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Juvenile granulosa cell tumor in a three-year-old infant. An immunohistochemical and ultrastructural study
M Hisaoka1, A Horie, Y Kajiwara
1Department of Pathology, University of Occupational and Environmental Health, Kitakyushu, Japan.
Insights
Juvenile granulosa cell tumors in infants can cause precocious pseudopuberty due to elevated estrogen. Transitional tumor cells, resembling theca cells, are the primary site of steroid hormone synthesis.
Area of Science:
- Gynecologic Oncology
- Pediatric Endocrinology
- Reproductive Biology
Background:
- Juvenile granulosa cell tumors (JGCT) are rare ovarian neoplasms in children.
- Precocious pseudopuberty is a clinical manifestation of estrogen overproduction.
Observation:
- A case of a three-year-old female infant with JGCT presenting with precocious pseudopuberty.
- Elevated serum and urinary estrogen levels were detected.
- Histological examination revealed an immature follicular pattern with theca-like stromal tissue.
Findings:
- Immunohistochemistry localized estradiol, estriol, progesterone, and testosterone in stromal and follicular cells.
- Electron microscopy identified polygonal, spindle, and transitional tumor cells.
- Transitional cells exhibited features of steroid synthesis and were steroid-hormone positive, suggesting a role in estrogen production.
- Spindle cells displayed myofibroblast characteristics, indicating a potential common mesenchymal origin.
Implications:
- Transitional cells in JGCT are likely the main source of steroid synthesis.
- These findings contribute to understanding thePathogenesis of JGCT and associated hormonal imbalances.
- The study suggests a common ovarian mesenchymal progenitor for the diverse tumor cell types.
Abstract:
A three-year-old female infant with a juvenile granulosa cell tumor of the ovary and precocious pseudopuberty is presented. Laboratory tests revealed elevated levels of serum and urinary estrogen. Histological examinations showed an immature follicular pattern surrounded by theca-like stromal tissue. The immunohistochemical localization of estradiol, estriol, progesterone and testosterone was studied by indirect immunoperoxidase staining of cryostat sections. All of these steroid hormones were localized mainly in stromal cells and in some follicular cells. Electron microscopic examination revealed three types of tumor cell, i.e. polygonal cells, spindle cells and transitional cells. Well developed smooth endoplasmic reticulum, mitochondria with tubulovesicular cristae, and lipid droplets in the cytoplasm, which are typical features of steroid synthesis, were conspicuous mainly in the last type of cell. Such transitional cells almost corresponded to steroid-positive cells. These cells were considered to have a close relationship to theca gland cells of developed ovarian follicles and to have mainly participated in steroid synthesis in the present tumor. In addition, some spindle cells showed features of myofibroblasts, such as intracytoplasmic filaments, dense body-like structures, and pinocytotic vesicles. These features suggest that the cells might all be derived from the same progenitor of ovarian mesenchyme.