Clinical characteristics and outcomes of patients with well-differentiated papillary peritoneal mesothelial tumors Journal Article
| Authors: | Offin, M.; Aguirre, N.; Yang, S. R.; Sauter, J. L.; Karagkounis, G.; Mohamed, M.; Cercek, A.; Kundra, R.; Zhang, Y.; Wang, H. M.; Morris, M. P.; Ladanyi, M.; Nash, G. M.; Zauderer, M. G. |
| Article Title: | Clinical characteristics and outcomes of patients with well-differentiated papillary peritoneal mesothelial tumors |
| Abstract: | Purpose: Well-differentiated papillary peritoneal mesothelial tumors (WDPMTs) are understudied and discrete from peritoneal mesotheliomas (PMs). We report clinicopathologic characteristics and outcomes of a large prospective WDPMT cohort. Methods: Patients with WDPMT identified between August 2007 and December 2020 were followed through January 2023. Clinical characteristics and outcomes were annotated. Overall survival (OS) was assessed from pathologic diagnosis. Germline variants were analyzed, and targeted next-generation sequencing (NGS; MSK-IMPACT) data were compared to PMs and diffuse pleural mesotheliomas (DPMs). Results: Among 54 patients, median age at diagnosis was 55 (range 20–76), 50% were female (n = 27), and 46% were smokers (n = 25; median 8 pack/years). Most (94%, n = 51) WDPMTs were found during surgical explorations for other indications, primarily other malignancies. Two patients underwent surgical resection for WDPMT; none received systemic therapy for WDPMT. Median OS was not reached (19/54; median follow up 4.5 years). Somatic NGS was available for 35% (19/54) of patients. TRAF7 alterations were enriched in WDPMT (89%; 17/19) compared with PM (0%; 0/50; p < 0.0001) and DPM (0%; 0/74; p < 0.0001). In WDPMT compared with PM and DPM, there were less BAP1 (0% [0/0] vs. 4% [8/50] vs. 46% [34/74]; p = 0.001 and p < 0.0001, respectively) and NF2 (0% [0/0] vs. 24% [12/50] vs. 31% [23/74]; p = 0.03 and p = 0.001 respectively) alterations. Pathogenic germline variants were present in 23% (4/17) of WDPMTs. Conclusions: Well-differentiated papillary peritoneal mesothelial tumors were primarily incidental findings. There was no WDPMT-related mortality, so there was no distinct role for routine cytoreductive surgery or systemic therapy. Genomic profiles can help to differentiate WDPMT from DPM and PM. © The Author(s) 2024. |
| Keywords: | adult; controlled study; human tissue; aged; middle aged; survival rate; young adult; major clinical study; overall survival; genetics; clinical feature; mortality; treatment; outcome assessment; follow up; follow-up studies; prospective study; prospective studies; metabolism; peritoneal neoplasms; cohort analysis; smoking; pathology; tumor marker; family history; mesothelioma; outcomes; therapy; peritoneum mesothelioma; peritoneum tumor; peritoneum; clinical outcome; high throughput sequencing; next-generation sequencing; humans; prognosis; human; male; female; article; neoplasms, mesothelial; biomarkers, tumor; well-differentiated papillary peritoneal mesothelial tumors |
| Journal Title: | Annals of Surgical Oncology |
| Volume: | 31 |
| Issue: | 12 |
| ISSN: | 1068-9265 |
| Publisher: | Springer |
| Date Published: | 2024-11-01 |
| Start Page: | 7973 |
| End Page: | 7977 |
| Language: | English |
| DOI: | 10.1245/s10434-024-16004-2 |
| PUBMED: | 39167353 |
| PROVIDER: | scopus |
| PMCID: | PMC11466981 |
| DOI/URL: | |
| Notes: | The MSK Cancer Center Support Grant (P30 CA008748) is acknowledge in the PDF -- Corresponding authors is MSK author: Michael Offin -- Source: Scopus |
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