Female genital tract tumors and gastrointestinal lesions in the Peutz-Jeghers syndrome
Tantipalakorn, C.; Khunamornpong, S.; Lertprasertsuke, N.; Tongsong, T.
Journal of the Medical Association of Thailand 92(12): 1686-1690
2009
ISSN/ISBN: 0125-2208 PMID: 20043574 Document Number: 12656
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. 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. 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.
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