Hideyuki Ishida1, Yusuke Tajima2, Tsuyoshi Gonda2, Kensuke Kumamoto2, Keiichiro Ishibashi2, Takeo Iwama2. 1. Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Saitama, 350-8550, Japan. 05hishi@saitama-med.ac.jp. 2. Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Saitama, 350-8550, Japan.
Abstract
PURPOSE: To investigate the recent incidence of malignant tumors associated with Peutz-Jeghers syndrome (PJS) in Japan to clarify if there are any differences in malignant tumor risk and the spectrum of malignancies by reviewing the literature on this subject. METHODS: We reviewed PJS cases reported in 1115 papers in Japan between January, 1989 and December, 2014. RESULTS: Malignant tumors were identified in 186 of the total 583 PJS cases from 523 evaluable studies. The estimated cumulative risk of a malignant tumor was 83.0 % at 70 years of age. Compared with a previous study, on a collective 91 cases reported up until 1988 in Japan, the reported proportion of gastrointestinal malignancies decreased, from 82.4 to 48.3 %, whereas that of gynecological malignancies increased, from 8.8 to 34.3 % (P < 0.01). Moreover, breast cancers were occasionally reported (4.8 %), even though none were reported in the previous study. Adenocarcinoma of the uterine cervix was the most common malignant tumor (46.8 %) among women with PJS. CONCLUSIONS: The increased number of reports of cervical adenocarcinoma in women with PJS is the prominent trend in Japan, and a subject of concern among gynecologists. The risk of breast cancer seems to be increasing, but confirmation of this trend will require further investigation.
PURPOSE: To investigate the recent incidence of malignant tumors associated with Peutz-Jeghers syndrome (PJS) in Japan to clarify if there are any differences in malignant tumor risk and the spectrum of malignancies by reviewing the literature on this subject. METHODS: We reviewed PJS cases reported in 1115 papers in Japan between January, 1989 and December, 2014. RESULTS:Malignant tumors were identified in 186 of the total 583 PJS cases from 523 evaluable studies. The estimated cumulative risk of a malignant tumor was 83.0 % at 70 years of age. Compared with a previous study, on a collective 91 cases reported up until 1988 in Japan, the reported proportion of gastrointestinal malignancies decreased, from 82.4 to 48.3 %, whereas that of gynecological malignancies increased, from 8.8 to 34.3 % (P < 0.01). Moreover, breast cancers were occasionally reported (4.8 %), even though none were reported in the previous study. Adenocarcinoma of the uterine cervix was the most common malignant tumor (46.8 %) among women with PJS. CONCLUSIONS: The increased number of reports of cervical adenocarcinoma in women with PJS is the prominent trend in Japan, and a subject of concern among gynecologists. The risk of breast cancer seems to be increasing, but confirmation of this trend will require further investigation.
Authors: A D Beggs; A R Latchford; H F A Vasen; G Moslein; A Alonso; S Aretz; L Bertario; I Blanco; S Bülow; J Burn; G Capella; C Colas; W Friedl; P Møller; F J Hes; H Järvinen; J-P Mecklin; F M Nagengast; Y Parc; R K S Phillips; W Hyer; M Ponz de Leon; L Renkonen-Sinisalo; J R Sampson; A Stormorken; S Tejpar; H J W Thomas; J T Wijnen; S K Clark; S V Hodgson Journal: Gut Date: 2010-07 Impact factor: 23.059
Authors: L A Boardman; S N Thibodeau; D J Schaid; N M Lindor; S K McDonnell; L J Burgart; D A Ahlquist; K C Podratz; M Pittelkow; L C Hartmann Journal: Ann Intern Med Date: 1998-06-01 Impact factor: 25.391