scholarly journals A Case of Giant Primary Pleuropulmonary Synovial Sarcoma

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.

2021 ◽  
Vol 7 (4) ◽  
pp. 224-226
Author(s):  
V S Haritha ◽  
Chandra Prakash ◽  
Rajesh Jain

Synovial sarcoma is a malignant mesenchymal tumour which mostly affects young adults and is mostly seen in extremities. Primary synovial sarcoma arising from the lung is rare, accounting for less than 0.5% of all lung carcinomas. Most commonly it arises from the lung followed by pleura and mediastinum. Primary pulmonary synovial sarcoma is an extremely aggressive malignant tumour that can invade adjacent organs and give distant metastasis. Histologically it is of two main types – monophasic and biphasic. IHC is a must for diagnosis following clinical examination and imaging. Here we report a case of an elderly male with right lung mass lesion infiltrating the visceral and mediastinal pleura. PET – computerized tomography (CT) guided biopsy was s/o synovial sarcoma monophasic type which was further confirmed by IHC. The multimodality treated for this includes wide resection, chemotherapy and radiotherapy. Synovial sarcoma is relatively chemosensitive though it is considered as a high grade tumour with a poor prognosis. Because of the advanced stage of the disease our patient was not a candidate for surgery and was taken up for chemotherapy. He had a survival of 6 months but had succumbed due to non – cancer related cause.


2021 ◽  
Vol 14 (1) ◽  
pp. e238366
Author(s):  
Sandeep Vijayan ◽  
Afsal P Mohammed ◽  
Mahesh Suresh Kulkarni ◽  
Sharath Kumar Rao

Synovial sarcomas are deep-seated, genetically distinct, malignant neoplasms seen in young adults, with a male preponderance. They have unusual clinical and pathological presentation and mimic many other sarcomas and carcinomas, making the diagnosis quite challenging. Although four variants are identified, occurrence in the hand is extremely rare and leads to significant morbidity. There is a high incidence of local recurrence and distant metastasis within the first 2 years. We report the case of an elderly woman with monophasic spindle cell synovial sarcoma who presented with painless swelling in her palm and underwent local excision of the neoplasm. At 2-year follow-up, she remains totally asymptomatic with normal function of the hand. We also attempt to give an overview about monophasic spindle cell synovial sarcoma with the differentials, which would help surgeons in prompt diagnosis and appropriate management.


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.


Hand ◽  
2021 ◽  
pp. 155894472097513
Author(s):  
Arthur Samia ◽  
Joshua Scarcella ◽  
Richard Zeri ◽  
Yifan Guo

There have been 8 synovial sarcomas of the median nerve reported. We report a case of a 15-year-old male with synovial sarcoma of the right-hand median nerve. Patient presented with a 2-month history of enlarging mass at the base of the right thenar eminence associated with numbness in the median nerve distribution. Physical examination revealed a soft mass over the thenar eminence and paresthesia in the median nerve distribution. He underwent excision of the tumor, which revealed a well-encapsulated lesion encompassing the median nerve, involving the first, second, and radial aspect of the third web space as well as recurrent branches of the median nerve. Following excision of the tumor, a thorough metastatic workup was negative for metastatic disease. He was staged as III, T2b, N0, M0—poorly differentiated monophasic synovial sarcoma of the right median nerve. Postoperatively the patient was started on chemotherapy and radiation. Intraneural synovial sarcoma is extremely rare. Our case is the youngest with the longest follow-up. He is currently at a status of 3 years posttreatment with no signs of recurrence and excellent use of his right hand. This case is of particular interest due to the rarity of the disease along with this being the best outcome reported in the literature to-date.


2019 ◽  
Vol 12 (11) ◽  
pp. e232135
Author(s):  
Judy Wang ◽  
Hong Kuan Kok ◽  
Iman Bayat

Synovial sarcomas are a rare but aggressive malignancy that primarily affects young patients. Diagnosis is often difficult and delayed due to its insidious onset, heterogenous presentation and mimicry of other pathologies. We present the case of a patient with a history of a slow-growing left arm mass that arose after a traumatic fracture of the humerus. Multimodal imaging was undertaken and reported the mass as being consistent with a vascular malformation of the brachial artery. The patient underwent surgical repair of the artery and intraoperative biopsies confirmed a diagnosis of synovial sarcoma. This case highlights the importance of maintaining suspicion for soft-tissue sarcomas in young patients presenting with a mass, and demonstrates the way in which these tumours may mimic other pathologies both clinically and radiologically. Early referral to a specialist sarcoma centre is key for further investigative workup.


2015 ◽  
Vol 22 (1) ◽  
pp. e1-e3 ◽  
Author(s):  
Jaspreet S Kambo ◽  
Bonnie Richardson ◽  
Diana N Ionescu ◽  
Tracy Tucker ◽  
Greg Kraushaar

Primary pulmonary synovial sarcoma (PPSS) is a rare malignancy. Its etiology, imaging features and optimal treatment are not well understood. Pulmonary pseudoaneurysms and lymphadenopathy are rare complications of synovial sarcomas. A 40-year-old woman with mild hemoptysis and thoracic back pain underwent a computed tomography scan that revealed multiple pulmonary lesions, paraesophageal lymphadenopathy and incidental bilateral pulmonary emboli. A diagnosis of PPSS was made through the identification of an SS18 translocation by fluorescence in situ hybridization. She was started on adriamycin, ifosfamide and mesna chemotherapy. Over the subsequent two months, she developed three pulmonary artery pseudoaneurysms, ultimately requiring endovascular coiling. Seven months after starting treatment, the patient was asymptomatic. The lesions and lymphadenopathy decreased in size. The present case highlights complications of a rare malignancy and demonstrates positive response to ifosfamide-based chemotherapy in the setting of PPSS.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Serenella Serinelli ◽  
Lorenzo Gitto ◽  
Daniel J. Zaccarini

Abstract Background Synovial sarcomas are infrequent malignant tumors occurring mostly in adolescents and young adults. The occurrence of synovial sarcoma in the hand-wrist area is rare (4 to 8.5% of all synovial sarcomas in different studies). Case presentation This report documents an uncommon case of synovial sarcoma occurring in the hand-wrist of a 69-year-old Caucasian woman. She was subsequently treated with surgical excision and radiotherapy without recurrence after follow up. Conclusions This paper aims to characterize the demographic, pathologic, and clinical features with a literature review. The present literature review confirms that hand-wrist synovial sarcomas are more frequent among males and subjects 10 to 40 years old. Most cases in this location are usually not larger than 5 cm in size. The five-year survival rate is higher than that reported in a previous review on hand synovial sarcomas, and this suggests an improved survival in recent decades.


2005 ◽  
Vol 446 (3) ◽  
pp. 310-315 ◽  
Author(s):  
B. Bode-Lesniewska ◽  
J. Hodler ◽  
A. von Hochstetter ◽  
L. Guillou ◽  
U. Exner ◽  
...  

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.


2021 ◽  
Vol 28 (3) ◽  
pp. 1909-1920
Author(s):  
Aaron M. Gazendam ◽  
Snezana Popovic ◽  
Sohaib Munir ◽  
Naveen Parasu ◽  
David Wilson ◽  
...  

Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5–10% of all STS. Synovial sarcoma differs from other STS by the relatively young age at diagnosis and clinical presentation. Synovial sarcomas have unique genomic characteristics and are driven by a pathognomonic t(X;18) chromosomal translocation and subsequent formation of the SS18:SSX fusion oncogenes. Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. Increasing size, age and tumor grade have been demonstrated to be negative predictive factors for both local disease recurrence and metastasis. Wide surgical excision remains the standard of care for definitive treatment with adjuvant radiation utilized for larger and deeper lesions. There remains controversy surrounding the role of chemotherapy in the treatment of SS and there appears to be survival benefit in certain populations. As the understanding of the molecular and immunologic characteristics of SS evolve, several potential systematic therapies have been proposed.


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