J Med Assoc Thai 2009; 92 (12):1686

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Female Genital Tract Tumors and Gastrointestinal Lesions in the Peutz-Jeghers Syndrome
Tantipalakorn C Mail, Khunamornpong S , Lertprasertsuke N , Tongsong T

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.

Case Report:
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.

Conclusion:
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.

Keywords:
Peutz-Jeghers Syndrome, Female genital tract tumor, Minimal deviation adenocarcinoma

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