Clinicopathologic comparison of sporadic and syndromic Peutz-Jeghers polyps
Peutz-Jeghers polyps are hamartomatous polyps that may define patients with Peutz-Jeghers syndrome (PJS), a rare inherited polyposis syndrome with high cancer risk. However, the clinical significance of sporadic Peutz-Jeghers polyps (PJPs) without other PJS stigmata relative to malignant potential and identification of new PJS probands is still unclear. The authors conducted a study to compare sporadic and syndromic PJPs in terms of clinicopathologic features. They identified 112 patients with 524 histologically confirmed PJPs and categorized them, based on number of polyps, into syndromic (three or more PJPs or diagnosed PJS, n=38), solitary (one PJP, n=61), and intermediate (two PJPs, n=13). Clinicopathologic features, including dysplasia in the polyp and development of neoplasia in the patient, were compared on a per patient and per polyp basis. Unlike patients with solitary and intermediate PJPs, who did not differ from each other, patients with syndromic PJPs were, in multivariate analysis, younger (P=.001) and more likely to develop neoplasia (P=.02) during a 62.6-month median follow-up than patients with sporadic PJPs. On an individual polyp basis, syndromic PJPs were more likely, in multivariate analysis, to occur in the small intestine (P<.001). These data suggest that strict criteria for PJS stratify patients into distinct groups with significant differences in clinicopathologic parameters, particularly regarding risk of neoplasia. Furthermore, it is important to recognize and remove sporadic PJPs to keep a polyp count for considering a PJS diagnosis in the future and because they may harbor dysplasia.
Liu BL, Ward SC, Polydorides AD. Clinicopathologic comparison between sporadic and syndromic Peutz-Jeghers polyps. Hum Pathol. 2023;141:69–77.
Correspondence: Dr. Alexandros D. Polydorides at alexandros.polydorides@mountsinai.org
ISUP consensus conference on issues in bladder cancer: comparison of grading system performance
Grade is a key prognostic factor in determining progression in nonmuscle invasive papillary urothelial carcinomas. The two most common grading methods are the World Health Organization (WHO) 2004 and 1973 schemes. The International Society of Urological Pathology (ISUP) organized a 2022 consensus conference on issues in bladder cancer and tasked its working group one with making recommendations for future iterations of bladder cancer grading. The ISUP, in collaboration with the European Association of Urology, developed a 10-question survey, which the organizations circulated to their memberships. The intent of the survey was to determine how pathologists and urologists were using grading schemes and to ascertain areas potentially needing improvement. An additional survey was circulated to the ISUP membership to obtain their opinions about interobserver variability in grading, urine cytology reporting practices, and challenges encountered in assigning grades. Working group one conducted comprehensive literature reviews of bladder cancer grading prognosis, interobserver variability, and the Paris System for reporting urine cytology, which were compared with survey results. Survey questions answered by the ISUP membership indicated notable differences in practice patterns between North American and European pathologists in terms of the grading scheme used and the frequency of diagnosis of papillary urothelial neoplasm of low malignant potential. Areas of common ground included difficulty in assigning grades, a desire to improve grading criteria, and a move toward subclassifying high-grade urothelial carcinomas. After working group one reviewed the survey results at the aforementioned consensus conference, the topics were discussed and the in-person meeting participants, including pathologists and urologists, were polled electronically. The surveys and in-person voting demonstrated a strong preference to refine current grading into a three-tier scheme with the division of WHO 2004 high grade into clinically relevant categories. Opinions varied more with regard to using the term papillary urothelial carcinoma with low malignant potential.
Downes MR, Hartmann A, Shen S, et al. International Society of Urological Pathology (ISUP) Consensus Conference on current issues in bladder cancer. Working group 1: Comparison of bladder cancer grading system performance. Am J Surg Pathol. 2024;48(1):e1–e10.
Correspondence: Dr. Michelle Downes at michelle.downes@sunnybrook.ca