Department of Anatomic Pathology, The University of Texas MD Anderson Cancer Center, Houston, USA.
Department of Anatomic Pathology, The University of Texas MD Anderson Cancer Center, Houston, USA.
Pathol Res Pract. 2024 Jul;259:155356. doi: 10.1016/j.prp.2024.155356. Epub 2024 May 17.
Four cases of a distinct carcinoma of the thymic gland are presented. The patients were 4 adult males with an age range from 40 to 47 years (mean, 43.5 years). Clinically, all patients presented with non-specific respiratory symptoms. None of the patients had any prior history of head and neck neoplasm or surgery in that anatomic area. Large anterior mediastinal masses were found on diagnostic imaging with concurrent metastatic disease to pleura, lungs, regional lymph nodes and bones. Microscopically, all tumors were composed of a solid proliferation of hyperchromatic, monomorphic small cells with focal cytoplasmic clearing embedded in a fibromyxoid stroma. In one case, occasional duct-like structures were identified. Immunohistochemically, the tumors were positive for pancytokeratin, CD117 and MYB and negative for myoepithelial markers. Systemic chemotherapy was initiated in all patients. Despite therapy, clinical follow-up revealed that all 4 patients died of their disease 11-23 months after their initial diagnosis. The cases in this series highlight a tumor that is different from conventional thymic carcinoma and that has the morphological and immunohistochemical features commonly seen in adenoid cystic carcinomas with high-grade transformation. Correct diagnosis is essential for patient management.
现报告 4 例胸腺腺癌。患者均为 4 名成年男性，年龄 40-47 岁（平均 43.5 岁）。临床均表现为非特异性呼吸系统症状。所有患者均无头颈部肿瘤或该解剖区域手术史。诊断性影像学检查发现大的前纵隔肿块，同时伴有胸膜、肺、局部淋巴结和骨骼转移。显微镜下，所有肿瘤均由增生的深染、形态单一的小细胞组成，伴局灶性胞质空泡，嵌入纤维粘液样基质中。1 例中偶见导管样结构。免疫组化染色示肿瘤细胞表达广谱细胞角蛋白、CD117 和 MYB，而肌上皮标志物阴性。所有患者均接受全身化疗。尽管进行了治疗，但临床随访显示，4 例患者在初始诊断后 11-23 个月均因疾病死亡。本系列病例提示一种与传统胸腺癌不同的肿瘤，具有腺样囊性癌伴高级别转化的形态学和免疫组化特征。正确诊断对患者管理至关重要。
