What question did this study set out to answer?

To synthesize current knowledge about clear cell stromal tumor of the lung, focusing on its pathogenesis and clinicopathological features.

February 13, 2026Open Access

A comprehensive review of clear cell stromal tumor of the lung: integrating clinicopathology and molecular pathogenesis

LLLiping LuoDaping Hospital LLLiang LvThird People's Hospital of Huzhou YZYing ZengThird People 's Hospital of Chongqing

Key Points

To synthesize current knowledge about clear cell stromal tumor of the lung, focusing on its pathogenesis and clinicopathological features.
Reviewed existing literature on clear cell stromal tumor
Documented characteristics and molecular findings from cases
Analyzed clinicopathological data associated with diagnosis
Identified a recurrent YAP1::TFE3 gene fusion in most cases
Described the tumor's histological features, including cell types and vascularity
Highlighted challenges in diagnosis due to the rarity and lack of specific markers

Abstract

Clear cell stromal tumor (CCST) is a newly recognized mesenchymal neoplasm of the lung, with a recurrent YAP1::TFE3 gene fusion in most cases and characterized histologically by spindle, epithelioid cells, with variably clear to pale eosinophilic cytoplasm and prominent vascularity. Due to its rarity and the absence of specific immunohistochemical markers beyond TFE3, diagnosis remains challenging. Currently, only thirty cases have been documented. This narrative literature review synthesizes current advances to enhance understanding of this entity.

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