: Child; Humans; Imaging, Three-Dimensional; Magnetic Resonance Imaging; Sarcoma, Ewing; Sarcoma, Synovial; Tomography, X-Ray Computed
PubMed: 38430473
DOI: 10.1002/pbc.30933 -
JNMA; Journal of the Nepal Medical... Jan 2024Synovial sarcoma is a mesenchymal tumour with partial epithelial differentiation. About 85-90% of SS occur in the extremities. We present a case of a 44-year-old woman...
UNLABELLED
Synovial sarcoma is a mesenchymal tumour with partial epithelial differentiation. About 85-90% of SS occur in the extremities. We present a case of a 44-year-old woman diagnosed with recurrent synovial sarcoma with breast and pulmonary nodules. The primary treatment for synovial sarcoma is wide surgical excision, while chemotherapy is reserved for metastatic cases. In the first-line metastatic setting, combination treatment with adriamycin and ifosfamide is administered. Despite the unfavourable prognosis, the patient's extended survival is fortunately not the typical outcome.
KEYWORDS
case reports; chemotherapy; immunohistochemistry; synovial sarcoma.
Topics: Female; Humans; Adult; Sarcoma, Synovial; Ifosfamide; Doxorubicin; Prognosis; Combined Modality Therapy
PubMed: 38410005
DOI: 10.31729/jnma.8408 -
Diagnostics (Basel, Switzerland) Feb 2024Primary pulmonary synovial sarcoma is a rare type of soft tissue tumor. Exceptionally it can occur during pregnancy, representing a challenge in management and treatment...
Primary pulmonary synovial sarcoma is a rare type of soft tissue tumor. Exceptionally it can occur during pregnancy, representing a challenge in management and treatment given its notable aggressiveness and the not infrequent incidence of maternal death. We report our case of metastatic recurrence of pulmonary synovial sarcoma during pregnancy, with the aim to emphasize the decision-making, diagnostic, and therapeutic multidisciplinary processes and the evolution of the pathology. Besides, we focused on the analysis of the limited literature data available on the topic.
PubMed: 38396463
DOI: 10.3390/diagnostics14040424 -
Indian Journal of Pathology &... Apr 2024Synovial sarcoma (SS) is rarely documented in the female genital tract, especially confirmed by molecular testing for SYT::SSX translocation and TLE1 immunostaining. A... (Review)
Review
Poorly differentiated biphasic synovial sarcoma of the vulva, displaying SS18::SSX1 fusion and weak to absent (mosaic) INI1/SMARCB1 immunostaining: A rare case with literature review.
Synovial sarcoma (SS) is rarely documented in the female genital tract, especially confirmed by molecular testing for SYT::SSX translocation and TLE1 immunostaining. A 62-year-old lady presented with a progressively increasing lump and pain over her right groin, for 6-month duration. Radiologically, a well-defined, solid-cystic mass was seen involving the right labia with necrotic areas, sparing the underlying muscles and the overlying skin. She underwent a biopsy followed by a surgical excision. Histopathologic examination revealed a spindle cell sarcoma, including tumor cells exhibiting a prominent hemangiopericytomatous pattern. There were focal areas of epithelial differentiation (pseudoglandular) along with areas of round cell morphology and increased mitoses (poor differentiation) in the resected specimen. Immunohistochemically, the tumor cells were diffusely positive for TLE1, patchily positive for pan keratin (AE1/AE3) and EMA, the latter more in the areas of epithelial differentiation, while negative for CD34, SMA, desmin, S100P, and SOX10. INI1/SMARCB1 showed a characteristic weak to absent (mosaic) staining pattern. Furthermore, the tumor displayed SS18::SSX 1 fusion by RT-PCR. This constitutes one of the few reported cases of vulvar SS, confirmed by molecular testing and the first documented vulvar SS showing a mosaic pattern of INI1/SMARCB1 immunostaining. A review of the literature and diagnostic implications are presented herewith.
Topics: Humans; Female; Sarcoma, Synovial; Middle Aged; SMARCB1 Protein; Immunohistochemistry; Vulvar Neoplasms; Vulva; Oncogene Proteins, Fusion; Biomarkers, Tumor; Histocytochemistry; Microscopy; Co-Repressor Proteins; Proto-Oncogene Proteins; Repressor Proteins
PubMed: 38391333
DOI: 10.4103/ijpm.ijpm_560_23 -
Asia-Pacific Journal of Clinical... Jun 2024Adult soft tissue sarcomas (STS) are rare and diverse. Current management is based on limited literature from the West. Therefore, data from different geographical...
BACKGROUND
Adult soft tissue sarcomas (STS) are rare and diverse. Current management is based on limited literature from the West. Therefore, data from different geographical regions is required, including the low-middle-income countries. This is our experience managing adult sarcomas in the tertiary cancer center of North India.
MATERIALS AND METHODS
This is a retrospective analysis of the structured sarcoma database of patients treated in the surgical oncology department between 1992 and 2020. The descriptive analysis includes demography, site distribution, diagnosis, histopathology variations, prior surgical interventions, and stage.
RESULTS
A total of 1106 soft tissue sarcoma patients were treated in three decades. Age distribution was 13%, 43%, 31%, and 11% in <20, 21-40, and 41-60 and >60 years, respectively. The male-to-female ratio was 1.73. The anatomical distribution was 17%, 42%, 23%, 7%, 7%, and 3% in upper extremity, lower extremity, trunk, retroperitoneum, head and neck, and viscera, respectively. Overall, 49% of patients had undergone prior suboptimal surgeries at community hospitals. Common histology subtypes were synovial sarcoma (18%), undifferentiated pleomorphic sarcoma (UPS) (13%), dermatofibrosarcoma protuberans (12%), and liposarcoma (9%). A pathological discordance of 13% was identified between the initial and the final histologies. Overall, 61% of tumors were high-grade. Memorial Sloan Kettering Stages II and III were present in 33% and 35% of patients, respectively.
CONCLUSIONS
This is one of the largest single institutional experiences of STS from the Asian population. Mostly young adults were affected with male preponderance. The lower extremity and trunk were common subsites. Frequent histologies were synovial sarcoma and UPS. A high rate of suboptimal surgical intervention at the community level and pathological discordance was noted. This study highlights the need to establish prospective structured databases for capturing quality information related to rare malignancies and providing insights for future research.
Topics: Humans; Male; Female; Adult; Sarcoma; India; Middle Aged; Retrospective Studies; Young Adult; Databases, Factual; Prospective Studies; Aged; Soft Tissue Neoplasms
PubMed: 38383968
DOI: 10.1111/ajco.14050