Synovial Sarcoma Presenting Research Articles

Unprecedented presentation of pelvic synovial sarcoma: A compelling case report

Synovial sarcoma (SS) is a rare tumor that typically arises in various body regions, with its manifestation in the abdominal-pelvic area being exceptionally uncommon. However, despite its rarity in this location, it often metastasizes to the thorax. Despite its slow growth, it can be misdiagnosed as benign in less than 10% of cases. Diagnosis necessitates identification of the SYT-SSX fusion transcript, alongside immunohistochemical and cytogenetic assessments. Treatment involves surgical resection followed by radiotherapy for localized tumors, while chemotherapy is necessary for metastatic cases. Despite treatment, synovial sarcomas have a high recurrence rate, with about half recurring within two years. We report the case of a 73-year-old patient from sub-Saharan Africa who presented to the emergency room with obstructive symptoms, revealing a mass near the anus compressing the rectum upon imaging, causing an obstructive syndrome. Despite initial tumor resection, histological analysis identified a neurofibromatous origin. Twenty months later, recurrence prompted a hospital visit, revealing thoracic metastases necessitating surgery and chemotherapy. Subsequent analysis confirmed perianal synovial sarcoma. This case underscores the rarity of pelvic-anal synovial sarcoma and emphasizes the importance of early detection and proper management. Synovial sarcoma should figure prominently on the list of differential diagnoses of high-grade pelvic masses due to the importance of adjuvant chemotherapy.

Case Report : Undifferentiated Synovial Sarcoma Presenting as a Large Thoracic Mass: A Rare and Challenging Diagnosis

Background: Undifferentiated synovial sarcoma (USS) is a rare and aggressive subtype of soft tissue sarcoma. Its presentation as a large thoracic mass poses significant diagnostic and therapeutic challenges.  Case Presentation: A 34-year-old male presented with a three-month history of progressive chest pain and hemoptysis. Imaging studies revealed a large, cystic thoracic mass with pleural effusion. Initial serological testing suggested hydatid cyst, but subsequent testing revealed negative Echinococcus (Hydatid) IgG antibodies.  Diagnostic and Therapeutic Interventions: Repeat fine-needle aspiration cytology (FNAC) was deferred due to revised ultrasound findings indicating a solid fibrocystic vascular lesion. Bronchial artery embolization (BAE) was performed to control life- threatening hemorrhage. Thoracotomy and en-bloc mass excision were subsequently undertaken.  Histopathological Diagnosis: Histopathological examination of the resected specimen revealed undifferentiated synovial sarcoma and spindle cell sarcoma.  Clinical Implications: This case highlights the importance of correlating imaging and serological findings, managing vascular complications, and the efficacy of BAE in controlling hemorrhage. The significance of thoracotomy and mass excision in achieving diagnostic clarity and therapeutic success is underscored.  Conclusion: Undifferentiated synovial sarcoma presenting as a large thoracic mass poses significant diagnostic and therapeutic challenges. A multidisciplinary approach, incorporating imaging, serological testing, and surgical intervention, is crucial for achieving optimal patient outcomes.

Rare presentation of primary intra-articular synovial sarcoma of knee joint with insights from literature review

ABSTRACT Synovial sarcoma is a rare and malignant soft tissue tumor, comprising 5%–10% of such cases. This tumor primarily afflicts adolescents and young adults, with slight male bias, often near large joints like the knee and hip. Intra-articular synovial sarcoma, an even rarer subset, can delay diagnosis due to its slow growth and vague clinical signs. We present a unique case of primary knee intra-articular synovial sarcoma in a 24-year-old female. Imaging revealed a distinct lesion adjacent to the medial femoral condyle and patellofemoral joint, initially suggestive of synovial haemangioma. However, histopathology confirmed biphasic synovial sarcoma, characterized by spindle and gland-like epithelial components. Immunohistochemistry further supported the diagnosis. Reviewing documented cases, we find that synovial sarcoma can affect a wide age range but often occurs in the knee joint, emphasizing the need for vigilance. Early detection and intervention are crucial for improved treatment outcomes in this rare but challenging malignancy.

Extremely rare presentation of primary jejunal monophasic synovial sarcoma

Extremely rare presentation of primary jejunal monophasic synovial sarcoma

Primary Renal Synovial Sarcoma Presenting as Metastatic Right Atrial Obstruction.

Primary Renal Synovial Sarcoma Presenting as Metastatic Right Atrial Obstruction.

Monophasic Synovial Sarcoma of Foot Masquerading Squamous Cell Carcinoma

Synovial sarcoma being a rare sarcoma typically arises near articular surface most commonly around knee joint. Hereby, a case report of a 38-year-old male with complaint of cauliflower-like proliferative growth on the plantar aspect of left foot. It rapidly progressed in size over a period of one month. Wedge biopsy was done and diagnosis of malignant mesenchymal tumour was suggested. Further clinical workup of the case was carried out and the surgeons proceeded with amputation of the foot. Gross examination of specimen showed a large, ulcerated, nodular, exophytic, cauliflower-like growth over the plantar aspect of left foot along with areas of haemorrhage and necrosis masquerading more common squamous cell carcinoma. Histopathology revealed characteristic hypocellular and hypercellular areas showing pleomorphic spindle cells in fascicles and whorls suggesting monophasic synovial sarcoma. Immunohistochemistry stains further confirmed the diagnosis. The current case report highlights the rare presentation of synovial sarcoma as a cauliflower type of growth at a rare location.

An Unusual Presentation of a Rare Tumor: Primary Renal Synovial Sarcoma

Primary renal synovial sarcomas are very rare and highly aggressive tumors. They are seen at an average age of 35 years. Common symptoms include side pain, abdominal pain, and hematuria. Imaging modalities have no specific findings indicating their presence, and the diagnosis is made based on immunohistochemical methods, which may need to be confirmed by cytogenetic analysis. Radical nephrectomy is the best treatment option currently available, with a combination of anthracyclines and ifosfamidine being administered as adjuvant chemotherapy. In this case report, we present a patient with a primary renal synovial sarcoma presenting with spontaneous massive retroperitoneal bleeding due to tumor rupture. To the best of our knowledge, this is only the second case of such clinical presentation reported in the literature.

Infratemporal fossa synovial sarcoma in a 3-month-old infant: an extremely rare tumour in infancy

Soft tissue sarcoma, especially synovial sarcoma, is extremely rare in infancy, with only a few cases reported worldwide. Due to the particularly small size of the head and neck region in infants, with important anatomical structures in close vicinity to one another, surgical resection is very challenging, if the goal is to ensure total disease elimination as well as maintaining function and good cosmetic outcome postoperatively. In the case reported here, following diagnosis, the child was given three cycles of neoadjuvant chemotherapy followed by infratemporal tumour resection via Bramley–Al-Kayat incision incorporated with a modified Blair incision. Postoperatively, the child recovered well, with no recurrence after three months. This case highlights the important role of a multidisciplinary approach in the diagnosis and managing such rare cases. We also discuss the clinical presentation, imaging features, histopathological features, treatment regime, and prognosis of synovial sarcoma of the infratemporal fossa in children, based on cases reported in the literature.

Pulmonary Synovial Sarcoma: An Unpleasant Surprise.

There has been tremendous progress in the diagnosis and management of malignant effusion, with thoracoscopic biopsy of the pleura providing the highest diagnostic yield.Even though advanced techniques such as image-guided biopsy and pleuroscopy have relatively high sensitivity and specificity, in rare cases, the diagnosis can still be missed.In clinical practice, all such techniques should be considered in order to achieve an early diagnosis and a better outcome.

Synovial Sarcoma: Ãâë Triple Sign Ãâû MRI Pattern

The synovial sarcoma (Figure 1) is a soft tissue tumour, first described by Jaffe in 1941 as a tumour that grows from the synovium of joints, bursae or tendon sheaths. It was later reported that it grew from undifferentiated mesenchymal cells. It can be localized or diffuse, intra or extra articular, with predominance in lower extremities, with the thigh as the most common location, followed by the popliteal fossa. It is usually diagnosed in adolescents or young adults, and because of its slow growth, it may mimic a benign pathology. Clinical symptoms usually include a palpable mass. Imaging is the examination of choice to lead diagnosis. Plain radiographs are usually the first line examination, either showing no lesion or rarely calcifications. Ultrasonography shows a lobulated well-defined mass, that’s hypoechoic, but, because of its non-specificity, MRI is required for better characterization of the lesion. It shows a large lobulated mass (usually >5cm), containing solid tumour, haemorrhage, necrosis and calcifications. Therefore, in T2 weighted sequences, it is heterogeneous, containing areas of intermediate signal, high signal and dark signal. The combination of these 3 shades of signal in the T2 weighted images is called the “triple sign”, and leads to diagnosis. Histological confirmation is necessary. And treatment of choice is the surgical excision of the mass [1-3]. Keywords: Tumour; Soft; Tissue; Sarcoma; Imaging References [1] Al-Ibraheemi A, Ahrens WA, Fritchie K, Dong J, and Oliveira AM, et al. (2019) Malignant tenosynovial giant cell tumor: The true “synovial sarcoma?” A clinicopathologic, immunohistochemical, and molecular cytogenetic study of 10 cases, supporting origin from synoviocytes. Mod Pathol. 32: 242-251. [2] Chambers LA, and Lesher JM (2018) Chronic thigh pain in a young adult diagnosed as synovial sarcoma: A case report. PM&R. 10: 969-973. [3] Mirzaian E, Tavangar SM, Montazeri S, and Yeganeh, FE (2019) Biphasic epithelial predominant synovial sarcoma presenting as painful thigh mass. Iran J Pathol. 14: 261-265

Primary Pleural Synovial Sarcoma Presenting as Small Nodules Hidden in Pleural Effusion

Kai Lin1,2, Biyun Yu3 and Chuan Shao3-5* Author Affiliations 1Department of Geriatric Medicine, Ningbo Medical Center Lihuili Hospital, China 2Department of General Practice, Ningbo Medical Center Lihuili Hospital, China 3Department of Pulmonary and Critical Care Medicine, Ningbo Medical Center Lihuili Hospital, China 4Department of Special Procurement Ward, The First Affiliated Hospital of Soochow University, China 5Department of General Practice, The First Affiliated Hospital of Soochow University, China Received: July 09, 2020 | Published: July 22, 2020 Corresponding author: Chuan Shao, Department of Pulmonary and Critical Care Medicine, Ningbo Medical Center Lihuili Hospital, Ningbo, China and Department of Special Procurement Ward, The First Affiliated Hospital of Soochow University, Suzhou, China and Department of General Practice, The First Affiliated Hospital of Soochow University, Suzhou, Address: No.1111 Jiangnan road, Ningbo Medical Center Lihuili Hospital, Ningbo, 315040, China DOI: 10.26717/BJSTR.2020.29.004742

Synovial sarcoma presenting as an intra-articular mass in a pediatric patient: a case report

BackgroundSynovial sarcoma (SS) is one of the reported sarcomas in the pediatric and adult populations. Delay in diagnosis and treatment is common in SS cases. SS may be excised before the correct diagnosis is made.Case presentationwe present a case involving a 4-year-old boy who visited our service with complaints of left knee pain and limited knee flexion. Initially, the child was diagnosed with osteochondromatosis. Surgical excision was opted, and initial histopathological examination revealed a fibrous histiocytoma. The slide and blocks were then brought to the King Faisal Specialist Hospital Research Center (KFSH&RC) and histopathologic analysis has shown a well-circumscribed nodule in the synovium with a sub-synovial monomorphic spindle cell sarcoma, confirmed by fluorescence in situ hybridization (FISH).ConclusionsTherefore, we strongly recommend considering all differential diagnoses for soft-tissue masses when planning surgical management.

1925 GIST Kidding: Primary Gastric Synovial Sarcoma Presenting as Hematemesis

INTRODUCTION: Synovial sarcoma (SS) is an uncommon subtype of mesenchymal derived solid tumors that is characterized by its unique histologic pattern. It is most often found in the extremities and rarely, the digestive tract, which can lead to gastrointestinal bleeding. Gastric tumors of mesenchymal origin often have overlapping radiographic and endoscopic appearances, which presents a significant challenge in diagnosis. We report one of only 3 confirmed cases of primary gastric synovial sarcoma of the lesser curvature in a patient who presented with hematemesis. CASE DESCRIPTION/METHODS: A 26-year-old male presented with a two-week history of worsening abdominal pain and intermittent hematemesis. He denied any history of hematochezia, melena, early satiety, weight loss, recent NSAID use or alcohol ingestion. Exam was notable for epigastric tenderness without rebound or guarding. Labs revealed microcytic anemia and thrombocytopenia without transaminitis or elevated lipase. Abdominal CT showed an 8 cm mass along the posterior gastric body with suspicion for intratumoral hemorrhage, highly suggestive of a gastrointestinal stromal tumor (GIST) [Figure 1]. EGD revealed a large, ulcerated, non-bleeding, non-circumferential, submucosal mass on the lesser curvature of the stomach, also concerning for a GIST [Figure 2]. Endoscopic ultrasound showed neither hepatic invasion nor lymphadenopathy. Partial gastrectomy pathology supported the endoscopic biopsy results, which showed high grade, monophasic, atypical spindle cells with t(X;18)(SYT-SSX) chromosomal translocation by fluorescence in situ hybridization (FISH). Immunohistochemistry (IHC) was strongly positive for TLE1 staining, but negative for C-KIT and DOG-1, to which GISTs are immunoreactive, confirming a diagnosis of primary gastric synovial sarcoma [Figure 3]. He tolerated 6 cycles of sarcoma-directed chemotherapy without evidence of progressive disease. DISCUSSION: Intramural gastric spindle cell malignancies are difficult to distinguish from one another, and require different disease-tailored therapies. Though GISTs account for 90% of these tumors, other rare pathologies exist. These include synovial sarcomas, which are characterized by a variable degree of epithelial differentiation associated with a disease-specific gene translocation. In patients with hematemesis found to have endoscopic and radiographic findings suspicious for GIST, ICH and FISH analyses are crucial to differentiate between mesenchymal malignancies to guide effective therapy.

Synovial sarcoma presenting as an enlarging dermal tumor

Synovial sarcoma presenting as an enlarging dermal tumor

Synovial sarcoma presenting as subcutaneous swelling: a rare presentation

Synovial sarcoma unlike its name is not originating from synovial cells. It accounts for approximately 10% of all soft tissue sarcomas with main predilection for extremities.We present here a case of synovial sarcoma of subcutaneous origin of knee joint which is rarest of presentations and early diagnosis is associated with lower risk of metastasis and hence better prognosis.

Synovial sarcoma: Do children do better?

ObjectivesSynovial sarcoma, a distinct subtype of soft tissue sarcomas (STS), is typically found in young patients. Long history of symptoms and heterogeneous clinical presentation sometimes delays diagnosis. Children have been reported to have a better prognosis than adults in some series.The main emphasis of this study was to determine differences between children and adults and to investigate prognostic factors regarding cancer specific survival (CSS). Methods248 patients treated between 1982 and 2014 at one department were included. Mean age was 37.0 years, including 43 patients <16 years. Demographic, pathology- and treatment-related information was ascertained. Median follow-up was 5.2 years. ResultsMedian duration of symptoms was 11.5 months in children and 12 months in adults (p = 0.238). Patients with a prior unplanned excision had a significantly longer duration of symptoms (p = 0.001). No difference was present between children and adults regarding tumour size, site, grade and superficial/deep location. Treatment was with surgical excision and (usually) adjuvant radiotherapy but five patients received preoperative radiotherapy and 43 patients chemotherapy. In patients treated with curative intent, five-year CSS rates were 75.5% for adults and 89.0% for children, with 10-year CSS rates of 56.1% and 82.2% (p = 0.026).In multivariate analysis, large tumour size (p < 0.005) and patient age (p = 0.024) were associated with worse CSS, irrespective of tumour location and site. ConclusionClinical presentation of synovial sarcoma is similar in children and adults, with no significant difference in tumour size, site, grade or location. Small tumour size and young patient age are independent positive prognostic factors influencing CSS.

A Rare Case of Metastatic Synovial Sarcoma Presenting as a Fatal Hemorrhagic Stroke (P6.331)

A Rare Case of Metastatic Synovial Sarcoma Presenting as a Fatal Hemorrhagic Stroke (P6.331)

256 Monophasic Synovial Sarcoma Presenting as a Parapharyngeal Mass—Report of a Rare Case

Synovial sarcoma (SS) is an aggressive soft tissue sarcoma, most commonly arising in the peri-articular tissue of extremities in young adults, harboring a specific chromosomal translocation t(X;18)(p11.2;q11.2). SS is a misnomer because it does not arise from the synovium; it appears to arise from pluripotent stem cells that are capable of differentiating into mesenchymal and/or epithelial structures and lacks synovial differentiation. SS accounts for ~5%-10% of soft tissue sarcomas. Occasionally, it arises in the head and neck region and carries a poor prognosis. Morphologically, SS can be biphasic (BSS), monophasic (MSS), or poorly differentiated. We report a rare case of MSS presenting as a parapharyngeal mass. The patient is a 27-year-old-man presenting with left-sided facial and mandibular pain. Imaging showed large parapharyngeal soft tissue mass in the region of the left pterygoid musculature measuring 3.6 x 2.9 x 2.5 cm with focal calcifications. He underwent resection of this mass, which showed spindle cell neoplasm with variably prominent hemangiopericytoma-like vascular pattern. Vague nuclear palisading was also noted. Immunohistochemical stains were focally positive for cytokeratins (AE1/AE3, CK7, CK8 (Cam5.2), and CK19), calponin, calretinin, EMA, GFAP, and synaptophysin with Ki67 labeling being 25%-30%. The tumor cells were negative for CD34, CD117, desmin, HMB45, myogenin, P63, S100, and SMA. RT-PCR showed SYT/SSX2 fusion transcript. Combining all, the diagnosis of MSS-spindle cell type was confirmed. Based on AFIP data, ~3% of SS arise in this location. SS is a rare entity to be encountered in the head and neck region and is often a challenge in terms of diagnosis, especially the MSS type. The differential of monophasic tumors is fibrosarcoma, malignant schwannoma, and hemangiopericytoma. We report this case of MSS because of its rare presentation as a parapharyngeal mass. Clinicians and pathologists should be aware of this unusual location and presentation of SS.

Pulmonary Synovial Sarcoma Presenting as Empyema

Pulmonary Synovial Sarcoma Presenting as Empyema

Omental synovial sarcoma mimicking an ovarian malignancy: A case report.

Synovial sarcoma is clinically rare, and cases of synovial sarcoma arising in the omentum are particularly rare. Only 3 cases have been reported in the literature to dtae, and they were associated with a poor prognosis. We herein report a rare case of aggressive primary omental synovial sarcoma presenting as an ovarian malignancy. A 53-year-old multigravida woman was referred to our hospital due to progressive abdominal distension. Magnetic resonance imaging revealed a large heterogeneous mass with an irregular component occupying the lower abdominal cavity, with an intact uterus. Intraoperative examination revealed a solid mass arising from the lower omentum. The diagnosis of omental synovial sarcoma was established based on postoperative pathological and immunohistochemical examination of the tumor. The patient underwent multiple surgical resection procedures due to the development of metastases in the liver, lungs and abdominal cavity and received adjuvant chemotherapy including doxorubicin-ifosfamide, pazopanib and trabectedin. Such rare neoplasms may be difficult to diagnose preoperatively based on radiological examinations alone. Thus, gynecologists should be aware of the possibility of omental synovial sarcoma, and combined surgical and chemotherapeutic interventions are required to control such aggressive tumors.