Objective
To investigate the clinicopathological features and clinical subtypes of Peutz-Jeghers syndrome (PJS) in Chinese cases.
Methods
The clinical and pathological data of 295 patients with PJS who were treated in Air Force General Hospital from Nov. 1994 to Aug. 2017 were retrospectively analyzed and a multifactor statistical study was carried out on.
Results
Two hundreds and ninety-five patients with PJS belonged to 7 nationalities and came from 26 provinces and urban areas. 99.0% (292/295) of the patients had black spots on the lip and buccal mucosa, and the median occurrence time was 2 years old (0–33 years). The median age of inital diagnosis and treatment was 15 years old (1–45 years). The median interval time between the occurrence of black spots and abdominal symptoms was about 10 years (0–45 years). PJS hamartoma polyps were found in alimentary canals of 293 patients (99.3%), and 96.9% distributed in the duodenum and small intestine (n=284), 90.4% distributed in the colorectal (n=265), 79.9% distributed in the stomach (n=234). Patients of black spot appearing at age <3 years and (or) initial treatment at age <14 years were classified as early-onset subtype, otherwise they could be included in delayed-onset subtype.
Conclusions
The clinical features of PJS are prominent and the harm of PJS hamartoma polyps is serious. The black spots on the lip and buccal mucosa can be used as an early warning signal to divide the PJS patients into 2 clinical subtypes, which should be differentiated in clinical therapy and follow-up strategy.
