scholarly journals Primary Pulmonary Synovial Sarcoma: A Very Rare Presentation

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Ekrem Cengiz Seyhan ◽  
Sinem Nedime Sokucu ◽  
Gulsah Gunluoglu ◽  
Nurdan Simsek Veske ◽  
Sedat Altin

Synovial sarcoma (SS) is a rare tumor originating from mesenchymal tissue and accounting for approximately 5–10% of all soft tissue sarcomas. A rare case of primary pulmonary SS in an asymptomatic 18-year-old man admitted to our hospital for investigation of a 6 × 6.5 cm, oval-shaped, well-delineated pleural based peripheral mass in the left lower lobe in his thorax CT is presented. Left lower lobectomy was done. Immunohistochemically, tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA), and vimentin so that the histopathological diagnosis was compatible with biphasic spindle cell type SS in the lung.

2020 ◽  
pp. 1-4
Author(s):  
Wickii T. Vigneswaran ◽  
Brittany C. Fields ◽  
Vijayalakshmi Ananthanarayanan ◽  
Wickii T. Vigneswaran

Synovial sarcomas represent an extremely rare subtype of an already rare group of malignancies, soft tissue sarcomas. Among these, primary pulmonary synovial sarcomas comprise an even smaller number, though they have become more frequently reported in the literature. This case report details a case of giant primary pulmonary synovial sarcoma in a 44-year-old male patient who presented with left-sided chest pain and shortness of breath and was found to have a large left-sided pleural effusion. No malignant cells were demonstrated on cytology of pleural fluid after thoracentesis; however, CT-guided needle biopsy of pleural nodules seen on imaging demonstrated pathologic features consistent with monophasic type synovial sarcoma. He was treated with neoadjuvant chemotherapy with minimal response; thus, he was referred for surgical management. A left extrapleural pneumonectomy with resection of the left hemi-diaphragm and Gore-Tex prosthetic reconstruction was performed. Imaging at six-month follow-up demonstrated a new nodule in the contralateral lung, suggestive of metastasis at that time and the patient later developed ascites at nine months, consistent with further intra-abdominal metastasis. Perhaps early diagnosis and aggressive multimodality therapy may have a place in the treatment of this aggressive disease.


2019 ◽  
Vol 6 (6) ◽  
pp. 2190
Author(s):  
Dheer S. Kalwaniya ◽  
Jaspreet S. Bajwa

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.


2019 ◽  
Vol 2 (2) ◽  
pp. e40-e41
Author(s):  
Muhammad Iqbal ◽  
Sanjay Agarwal ◽  
Iqbal S. Shergill

Spindle cell lipoma (SCL) in the scrotum is a very rare presentation with only a few cases reported in the literature. The most common sites for this benign lesion are the neck, shoulder, and back. We present a 72-year-old male patient with painless swelling on the left half of the scrotum with a histopathological diagnosis of SCL.


2003 ◽  
Vol 127 (4) ◽  
pp. e201-e204 ◽  
Author(s):  
Nahal Boroumand ◽  
Vinay Raja ◽  
Dennie V. Jones ◽  
Abida K. Haque

Abstract Pulmonary synovial sarcoma is a rare neoplasm recently recognized as a distinct entity and characterized by t(X;18) translocation and production of at least 2 fusion genes, SYT-SSX1 and SYT-SSX2. We report a case of primary pulmonary synovial sarcoma with the SYT-SSX2 phenotype and a rapidly progressive downhill course. Previous reports have suggested that the soft tissue synovial sarcomas with SYT-SSX2 phenotype have a favorable clinical outcome. To the best of our knowledge, this is also the first report of CD117 (c-Kit) expression in a pulmonary synovial sarcoma. A 45-year-old woman presented with left chest pain and was found to have a left lower lobe tumor that was originally diagnosed as a sarcomatoid carcinoma. After the patient underwent chemotherapy and brachytherapy, the specimen from a left pneumonectomy showed a large spindle cell tumor, which was reclassified as a synovial sarcoma based on the results of immunophenotyping and molecular genetic studies. Differentiation between sarcoma and carcinosarcoma is crucial for implementing appropriate therapy. Furthermore, if the tumor expresses c-Kit, it may respond to target-based therapy.


2021 ◽  
Vol 14 (8) ◽  
pp. e242313
Author(s):  
Quan Do ◽  
Vatsala Katiyar ◽  
Andrea Breaux ◽  
Vikas Singh

Synovial sarcoma (SS) is a rare soft tissue sarcoma usually arising in the deep soft tissues of the limbs, trunk, and head and neck region. Due to its rarity, diagnosis can be difficult to establish, especially when it involves an uncommon location like the breast. In this case report, we describe a young woman who was found to have primary SS of the breast. Initial immunohistochemistry staining was focally positive for cytokeratin and S100 and she was misdiagnosed with atypical spindle cell carcinoma. Due to the unusual presentation, further testing was performed which showed TLE1 and epithelial membrane antigen positivity, establishing the diagnosis of SS of the breast. A FISH was later sent out and was positive for SS18-SSX fusion transcript. This case highlights the importance of considering rare histopathology in breast lesions and using additional staining and cytogenetics to confirm diagnosis.


2012 ◽  
Vol 15 (5) ◽  
pp. 297 ◽  
Author(s):  
Osman Nuri Tuncer ◽  
Ozan Erbasan ◽  
İlhan Golbasi

Synovial sarcoma (SS), a mesenchymal spindle cell tumor, displays variable epithelial differentiation, including glandular formation, and features a specific chromosomal translocation, t(X;18)(p11;q11). SS accounts for 5% to 10% of soft-tissue sarcomas. These tumors occur mostly in the joints, especially near the knee, but they also occur in other locations. Primary intravascular SS (IVSS) are extremely rare; only 6 well-documented cases have been reported in the English literature. We describe a new case of primary IVSS of the superior vena cava (SVC) in a 16-year-old boy. A transthoracic echocardiogram confirmed a large (4.8 ◊ 4.6 cm) circumscribed mass filling the right atrium, as well as a moderate pericardial effusion. The mass extended from the SVC to the tricuspid valve but did not prevent valve coaptation. Surgery via a transatrial approach revealed a huge mass (8 to 12 cm) attached to the SVC via a 5-mm pedicle. The tumor was excised, and the patient experienced an uneventful postoperative course. Fluorescence in situ hybridization analysis revealed the presence of the SS-specific translocation.


2020 ◽  
Vol 17 (3) ◽  
pp. 45-49
Author(s):  
Ajay Sebastian Carvalho ◽  
Kishan Kumar Yadav ◽  
Vijay Kumar Gupta

Synovial sarcoma (SS) accounts for 5- 10% of all adult soft-tissue sarcomas and only 5% arises in the spine. It presents like any other spinal tumor, namely axial pain with symptoms due to neural compression. Imaging findings can also be similar to any other benign spinal tumor. We present a 43-year-old male who presented with symptoms of radiculopathy and neural compression and imaging revealed a dumbbell tumor at C6 to D1 with transforaminal and paraspinal extension on the right side. After surgical excision the histopathology revealed a rare synovial sarcoma of the spine. Synovial sarcoma of the spine though rare, are difficult to differentiate based on their presentation and imaging characteristics from benign spinal tumors. However, subtle findings on imaging and a pre-operative biopsy may aid in performing a more definitive surgery upfront rather than a re-do surgery after the histopathological diagnosis.  


2021 ◽  
Vol 22 (1) ◽  
pp. e48-e50
Author(s):  
Leonid Emerel ◽  
Humberto Trejo Bittar ◽  
Olugbenga T. Okusanya

Author(s):  
Kadir Burak Akgün ◽  
Mukadder Çalıkoğlu ◽  
Rabia Bozdoğan Arpacı ◽  
Pınar Ergen

Synovial sarcoma is a highly aggressive malignancy with a 10-year disease-free survival of around 50%. Synovial sarcoma accounts for 5 to 10% of all soft tissue sarcomas. It is more common in young people and adolescents and it is seen in lower extremities, especially around the knee. The prognosis of the disease is worse in the elderly population. Microscopic imaging, positive staining of epithelial markers and molecular study are required to confirm the the presence of the disease. We present a 44-year-old male patient with a history of falling from the same level and presenting with only shoulder pain. Further investigations have shown that our patient is a rare form of biphasic synovial sarcoma originating from the pulmonary system, which is rarely reported in the literature.


Mastology ◽  
2020 ◽  
Vol 30 (Suppl 1) ◽  
Author(s):  
Juliana Lopes de Aguiar Araujo ◽  
Ubiratan Wagner de Sousa ◽  
Fernanda Mabel Batista de Aquino ◽  
Luisa Gurgel de Lira ◽  
Iris Maria Rodrigues de Andrade Almeida

Introduction: Breast synovial sarcoma is extremely rare, with few cases described. It corresponds to 6–9% of soft tissue sarcomas and is more frequent in extremities (80%), trunk (8%), and abdomen (7%) in young adults. It usually does not affect the breast. Objectives: To report a rare case of monophasic breast synovial sarcoma and provide data for the global literature. Method/Case report: G.S.B., 97 years old, presented a 7 cm nodule in the left breast and negative axillary nodes. Ultrasound (US) revealed a heterogeneous nodule of 6.0 x 5.5 cm, BI-RADS 5. She did not have mammography. Core biopsy showed spindle cell neoplasm. Immunohistochemistry (IHC) indicated mesenchymal lesion, without differentiating stromal components of fibroepithelial tumor from the mesenchymal lesion. Rapid growth with ulceration and tumor bleeding were identified. Urgent mastectomy showed a malignant neoplasm of spindle cell pattern and high grade, with 12 cm, involved lateral margin, and 19 negative axillary lymph nodes. IHC of the surgical specimen indicated monophasic synovial sarcoma. Before the wide excision, she had a rapidly progressive recurrence in the sternum, making it non-resectable. During radiotherapy (RT), local progression was identified. She has been receiving chemotherapy (CT) with ifosfamide and adriamycin. No evidence of distant disease was found after 9 months of diagnosis. Results/Discussion: Synovial sarcoma corresponds to approximately 0.06% of all breast neoplasms, originating from their mesenchymal tissue, with variable epithelial differentiation. The term synovial sarcoma is inadequate, deriving from its frequent juxta-articular location. Its incidence is approximately 1.5 per 1 million individuals, with a mean age of 32 years and a male:female ratio of 1.2:1. The main histological subtypes are: classic biphasic and monophasic. Translocation t(X;18) (p11.2; q11.2) and expression of SYT/SSX gene fusion are present in more than 95% of cases. IHC shows an intense expression of vimentin and CD99, and focal of Bcl2, EMA, CKAE1-AE3, actin, and desmin, as well as negativity for S100, cytokeratins, hormone receptors, myosin, and caldesmon. The differential diagnosis is made with other spindle cell entities, such as fibromatosis, solitary fibrous tumor, myofibroblastoma, metaplastic carcinoma, and other sarcomas. Synovial sarcoma has a moderate response to chemotherapy with anthracyclines. The treatment includes wide surgical resection and RT. Metastases occur in about 50% of cases and are present at diagnosis in 16% to 25%; they are more frequent in the lung (75%), regional lymph nodes (15%), and bones (10%), tending to late recurrence and metastases. The 5-year disease-free survival is 60%. Conclusion: The heterogeneity of the disease and its low incidence hinder prospective studies addressing therapeutic options with better long-term results.


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