背景:肺肉瘤样癌(PSC),一种罕见的肿瘤,占所有恶性肺部肿瘤的0.1%至0.4%。鉴于PSC的稀有性,它的临床过程,治疗指南,和患者的结果仍然很大程度上未知。因此,必须提醒临床医生注意这种极为罕见且具有指导性的早发性癌症.
方法:本报告描述了一名28岁女性患有PSC,最初被误诊为Whipple病.经过仔细的临床检查,对PSC做出了决定性的诊断。成像,和患者活检样本的组织病理学评估。放射学成像显示患者左上叶有多个结节和肿块形成,最大尺寸为5.4×3.2厘米。
方法:组织病理学检查表明存在与坏死相关的恶性肿瘤,提示肉瘤,病理分期为cT4N1M1。
结果:阿霉素和异环磷酰胺的治疗方案,导致稳定的疾病状态。
结论:稀有性和肿瘤起源挑战诊断,强调组织学检查的重要作用,免疫组织化学,和流式细胞术,以实现准确的诊断。本报告总结了现有出版物,以全面概述PSC,包括其临床表现,射线成像,病理特征,诊断挑战,治疗策略,和预后,旨在提高对PSC的理解。
BACKGROUND: Pulmonary sarcomatoid carcinoma (PSC), a rare tumor, comprises 0.1% to 0.4% of all malignant lung tumors. Given the rarity of PSC, its clinical course, therapeutic guidelines, and patient outcomes remain largely unknown. Therefore, it is imperative to alert clinicians to this extremely rare and instructive early-onset cancer.
METHODS: This report describes a 28-year-old woman with PSC, who was initially misdiagnosed with Whipple\'s disease. A conclusive diagnosis of PSC was made following careful clinical examination, imaging, and histopathological evaluation of the patient\'s biopsy sample. Radiological imaging revealed multiple nodules and mass formations in the left upper lobe of the patient\'s lung, with the largest measuring of 5.4 × 3.2 cm.
METHODS: Histopathological examination indicated the presence of a malignant neoplasm associated with necrosis suggestive of sarcoma, which was pathologically staged as cT4N1M1.
RESULTS: A regimen of doxorubicin and ifosfamide was administered therapeutically, resulting in a stable disease state.
CONCLUSIONS: The rarity and tumor origin challenge the diagnosis, which emphasizes the imperative role of histological examination, immunohistochemistry, and flow cytometry in achieving an accurate diagnosis. This report summarizes the existing publications to provide a comprehensive overview of PSC, including its clinical manifestations, radiographic imaging, pathologic features, diagnostic challenges, treatment strategies, and prognosis, and aims to improve the understanding of PSC.