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.
ABSTRACT
INTRODUCTION: Although there are many patient-reported outcome measures used for ventral hernia (VH), disease-specific instruments, such as the Hernia-related Quality-of-Life (QoL) Survey (HerQLes) and Abdominal Hernia-Q (AHQ), have shown greater accuracy in capturing all VH-related QoL. We present a novel calibration that allows providers to convert scores between the AHQ and HerQLes, enabling better unification of QoL data. METHODS: Patients with VH were prospectively identified and simultaneously administered both the AHQ and HerQLes pre- and post-operatively. To ensure the validity of the calibration, responses were excluded if patients answered instruments on different dates or if the responses were discordant on corresponding questions within each instrument. The calibration was estimated using a linear mixed effects model, including linear and quadratic scores, timing of survey relative to surgery and their interactions as fixed effects, and patients as random effects to account for multiple surveys from the same patient. RESULTS: In total, 109 patients were included, responding to 300 pairs of surveys (112 preoperative and 188 postoperative), of which 17 (5.6%) were excluded because of discordant responses. Conversion of the HerQLes to AHQ was most accurate when including whether the survey was completed pre- or post-operatively, with a mean squared error of 0.0091. Similarly, converting the AHQ to HerQLes was most accurate when factoring in the timing of survey administration, with a mean squared error of 0.016. CONCLUSIONS: We present a novel and accurate method to convert scores between the AHQ and HerQLes. Being able to unify QoL data from different PROMs supports efforts to more broadly integrate PROMs in surgery and to understand patient-defined measures of success.