AlQatari Abdullah Abdulaziz, Ahmed Ayesha, AlHije Fatima, Sabry Mohammed, Elbawab Hatem
Thoracic Surgery Division, Department of General Surgery, King Fahad Hospital of The University, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia.
Department of Pathology, King Fahad Hospital of The University, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia.
Med Arch. 2023;77(6):496-499. doi: 10.5455/medarh.2023.77.496-499.
Synovial sarcoma is a rare and aggressive soft tissue malignancy most commonly arises from periarticular tissue of the extremities. Although several cases in the literature have reported different origins, primary pulmonary synovial sarcoma (PPSS) is an exceedingly rare and underrecognized entity, accounting for 0.5% of all lung malignancies. Clinical presentation includes chest pain, dyspnea, cough, and hemoptysis. The finding of hemothorax is a rare presentation and was barely reported in the literature. Due to its rarity and aggressive nature, the optimal treatment is unclear, while the mainstay remains surgical resection with chemo- and/or radiation therapy.
To report a case of hemorrhagic effusion subsequently diagnosed with primary pulmonary synovial sarcoma with the main objective of enriching the literature regarding this rare malignancy.
A 52-year-old male smoker with a background of coronary artery disease, hypertension, and diabetes mellitus was referred to our hospital. The patient presented with a history of chest pain, dyspnea, and massive right-sided pleural effusion. Laboratory investigations were unremarkable except for anemia. Chest x-ray showed a complete opacity on the right lower zone with right-sided pleural effusion. Thoracentesis was done and revealed hemorrhagic exudative effusion. Computed tomography (CT) scan showed a right heterogeneous lung mass compressing the medial segment of the middle lobe. Subsequently, the patient underwent bronchoscopy, which showed compression and edema on the right middle lobe bronchus with traces of blood coming from the right lower lobe. The patient underwent a right posterolateral thoracotomy, a fungating mass eroding the medial segment of the middle lobe was resected that was diagnosed as high-grade primary pulmonary synovial sarcoma. Radiotherapy was instituted. The patient died after two years due to recurrence.
PPSS is an aggressive disease with poor prognostic outcomes, and Its presentation is almost similar to other lung malignancies. Meanwhile, there is no definitive management guideline, and most management depends on surgical resection if feasible with adjuvant chemo-radiation therapy.
滑膜肉瘤是一种罕见的侵袭性软组织恶性肿瘤，最常见于四肢关节周围组织。尽管文献中有几例报道了不同的起源，但原发性肺滑膜肉瘤（PPSS）极为罕见且未得到充分认识，占所有肺恶性肿瘤的0.5%。临床表现包括胸痛、呼吸困难、咳嗽和咯血。血胸的表现罕见，文献中鲜有报道。由于其罕见性和侵袭性，最佳治疗方法尚不清楚，而主要治疗手段仍是手术切除并辅以化疗和/或放疗。
报告一例随后被诊断为原发性肺滑膜肉瘤的出血性胸腔积液病例，主要目的是丰富关于这种罕见恶性肿瘤的文献。
一名52岁有冠状动脉疾病、高血压和糖尿病病史的男性吸烟者被转诊至我院。患者有胸痛、呼吸困难和大量右侧胸腔积液病史。实验室检查除贫血外无异常。胸部X线显示右下区完全致密影伴右侧胸腔积液。进行了胸腔穿刺，抽出了血性渗出液。计算机断层扫描（CT）显示右肺有一个不均匀肿块压迫中叶内侧段。随后，患者接受了支气管镜检查，显示右中叶支气管受压和水肿，有来自右下叶的微量血迹。患者接受了右后外侧开胸手术，切除了一个侵蚀中叶内侧段的蕈状肿块，诊断为高级别原发性肺滑膜肉瘤。开始进行放疗。患者两年后因复发死亡。
PPSS是一种侵袭性疾病，预后不良，其表现与其他肺恶性肿瘤几乎相似。同时，没有明确的管理指南，大多数治疗取决于可行时的手术切除及辅助放化疗。
