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[Juvenile granulosa cell tumor].
A Zuntová1, K Motlík, V Smelhaus
1Patologicko-anatomický ústav fakulty dĕtského lékarství Univerzity Karlovy, Praha.
Sbornik Lekarsky
|April 1, 1990
Summary
Juvenile granulosa cell tumors are rare ovarian tumors primarily affecting young girls. This study presents two cases of bilateral and unilateral tumors causing isosexual pseudopuberty.
Area of Science:
- Gynecologic Oncology
- Pediatric Endocrinology
- Pathology
Background:
- Juvenile granulosa cell tumors (JGCTs) are rare gonadal stromal tumors.
- Defined by Scully in 1976, they predominantly affect girls in their first two decades of life.
- Characterized microscopically by blastemoid structures, solid follicular formations, and cysts.
Observation:
- Case 1: An 8-month-old girl presented with a bilateral JGCT.
- Case 2: A 9-month-old girl had a unilateral tumor, likely a less differentiated JGCT variant.
- Both cases exhibited symptoms of isosexual pseudopuberty.
Findings:
- Histological examination revealed characteristic features of JGCTs.
- The tumors were classified as poorly differentiated gonadal-stromal tumors (ICD-O code M-8590/1).
- Tumor bilaterality was noted in one case.
Implications:
- Highlights the importance of early diagnosis of JGCTs in infants presenting with precocious puberty.
- Emphasizes the need for careful pathological assessment to differentiate JGCT variants.
- Contributes to understanding the clinical presentation and classification of these rare pediatric tumors.