Female genital tract tumors and gastrointestinal lesions in the Peutz-Jeghers syndrome.
BACKGROUND
Peutz-Jeghers Syndrome (PJS) is a rare genetic disorder characterized by mucocutaneous melanin deposition, and intestinal hamartomatous polyps, with an increased risk of breast, gastrointestinal, and female genital tract cancers.


METHODS
Multiple genital tract neoplasms in a 52-year-old northern Thai woman with PJS are described. The patient presented with abdominal distention. A pelvic ultrasound scan showed a left adnexal mass, diagnosed as mucinous cyst. An ovarian microscopic cystadenoma was diagnosed together with a minimal deviation mucinous adenocarcinoma (MDA) of the uterine cervix and mucinous metaplasia in tubal mucosa and endometrium. Pathological findings warranted a search for evidence of PJS Typical pigmentation at the hard palate and colonoscopic finding of hamartomatous polyps established the diagnosis of PJS. At four-year follow-up, the patient still showed no evidence of tumor recurrence.


CONCLUSIONS
A case of PJS complicated by multiple and contemporaneous genital tract tumors with rare histological findings is presented. The presented case suggests MDA and mucinous metaplasia warrant a search for PJS.
Tantipalakorn
Charuwan
C
Department of Obstetrics and Gynecology, Chiang Mai University, Chiang Mai 50200, Thailand.
Khunamornpong
Surapan
S
Lertprasertsuke
Nirush
N
Tongsong
Theera
T
D002288:Adenocarcinoma, Mucinous / Q000473:pathology / Q000601:surgery; D003537:Cystadenoma / Q000473:pathology / Q000601:surgery; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging; D041981:Gastrointestinal Tract / Q000473:pathology*; D005833:Genital Neoplasms, Female / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000473:pathology*; D005836:Genitalia, Female / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007044:Hysterectomy; D008875:Middle Aged; D010051:Ovarian Neoplasms / Q000473:pathology / Q000601:surgery; D010052:Ovariectomy; D010580:Peutz-Jeghers Syndrome / Q000175:diagnosis* / Q000473:pathology; D012307:Risk Factors; D058994:Salpingectomy; D014463:Ultrasonography