Molecular Pathology 2019 August 1 [Link]
Hung YP, Dong F, Dubuc AM, Dal Cin P, Bueno R, Chirieac LR
Localized pleural mesothelioma is a rare lone circumscribed pleural tumor that is microscopically like to diffuse malignant pleural mesothelioma. However, the molecular characteristics together with nosologic human relationship amongst its diffuse counterpart rest unknown. In a consecutive cohort of 1110 patients amongst pleural mesotheliomas diagnosed in 2005-2018, nosotros identified vi (0.5%) patients diagnosed amongst localized pleural mesotheliomas. We gathered clinical history, evaluated the histopathology, together with inwards guide cases performed karyotypic analysis together with targeted next-generation sequencing. The cohort included 3 women together with 3 men (median historic menses 63; hit 28-76), oftentimes presenting incidentally during radiologic evaluation for unrelated conditions. Neoadjuvant chemotherapy was administered inwards 2 patients. All tumors (median size 5.0 cm; hit 2.7-13.5 cm) demonstrated gross circumscription (with microscopic invasion into lung, soft tissue, and/or rib inwards iv cases), mesothelioma histology (four biphasic together with 2 epithelioid types), together with mesothelial immunophenotype. Of iv patients amongst at to the lowest degree 6-month follow-up, 3 were endure (up to 8.9 years). Genomic characterization identified several subgroups: (1) BAP1 mutations amongst deletions of CDKN2A together with NF2 inwards 2 tumors; (2) TRAF7 mutations inwards 2 tumors, including 1 harboring trisomies of chromosomes 3, 5, 7, together with X; together with (3) genomic near-haploidization, characterized past times extensive loss of heterozygosity sparing chromosomes v together with 7. Localized pleural mesotheliomas seem genetically heterogeneous together with include BAP1-mutated, TRAF7-mutated, together with near-haploid subgroups. While the BAP1-mutated subgroup is like to diffuse malignant pleural mesotheliomas, the TRAF7-mutated subgroup overlaps genetically amongst adenomatoid tumors together with well-differentiated papillary mesotheliomas, inwards which recurrent TRAF7 mutations accept been described. Genomic near-haploidization, identified lately inwards a subset of diffuse malignant pleural mesotheliomas, suggests a new machinery inwards the pathogenesis of both localized pleural mesothelioma together with diffuse malignant pleural mesothelioma. Our findings depict distinctive genetic features of localized pleural mesothelioma, amongst both similarities to together with differences from diffuse malignant pleural mesothelioma.
from Mesothelioma Line https://ift.tt/2Z2CHIn
via IFTTT Sumber http://lawyersmesotheliomalaw.blogspot.com