Uterine Inflammatory Myofibroblastic Tumors: Proposed Risk Stratification Model Using Integrated Clinicopathologic and Molecular Analysis.
Publication Title
The American journal of surgical pathology
Document Type
Article
Publication Date
2-1-2023
Keywords
oregon; portland; chiles; Female; Humans; Middle Aged; Anaplastic Lymphoma Kinase; Receptor Protein-Tyrosine Kinases; Neoplasms, Connective and Soft Tissue; Granuloma, Plasma Cell; Uterus; Risk Assessment
Abstract
Inflammatory myofibroblastic tumor (IMT) of the uterus is a rare mesenchymal tumor with largely benign behavior; however, a small subset demonstrate aggressive behavior. While clinicopathologic features have been previously associated with aggressive behavior, these reports are based on small series, and these features are imperfect predictors of clinical behavior. IMTs are most commonly driven by ALK fusions, with additional pathogenic molecular alterations being reported only in rare examples of extrauterine IMTs. In this study, a series of 11 uterine IMTs, 5 of which demonstrated aggressive behavior, were evaluated for clinicopathologic variables and additionally subjected to capture-based next-generation sequencing with or without whole-transcriptome RNA sequencing. In the 6 IMTs without aggressive behavior, ALK fusions were the sole pathogenic alteration. In contrast, all 5 aggressive IMTs harbored pathogenic molecular alterations and numerous copy number changes in addition to ALK fusions, with the majority of the additional alterations present in the primary tumors. We combined our series with cases previously reported in the literature and performed statistical analyses to propose a novel clinicopathologic risk stratification score assigning 1 point each for
Area of Special Interest
Women & Children
Area of Special Interest
Cancer
Specialty/Research Institute
Oncology
Specialty/Research Institute
Obstetrics & Gynecology
Specialty/Research Institute
Pathology & Laboratory Medicine
DOI
10.1097/PAS.0000000000001987
