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Precocious puberty caused by an hCG-producing tumor of the septum pellucidum
1Department of Pediatrics, Nihon University School of Medicine, Tokyo, Japan.
Insights
A rare hCG-producing mixed tumor of choriocarcinoma and teratoma was found in a young boy with precocious puberty. This tumor caused elevated human chorionic gonadotropin (hCG) levels but did not follow typical hormone patterns for puberty.
Area of Science:
- Neuroendocrinology
- Pediatric Oncology
- Reproductive Endocrinology
Background:
- Precocious puberty in children requires careful etiological investigation.
- Germ cell tumors can rarely occur in the central nervous system.
- Human chorionic gonadotropin (hCG) producing tumors can mimic pituitary-gonadal axis activation.
Observation:
- A 6-year-old boy presented with rapidly progressing precocious puberty.
- Immunohistochemical and histological examination revealed a mixed tumor (choriocarcinoma and teratoma) in the septum pellucidum.
- The tumor was confirmed to be hCG-producing.
Findings:
- Serum hCG levels were significantly elevated.
- Serum luteinizing hormone (LH) levels were low, as measured by LH immunoradiometric assay (IRMA).
- The patient's hormonal profile did not align with typical patterns of precocious puberty, lacking high basal LH and appropriate LH response to GnRH stimulation.
Implications:
- This case highlights a rare instance of an intracranial hCG-producing germ cell tumor causing precocious puberty.
- It underscores the importance of considering non-pituitary sources of hCG in cases of pubertal abnormalities.
- Accurate hormonal assays and tumor localization are critical for diagnosis and management of such complex pediatric cases.
Abstract:
A 6 year 9 month old boy with rapidly progressing precocious puberty was immunohistochemically and histologically diagnosed as having an hCG-producing mixed tumor consisting of choriocarcinoma and teratoma in the septum pellucidum. His serum hCG was elevated, but the serum LH was low as determined by LH immunoradiometric assay (IRMA). He did not exhibit a characteristic endocrinological pattern, e.g., high basal levels of LH and failure to respond with high LH levels to the LH-RH stimulation test using the conventional LH RIA method.