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.

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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
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article

A potential new entity pending further validation of pulmonary primary interstitial Tumor: Lymphangioleiomyomatosis-like

Xiaochen Shen, Dingrong Zhong, Huang Chen, Lingyu Zhao et al.
Respiratory Medicine Case Reports
Tuberous Sclerosis Complex Research
article

A potential new entity pending further validation of pulmonary primary interstitial Tumor: Lymphangioleiomyomatosis-like

Xiaochen Shen, Dingrong Zhong, Huang Chen, Lingyu Zhao, Yun Niu
article en

Abstract

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.

Respiratory Medicine Case ReportsVol. 57
Chinese Academy of Medical Sciences & Peking Union Medical College (CN), China-Japan Friendship Hospital (CN), Peking Union Medical College Hospital (CN)
Good health and well-being
Openalex Percentile: Top 80%
Tuberous Sclerosis Complex Research
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A potential new entity pending further validation of pulmonary primary interstitial Tumor: Lymphangioleiomyomatosis-like — Xiaochen Shen, Dingrong Zhong, et al. · Respiratory Medicine Case Reports (2025) | TGRS Research Map | TGRS