Primary Pulmonary Synovial Sarcoma Research Articles

A case of poorly differentiated type primary pulmonary synovial sarcoma

A case of poorly differentiated type primary pulmonary synovial sarcoma

Primary Pulmonary Synovial Sarcoma; Abnormal Presentation of a Rare Lung Tumor

Primary synovial sarcomas of the lung are a very rare entity in thoracic surgery. Unusual presentation make it hard to diagnose such disease and manage it probably even harder. We present two cases with same tumor but different appearance.

EP14.03-04 Rare but Aggressive: A Case of Primary Pulmonary Synovial Sarcoma

EP14.03-04 Rare but Aggressive: A Case of Primary Pulmonary Synovial Sarcoma

Pulmonary synovial sarcoma: A case series and review of literature

Synovial sarcoma is a rare malignant mesenchymal tumor that can develop at any anatomic site. Pulmonary sarcomas constitute 0.1–0.5% of all primary lung malignancies. Primary pulmonary synovial sarcoma is highly uncommon. Most of the patients present with large intrathoracic masses with complaints of cough, chest pain, shortness of breath, or hemoptysis. Multimodality treatment in the form of wide excision, chemotherapy, and radiotherapy is the mainstay of therapy. Synovial sarcoma is considered a high-grade tumor with a poor prognosis. We hereby present three cases of histologically proven synovial sarcoma with predominantly pulmonary involvement, along with their management and in hospital outcome.

A Rare Case of Primary Pulmonary Synovial Sarcoma

A Rare Case of Primary Pulmonary Synovial Sarcoma

An uncommon case of primary synovial sarcoma of the lung with a rare intraoperative finding

We report a case of a 32-year-old male who was evaluated for right chest pain and cough with streaky hemoptysis. Chest X-ray and contrast-enhanced computerized tomography scan of the chest showed a large (20x16×16 cm), heterogeneously enhancing mass arising from the right lower lobe. Ultrasound-guided Tru-cut biopsy revealed spindle cell sarcoma. In immunohistochemistry, tumor cells expressed epithelial membrane antigen, CD99, and bcl-2. The final diagnosis of primary pulmonary synovial sarcoma was confirmed after positron emission tomography-computed tomography revealed that the lesion was confined to the right lung. The patient was managed by multimodal treatment. The patient underwent right anterolateral thoracotomy and lower lobectomy with staged systematic mediastinal lymphadenectomy. Intra-operatively, he was given 2 units of packed red blood cells (RBCs). The patient developed dark-colored urine near the end of the transfusion of the second unit of packed RBCs, but without any hemodynamic instability. All the tests for possible blood transfusion reactions were negative; thus, we concluded that this event was probably secondary to tumor lysis due to the handling of the large primary lesions during surgery.

The Role of Surgery for Primary Pulmonary Synovial Sarcoma: A Population-Based Study

IntroductionThe rarity of primary pulmonary synovial sarcoma (PPSS) and the lack of prospective clinical trials resulted in poorly understood treatment modality and clinical outcomes. This study aimed to better understand PPSS based on patients from the Surveillance, Epidemiology, and End Results database. Materials and methodsClinical and survival data of PPSS patients who were diagnosed during 1989 through 2016 and retrieved from the Surveillance, Epidemiology, and End Results database were studied. Kaplan–Meier analyses and Cox proportional hazards model were applied to evaluate the overall survival (OS) and disease-specific survival (DSS) of PPSS patients. ResultsA total of 122 patients with PPSS were included (median age: 50 y). PPSS accounted for 4.5% (122/2741) of total primary synovial sarcoma. Most of the patients were diagnosed as poor or undifferentiated grade (52.0% and 34.0%). Cancer-directed surgery was performed for 74.4% of PPSS patients and 28.2% of patients received radiotherapy. The 1-year, 3-year, 5-year, and 10-year OS rates of PPSS patients were 75.4%, 50.8%, 41.8%, and 39.3%, respectively. Cancer-directed surgery was shown to improve the survival of PPSS patients with localized or regional stage (P < 0.05), yet surgical resection did not prolong the OS and DSS of patients with distant stage (P > 0.1). Postoperational radiotherapy was associated with shortened survival time (P < 0.05). PPSS patients who received lobectomy had statistically prolonged OS and DSS than those with pneumonectomy (P < 0.001). ConclusionsPPSS is a rare and special subtype of synovial sarcoma. Treatment with lobectomy or sublobar resection alone may contribute to a superior prognosis compared with other managements.

The early-stage of primary pulmonary synovial sarcoma: A case report.

BackgroundPrimary pulmonary synovial sarcoma is a rare case of lung cancer. Surgical resection is standard therapy. Early-stage and complete resection offers a good prognosis.Case presentationA 50-year-old woman complained of intermittent left chest pain. Chest X-ray showed a mass around the heart, MSCT also showed enhanced solid lesion in the lower left lung anterobasal segment adhered to the pericardium. The patient underwent surgery with wedge resection with both immunohistochemical (IHC) EMA and CD99 positive. After surgery, she received 3-cycle chemotherapy that consisted of a combination of doxorubicin and ifosfamide.DiscussionSurgical resection is the mainstay treatment for primary pulmonary synovial sarcoma. Chemotherapy can be considered for a patient with high-risk metastases. Early-stage and young age showed a good prognosis. Patient with stage IA still survives for 49 months after completing surgical resection and chemotherapy.ConclusionResection of the tumor followed by chemotherapy may increase the survival tumor.

Primary pulmonary synovial sarcoma

ObjectivePrimary pulmonary synovial sarcoma (PPSS) is extremely rare. This study aims to identify the clinicopathologic and therapeutic factors determining survival in PPSS. MethodsWe performed a retrospective analysis of 121 patients from the Surveillance, Epidemiology, and End Results Database as well as 12 patients from our own institution diagnosed with PPSS. Patient survival was evaluated using the Kaplan-Meier method. ResultsThe median survival time for 12 PPSS patients in our institution was 78 months. Postoperative chemotherapy (P = .027 for overall survival and P = .035 for disease-specific survival) was associated with superior survival, whereas pneumonectomy (P = .011 for overall survival and P = .006 for disease-specific survival) was associated with worse survival. Single lobe involvement (P = .022) and the absence of lymph node involvement (P = .045) were associated with improved disease-specific survival and overall survival, respectively. In the Surveillance, Epidemiology, and End Results Database, the median survival time was 23 months. Significantly superior survival was observed in patients with earlier American Joint Committee on Cancer stage (Ⅰ-Ⅱ) (P < .001 for both overall survival and disease-specific survival). Patients who were diagnosed within the recent decade did not achieve a better survival (P = .599 for overall survival and P = .596 for disease-specific survival). ConclusionsPPSS was aggressive with a very poor prognosis. The seventh American Joint Committee on Cancer stage might aid in predicting survival. Pneumonectomy and lymph node involvement might be associated with worse survival, whereas single lobe involvement and postoperative chemotherapy might be associated with improved survival.

Primary thoracic synovial sarcomas: A case report

Primary pulmonary synovial sarcoma (SS) is a rare neoplasm. Its clinicoradiologic attributes are not yet well defined. We report the observation of a patient followed for primary pulmonary synovial sarcoma. We report the radio‐clinical features of this rare tumor.

A Rare Case of Primary Pulmonary Synovial Sarcoma

Synovial Sarcoma (SS) is a rare mesenchymal neoplasm arising commonly from the periarticular tissue. Primary SS involving the lung accounts for less than 0.5% of all lung tumours. SS are high grade aggressive tumours affecting the extremities of adolescents and young adults. Clinically, patient presents with cough, breathing difficulty, chest pain or haemoptysis and imaging studies may reveal a mass lesion but for definitive diagnosis, histopathology and immunohistochemical examination is needed. Hereby, authors report a case of 39-year-old female diagnosed with primary pulmonary SS with particular emphasis on the pathologic findings.

Staged Surgical Management of Synchronous Primary Pulmonary Synovial Sarcoma with Multiple Pancreatic Metastases: Report of a Rare Case and Review of the Literature

Synovial sarcoma (SS) is arare malignant lesion, which accounts for about 9% of all soft tissue sarcomas and has a high metastatic potential. These lesions may have a long, indolent course followed by late metastases, which occur in approximately half of cases.Pancreatic metastasis by sarcomas is uncommon and is most frequentlydiagnosed in patients with widespread metastatic who are not appropriate candidates for resection. Whether pancreatic resections for metastases from SS are beneficial in terms of long-term outcomes remains elusive with only a few cases reported in literature.We herein present a rare case of a middle aged female patient who presented to our department with synchronous primary pulmonary SS and pancreatic metastases and underwent staged resections.

A rare case of aggressive primary pulmonary synovial sarcoma: A case report

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.

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.

Primary Pulmonary Synovial Sarcoma: How to Expect the Unexpected?

Primary pulmonary synovial sarcoma (PPSS) is a rare and aggressive malignancy with a presentation similar to that of lung carcinoma. We present an 18-year-old male who presented with complaints of chest pain, cough, and hemoptysis. Physical examination and blood parameters were normal. Imaging studies revealed a large, well-defined, round homogeneous mass in the left lower lobe. His fine-needle aspiration cytology was suggestive of a round cell tumor. The patient was operated under general anesthesia through left posterolateral thoracotomy. The tumor was removed en masse and sent for histopathological examination. His histopathology and immunohistochemistry suggested PPSS of biphasic variety.

SOLITARY PULMONARY NODULE PRESENTING AS A SARCOMATOID CARCINOMA IN A PREGNANT WOMAN: A DIAGNOSTIC DILEMMA

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: The majority of lung cancers are made up of epithelial tumors with adenocarcinoma being the most common. Sarcomatoid carcinoma of the lung is rare and is present in only 0.52% of all diagnosed cases of NSCLC. The WHO has classified mesenchymal tumors of the lung into vascular, vessel-associated tumors and nonvascular spindle cell tumors. The etiology of sarcomas is usually genetic in nature, a result of environmental toxin exposures, or due to a mutation. CASE PRESENTATION: A 37 year-old female who was six months pregnant, presented with one episode of hemoptysis in the setting of a chronic cough. No history of lung disease or other health conditions. No prior smoking or vaping history. Initial chest X-ray revealed a right lung nodule within the superior segment of the right lower lobe, initially concerning for a pulmonary hamartoma or carcinoid. CT chest was delayed due to her pregnancy, and ultimately deferred until after delivery three months later which showed an enlarging heterogeneous, 3.5 cm x 3.4 cm right lower lobe mass. She refused CT/PET as she was breastfeeding her newborn. She eventually agreed to surgery and underwent video-assisted thoracoscopic surgery (VATS) with mediastinal and regional lymphadenectomy and RLL lobectomy. Pathology revealed a low-grade sarcoma, with multiple analyses concerning for low-grade endometrial stromal sarcoma (ESS) vs. vascular neoplasm. Further molecular analysis with FISH confirmed low-grade vascular sarcoma with regional lymph node involvement (ESS was negative). Her PET scan following surgery was negative for any disease. DISCUSSION: Our patient had a RLL mass showing a malignant spindle cell neoplasm with a positive right hilar lymph node favored to be a low-grade sarcoma. Given its morphology, the differential diagnoses were low-grade endometrial stromal sarcoma, malignant peripheral nerve sheath tumor (MPNST), solitary fibrous tumor (SFT), inflammatory myofibroblastic tumor, PEComa, and synovial sarcoma. A definitive diagnosis is of utmost importance as certain types of sarcoma confer a poorer prognosis. For example, primary pulmonary synovial sarcoma is usually extremely aggressive with a 5-year survival rate of only 50%. CONCLUSIONS: This case is very unique in a few ways. First, it is an uncommon cause for a solitary pulmonary nodule along with being an uncommon malignancy. Second, it presents a complicated diagnostic dilemma in a pregnant patient who was reluctant to have diagnostic imaging and procedures that would have aided in the diagnosis. Reference #1: Hashimoto H, Tsugeno Y, Sugita K, Inamura K (2019) Mesenchymal tumors of the lung: diagnostic pathology, molecular pathogenesis, and identified biomarkers. J Thorac Dis 11:S9–S24 Reference #2: Rahouma M, Kamel M, Narula N, Nasar A, Harrison S, Lee B, Stiles B, Altorki NK, Port JL (2018) Pulmonary sarcomatoid carcinoma: an analysis of a rare cancer from the Surveillance, Epidemiology, and End Results database. Eur J Cardiothorac Surg 53:828–834 DISCLOSURES: No relevant relationships by Bhavik Gupta, source=Web Response No relevant relationships by Bryan Stone, source=Web Response No relevant relationships by Christopher Wyckoff, source=Web Response

Nhân một trường hợp bướu rất hiếm gặp ở lồng ngực sarcôm hoạt dịch nguyên phát ở phổi

A CASE VERY RARE TUMOR IN THORAX PRIMARY PULMONARY SYNOVIAL SARCOMA: CASE REPORT Background:Primary synovial sarcoma of lung is an extremely rare tumor. The etiology and pathogenesis of sarcomas lung are not well understood. A definitive diagnosis requires detailed connecting clinical symptoms, radiological imaging, immunohistochemical staining and may be molecular genetics techniques. Case Report:A male patient, 46 years old, history disease about 5 days with dry cough, dyspnea and right chest pain. Image manifestations: right pleural effusion and lung collapse. He had been taken the right pleural drainage and then the right lung surgery to remove the tumor. Right lung tumors are associated with right pleura, pericardium and mediastinal invasions. The diagnosis is confirmed with immunohistochemical staining of positive specific markers: PanCK, CD 5/6, CD99, Bcl-2, Calretinin, WT-1, Podoplanin. Conclusions: Primary Pulmonary Synovial Sarcom is a mesenchymal malignant disease in the lungs, very rare. The disease progresses rapidly, involving the parenchyma of the lungs, pleura, or pericardium, the lymph nodes or nearby tissues. The diagnosis have determined by immunohistochemistry. Key word: Primary Pulmonary Synovial Sarcoma; Immunohistochemistry; SSX SYT-SSX Fusion Gene.

225 - A case report of the diagnosis of a primary pulmonary synovial sarcoma using endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA) in conjunction with rapid on-site evaluation (ROSE)

225 - A case report of the diagnosis of a primary pulmonary synovial sarcoma using endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA) in conjunction with rapid on-site evaluation (ROSE)

Primary biphasic pulmonary synovial sarcoma of lung: A rare presentation in a young female

Primary biphasic pulmonary synovial sarcoma of lung: A rare presentation in a young female - IJPO- Print ISSN No: - 2394-6784 Online ISSN No:- 2394-6792 Article DOI No:- 10.18231/j.ijpo.2019.060, Indian Journal of Pathology and Oncology-Indian J Pathol Oncol

A case of five-year relapse-free survival after thoracoscopic lobectomy for primary pulmonary synovial sarcoma

A case of five-year relapse-free survival after thoracoscopic lobectomy for primary pulmonary synovial sarcoma