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Malignant Mesothelioma With <i>EWSR1-ATF1</i> Fusion in Two Adolescent Male Patients

Hezhen Ren, Shahrad R. Rassekh, Atilano Lacson, Cheng‐Han Lee, Brendan C. Dickson, Catherine T. Chung, Anna F. Lee

2021Pediatric and Developmental Pathology22 citationsDOIOpen Access PDF

Abstract

Malignant mesothelioma is a neoplasm of serosal surfaces, most commonly affecting the pleura. The peritoneum, pericardium, and tunica vaginalis are less frequently involved. Malignant mesothelioma with EWSR1-ATF1 fusion in young adults was recently reported in the literature. Here, we present two pediatric cases of EWSR1-ATF1 translocation-associated malignant mesothelioma in the peritoneum and pericardium respectively. Both cases lacked a known exposure history. Microscopy in both cases showed predominantly epithelioid morphology with ample eosinophilic cytoplasm, and immunohistochemistry was positive for pan-keratin, calretinin, and WT1. Both cases showed EWSR1-ATF1 gene rearrangement by RNA sequencing, which was instrumental in confirming the diagnosis of malignant mesothelioma and to exclude more common pediatric sarcomas, especially in the context of limited sampling.

Topics & Concepts

MesotheliomaPeritoneumCalretininPathologyPericardiumContext (archaeology)Fusion geneAsbestosImmunohistochemistryMedicineSarcomaBiologyInternal medicineGeneMaterials scienceBiochemistryMetallurgyPaleontologyOccupational and environmental lung diseasesInterstitial Lung Diseases and Idiopathic Pulmonary FibrosisMedical Imaging and Pathology Studies
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