Department of Thoracic Surgery, Kansai Medical University, Osaka, Japan.
Department of Pathology and Laboratory Medicine, Kansai Medical University, Osaka, Japan.
Thorac Cancer. 2022 Aug;13(15):2264-2266. doi: 10.1111/1759-7714.14547. Epub 2022 Jul 2.
Thoracic SMARCA4-deficient undifferentiated tumors are a new type of neoplasm that commonly occur in the mediastinum, progress rapidly, and show a poorer prognosis. We report a case of thoracic SMARCA4-deficient undifferentiated tumor in the right thoracic cavity in a patient with a history of heavy smoking and presenting with respiratory distress and hemoptysis. Imaging showed pleural effusion and thickening. A diagnostic right pleural biopsy yielded multiple white nodules and pale bloody pleural effusion accumulated in the right thoracic cavity. Histopathologically, the tumor cells were large, some exhibited rhabdoid cytology, and they were surrounded by an infiltration of inflammatory cells. These tumor cells were negative for SMARCA4, p40, NUT, and claudin-4, leading to establishing a diagnosis of thoracic SMARCA4-deficient undifferentiated malignancy. We treated the patient with atezolizumab, carboplatin, and nab-paclitaxel. The patient achieved stable disease at 7 months during this study. Although there is no standard treatment of this disease, our reported treatment may contribute to improved prognosis, requiring further research.
胸部缺失 SMARCA4 的未分化肿瘤是一种新型肿瘤，通常发生在纵隔，进展迅速，预后较差。我们报告了一例右胸腔内胸部缺失 SMARCA4 的未分化肿瘤病例，患者有大量吸烟史，表现为呼吸困难和咯血。影像学显示胸腔积液和胸膜增厚。右侧胸腔诊断性胸膜活检取出多个白色小结节和积聚的淡血性胸腔积液。组织病理学上，肿瘤细胞较大，部分呈横纹肌样细胞学特征，周围有炎症细胞浸润。这些肿瘤细胞对 SMARCA4、p40、NUT 和 Claudin-4 均为阴性，从而确立了胸部缺失 SMARCA4 的未分化恶性肿瘤的诊断。我们用阿特珠单抗、卡铂和白蛋白紫杉醇治疗了该患者。在这项研究中，该患者在 7 个月时达到疾病稳定。尽管这种疾病没有标准的治疗方法，但我们报告的治疗方法可能有助于改善预后，需要进一步研究。
