Abstract
PURPOSE: There are only rough estimates of the worldwide incidence of pilonidal sinus carcinoma. The purpose of the study is to explore the demographic characteristics of this disease and to provide more precise information about its incidence. METHODS: The study included questioning the surgeons and pathologists in Germany in addition to a literature research. The literature investigation included all published articles about pilonidal carcinoma in all languages. The questionnaire included 1050 pathologists and all 834 hospitals with a surgical division in Germany. The outcome measures included the total number of cases, the language of publication, gender, age, country of origin, interval until the diagnosis of carcinoma, and reported incidence based on local studies. RESULTS: From 1900 to 2022, we found 140 cases of pilonidal sinus carcinoma in 103 articles. The investigation revealed two additional unpublished cases from Germany. The male-to-female ratio was 7.75:1. The countries with the most cases were the USA (35 cases, 25.0%), Spain (13 cases, 9.3%), and Turkey (11 cases, 7.6%). The average age was 54.0 ± 11.8 years and the interval between the diagnosis of the disease and the development of carcinoma was 20.1 ± 14.1 years. There was a parallel increase in reported cases of pilonidal sinus disease and pilonidal carcinoma over the last century. The reported incidence varied from 0.03% to 5.56%. The worldwide calculated incidence equaled 0.17%. CONCLUSION: Due to underreporting and other causes, the incidence of carcinoma emerging on the background of pilonidal sinus disease is higher than reported.