A potential new entity pending further validation of pulmonary primary interstitial Tumor: Lymphangioleiomyomatosis-like
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.
Authors
- Xiaochen Shen
- Dingrong Zhong (ORCID: https://orcid.org/0000-0002-6229-1830)
- Huang Chen (ORCID: https://orcid.org/0000-0003-4233-292X)
- Lingyu Zhao (ORCID: https://orcid.org/0000-0002-5183-4625)
- Yun Niu (ORCID: https://orcid.org/0009-0006-5965-8917)
Institutions
- Chinese Academy of Medical Sciences & Peking Union Medical College (CN)
- China-Japan Friendship Hospital (CN)
- Peking Union Medical College Hospital (CN)
Publication Details
- Journal
- Respiratory Medicine Case Reports
- Published
- 2025-01-01
- DOI
- https://doi.org/10.1016/j.rmcr.2025.102241
- Primary Topic
- Tuberous Sclerosis Complex Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00