Clear Cell Stromal Tumor of Lung: A Clinicopathologic, Immunohistochemical, and Molecular Characterization of 8 Cases

Igor Odintsov; Alexandra Isaacson; Karen J Fritchie; Yin P Hung; Pooria Khoshnoodi; Lynette M Sholl; Christopher D M Fletcher; William J Anderson

doi:10.1016/j.modpat.2024.100632

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Clear Cell Stromal Tumor of Lung: A Clinicopathologic, Immunohistochemical, and Molecular Characterization of 8 Cases

Journal article

Peer reviewed

Clear Cell Stromal Tumor of Lung: A Clinicopathologic, Immunohistochemical, and Molecular Characterization of 8 Cases

Igor Odintsov, Alexandra Isaacson, Karen J Fritchie, Yin P Hung, Pooria Khoshnoodi, Lynette M Sholl, Christopher D M Fletcher and William J Anderson

Modern pathology, Vol.38(1), 100632

01/2025

DOI: 10.1016/j.modpat.2024.100632

PMID: 39424226

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Abstract

Clear cell stromal tumor (CCST) is a recently described mesenchymal neoplasm of the lung, characterized by spindle cells with variably clear-to-pale eosinophilic cytoplasm and prominent vascularity, as well as a recurrent YAP1::TFE3 gene fusion in most cases. Diagnosis can be challenging given its rarity and the lack of supportive immunohistochemical markers aside from TFE3. To date, less than 20 cases have been reported and data on clinical behavior are also limited. While most appear to be benign, aggressive behavior has been reported rarely. Here, we present the largest multi-institutional series of CCST to date, comprising a total of 8 cases and including 6 previously unpublished cases. We investigate its clinicopathologic and genomic features, while also assessing the diagnostic utility of immunohistochemistry (IHC) for YAP1 C-terminus (YAP-CT). Five patients were male and three were female. The median age was 59 years (range: 35 - 84). In all cases, a TFE3 rearrangement was demonstrated by either FISH or DNA / RNA sequencing. In 7 tumors, the YAP1::TFE3 fusion was identified by sequencing. We demonstrate that the combination of YAP1-CT loss and TFE3 overexpression by IHC reliably predicts an underlying YAP1::TFE3 fusion in these neoplasms and may be more sensitive than TFE3 FISH. Although the median follow-up time for our study was short (18 months, available in 7 cases), all cases pursued a benign clinical course, with no recurrences or metastases. Our study provides further characterization of this novel entity, supporting its wider recognition.

Immunohistochemistry

YAP1

mesenchymal

Pulmonary

TFE3

Details

Title: Subtitle: Clear Cell Stromal Tumor of Lung: A Clinicopathologic, Immunohistochemical, and Molecular Characterization of 8 Cases
Creators: Igor Odintsov - Brigham and Women's Hospital
Alexandra Isaacson - Cleveland Clinic
Karen J Fritchie - Cleveland Clinic
Yin P Hung - Massachusetts General Hospital
Pooria Khoshnoodi - University of Vermont Medical Center
Lynette M Sholl - Brigham and Women's Hospital
Christopher D M Fletcher - Brigham and Women's Hospital
William J Anderson - Brigham and Women's Hospital
Resource Type: Journal article
Publication Details: Modern pathology, Vol.38(1), 100632
DOI: 10.1016/j.modpat.2024.100632
PMID: 39424226
NLM abbreviation: Mod Pathol
ISSN: 0893-3952
eISSN: 1530-0285
Publisher: ELSEVIER SCIENCE INC
Grant note: Department of Pathology at Brigham and Women's Hospital
This work was supported by academic funds from the Department of Pathology at Brigham and Women's Hospital.
Language: English
Electronic publication date: 10/16/2024
Date published: 01/2025
Academic Unit: Pathology
Record Identifier: 9984737228402771

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