Isolated Inhibin-Producing Granulosa Cell Tumor Causing Primary Amenorrhea: A Case Report

Area of Science:

• Gynecologic Oncology
• Pediatric Endocrinology
• Reproductive Endocrinology

Background:

• Juvenile granulosa cell tumors (JGCTs) are the most common pediatric ovarian sex cord-stromal tumors.
• JGCTs are often hormonally active, leading to endocrine dysfunction like precocious puberty or menstrual irregularities.
• Most JGCTs are diagnosed at stage I and have a favorable prognosis with surgical treatment.

Observation:

• A 15-year-old presented with primary amenorrhea, indicating a lack of menstruation.
• Hormonal evaluation showed suppressed FSH, elevated LH, normal estradiol, and markedly elevated inhibin B.
• Imaging revealed a mildly enlarged right ovary with a 1.6-cm "ovarian follicle."

Findings:

• Histopathology confirmed the diagnosis of JGCT after a right salpingo-oophorectomy.
• The patient experienced menarche two months post-surgery, indicating restoration of normal reproductive function.
• Elevated inhibin B was a key indicator in diagnosing this case of JGCT.

Implications:

• Clinical presentation of JGCTs can vary based on tumor endocrine function and pubertal stage.
• Inhibin-producing JGCTs should be considered in the differential diagnosis for pediatric and adolescent patients with amenorrhea.
• Early diagnosis and surgical management of JGCTs lead to favorable outcomes and restoration of reproductive health.