Peutz-Jeghers syndrome with concurrent lobular endocervical glandular hyperplasia and sex cord tumor with annular tubules: a case report.

BACKGROUND

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by mucocutaneous pigmentation, gastrointestinal hamartomatous polyps, and an increased risk of specific neoplasms, including distinctive gynecological tumors.

CASE PRESENTATION: This study reports a rare case of a 25-year-old woman with a confirmed diagnosis of PJS who presented with watery vaginal discharge. Gynecological evaluation revealed concurrent cervical and ovarian masses. Histopathology of the cervical lesion demonstrated lobular endocervical glandular hyperplasia (LEGH), while the ovarian mass was identified as a sex-cord tumor with annular tubules (SCTATs), exhibiting both simple and complex ring-like tubules surrounding hyaline cores.

CONCLUSION

This case represents an uncommon co-occurrence of LEGH and SCTATs in a single PJS patient, highlighting the broad spectrum of PJS-associated gynecologic pathology. This study underscores the need for early and regular gynecological surveillance in women with PJS.