Malignant Mesothelioma With EWSR1-ATF1 Fusion in Two Adolescent Male Patients. (November 2021)

• Record Type: Journal Article
• Authors: Ren, Hezhen; Rassekh, S Rod; Lacson, Atilano; Lee, Cheng-Han; Dickson, Brendan C; Chung, Catherine T; Lee, Anna F
• Is Part Of: Pediatric and developmental pathology. Volume 24:Number 6(2021)
• Page Start: 570
• Page End: 574
• Publication Date: 2021-11
• Subjects: peritoneum -- pericardium -- mesothelioma -- RNA sequencing -- immunohistochemistry -- EWSR1 -- gene rearrangement; Pediatric pathology -- Periodicals; Children -- Diseases -- Periodicals; Diagnosis, Laboratory -- Periodicals; Abnormalities, Human -- Periodicals; Child development -- Periodicals; Pediatrics -- Periodicals; 616.07
• DOI: 10.1177/10935266211021222
• Languages: English
• ISSNs: 1093-5266

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.