Hemangioblastoma-like Clear Cell Stromal Tumor of the Lung: A Comprehensive Review With Insights From a Case.

Abstract

Hemangioblastoma-like clear cell stromal tumor (HLCCST) of the lung is an exceptionally rare mesenchymal neoplasm that is generally considered benign. Current knowledge of this tumor remains limited. Histologically, HLCCST is defined by a hypervascular stroma with dilated blood vessels and is predominantly composed of epithelial-like cells arranged in solid sheets or nests with uniform morphology. Molecular genetic studies have identified YAP1::TFE3 gene fusions as a hallmark in most reported cases. To date, in addition to our case, a total of 19 HLCCST cases have been reported across 7 publications. Here, we present a comprehensive review of HLCCST, detailing its clinicopathologic features, key molecular alterations, and prognostic data of HLCCST. In addition, we emphasize the importance of accurate recognition and diagnosis of this rare tumor to ensure appropriate treatment and improved patient outcomes.