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. 2024 Nov;31(12):7973-7977.
doi: 10.1245/s10434-024-16004-2. Epub 2024 Aug 21.

Clinical Characteristics and Outcomes of Patients with Well-Differentiated Papillary Peritoneal Mesothelial Tumors

Michael Offin #  1   2 , Nicole Aguirre #  3 , Soo Ryum Yang  4 , Jennifer L Sauter  4 , Georgios Karagkounis  3 , Mostafa Mohamed  3 , Andrea Cercek  5 , Ritika Kundra  4 , Yanming Zhang  4 , Hui Mei Wang  4 , Marty P Morris  3 , Marc Ladanyi  4 , Garrett M Nash #  3 , Marjorie G Zauderer #  6   7
Affiliations

Clinical Characteristics and Outcomes of Patients with Well-Differentiated Papillary Peritoneal Mesothelial Tumors

Michael Offin et al. Ann Surg Oncol. 2024 Nov.

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.

Keywords: Mesothelioma; Next-generation sequencing; Outcomes; Treatment; Well-differentiated papillary peritoneal mesothelial tumors.

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Figures

Fig. 1
Fig. 1
Oncoprint comparing the genomic landscapes of patients with well-differentiated papillary peritoneal mesothelial tumors (WDPMT; n = 19) versus previously published data from patients with peritoneal mesotheliomas (PM; n = 50) and diffuse pleural mesotheliomas (DPM; The Cancer Genome Atlas [TCGA]; n = 74)
Fig. 2
Fig. 2
Characteristics and first-degree family history of cancers in patients with well-differentiated papillary peritoneal mesothelial tumors (WDPMT) and germline mutations detected by next-generation sequencing of biopsy samples
See this image and copyright information in PMC

References

    1. Shrestha R, Nabavi N, Volik S, et al. Well-differentiated papillary mesothelioma of the peritoneum is genetically distinct from malignant mesothelioma. Cancers. 2020. 10.3390/cancers12061568. - PMC - PubMed
    1. Bueno R, Stawiski EW, Goldstein LD, et al. Comprehensive genomic analysis of malignant pleural mesothelioma identifies recurrent mutations, gene fusions and splicing alterations. Nat Genet. 2016;48:407–16. 10.1038/ng.3520. - PubMed
    1. Malpica A, Sant’Ambrogio S, Deavers MT, Silva EG. Well-differentiated papillary mesothelioma of the female peritoneum: a clinicopathologic study of 26 cases. Am J Surg Pathol. 2012;36:117–27. 10.1097/PAS.0b013e3182354a79. - PubMed
    1. Sun M, Zhao L, Weng Lao I, et al. Well-differentiated papillary mesothelioma: a 17-year single institution experience with a series of 75 cases. Ann Diagn Pathol. 2019;38:43–50. 10.1016/j.anndiagpath.2018.10.012. - PubMed
    1. Churg A, Galateau-Salle F. Well differentiated papillary mesothelial tumor: a new name and new problems. Mod Pathol. 2022;35:1327–33. 10.1038/s41379-022-01082-y. - PubMed

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