Update on our investigation of malignant tumors associated with Peutz-Jeghers syndrome in Japan.
Surg Today
; 46(11): 1231-42, 2016 Nov.
Article
em En
| MEDLINE
| ID: mdl-26746637
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.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias da Mama
/
Síndrome de Peutz-Jeghers
/
Adenocarcinoma
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Neoplasias do Colo do Útero
/
Neoplasias Gastrointestinais
Tipo de estudo:
Etiology_studies
/
Incidence_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Adolescent
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Adult
/
Aged
/
Aged80
/
Child
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Female
/
Humans
/
Middle aged
País/Região como assunto:
Asia
Idioma:
En
Revista:
Surg Today
Ano de publicação:
2016
Tipo de documento:
Article
País de afiliação:
Japão