Dysgerminoma with Estrogen-Producing Functioning Stroma Presenting Precocious Puberty
Shunsuke Nagase1, Kanako Ogura1, Karin Ashizawa1
1Department of Diagnostic Pathology, Juntendo University Nerima Hospital, Tokyo, Japan.
Case Reports in Pathology
|May 31, 2021
Summary
This study reports the first case of ovarian dysgerminoma causing precocious puberty in a child. The tumor
Area of Science:
- Gynecology
- Pediatric Endocrinology
- Oncology
Background:
- Dysgerminoma, a malignant ovarian germ cell tumor, rarely presents with endocrine manifestations.
- Precocious puberty is typically associated with other ovarian pathologies.
Observation:
- A 7-year-old girl presented with precocious puberty (Tanner stage 3 breast development).
- Elevated serum estradiol (E2) and suppressed gonadotropins (luteinizing hormone [LH] and follicle-stimulating hormone [FSH]) were noted.
- Microscopic examination of the ovarian mass revealed dysgerminoma with estrogen-producing theca-like spindle cells.
Findings:
- Immunohistochemistry confirmed steroidogenic enzyme expression (P450 aromatase) in the spindle cells, indicating estrogen biosynthesis.
- The tumor was diagnosed as pure dysgerminoma with an estrogen-producing functioning stroma.
- Post-operative normalization of serum E2 and gonadotropin levels confirmed the tumor's endocrine activity.
Implications:
- This case highlights that conventional dysgerminomas can cause endocrine abnormalities through functioning stroma.
- It underscores the importance of considering ovarian tumors in the differential diagnosis of precocious puberty.
- This finding expands the known clinical presentations of ovarian dysgerminoma.
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