Zhao Lingyu, Shen Xiaochen, Niu Yun, Chen Huang, Zhong Dingrong
Department of Pathology, China-Japan Friendship Hospital, Beijing, 100029, China.
Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100006, China.
Respir Med Case Rep. 2025 Jun 25;57:102241. doi: 10.1016/j.rmcr.2025.102241. eCollection 2025.
Primary pulmonary interstitial tumors represent a group of lung diseases. We encountered a case of a pulmonary primary interstitial tumor not previously reported in the literature. Based on existing research, this disease cannot be classified into any known subtype. We propose this disease as a potential new entity pending further validation-Pulmonary Primary Interstitial Tumor-Lymphangioleiomyomatosis-like (PIT-LAM). Histologically, this tumor is located within the interstitium, exhibiting abundant small blood vessels with spindle-shaped tumor cells surrounding them. While morphologically resembling lymphangioleiomyomatosis and hemangioblastoma, the immunohistochemical expression patterns differ. This paper provides a detailed description and analysis of clinical data, pathological examination, immunohistochemistry, along with incorporating a differential diagnostic analysis to advance understanding and foster novel insights into this condition.
原发性肺间质肿瘤是一组肺部疾病。我们遇到了一例文献中此前未报道过的肺原发性间质肿瘤病例。根据现有研究，这种疾病无法归入任何已知亚型。我们提议将这种疾病作为一种潜在的新实体，待进一步验证——肺原发性间质肿瘤-淋巴管平滑肌瘤样（PIT-LAM）。组织学上，该肿瘤位于间质内，有丰富的小血管，周围环绕着梭形肿瘤细胞。虽然形态上类似于淋巴管平滑肌瘤病和成血管细胞瘤，但免疫组化表达模式不同。本文提供了临床数据、病理检查、免疫组化的详细描述和分析，并纳入鉴别诊断分析，以增进对这种疾病的理解并产生新的见解。
