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A middle-aged woman with a history of gastrointestinal polyps presented with abnormal uterine bleeding and an enlarged, barrel-shaped cervix. The patient underwent hysterectomy and salpingo-oophorectomy, which revealed minimal deviation adenocarcinoma of the cervix.
Which disorder is most likely in this patient?
von Hippel-Lindau disease
Peutz-Jeghers syndrome (PJS)
Answer: d. Peutz-Jeghers syndrome
Peutz-Jeghers syndrome is an autosomal dominant disorder usually involving mutation in the STK11 gene. Affected patients classically have hyperpigmentation of the oral cavity and distal upper and lower extremities and hamartomatous gastrointestinal polyps. Patients with PJS have an increased risk for various cancers, particularly gastrointestinal and pancreaticobiliary adenocarcinoma. Gynecologic neoplasms that can be seen in PJS include cervical minimal deviation adenocarcinoma (adenoma malignum) and ovarian sex cord tumor with annular tubules, which is often small or microscopic, calcified, and hyperestrogenic.