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[Precocious puberty due to HCG secreting pinealoma (author's transl)]
Insights
A rare HCG secreting pineal tumor caused precocious puberty in a young boy. Surgical removal and radiation offered initial recovery, but the condition ultimately progressed despite chemotherapy.
Area of Science:
- Pediatric Endocrinology
- Neuro-oncology
- Oncologic Endocrinology
Background:
- Precocious puberty can stem from various causes, including hormone-secreting tumors.
- Pineal tumors, though rare, can exhibit ectopic hormone production.
Observation:
- A six-year-old boy presented with symptoms of precocious puberty, including polydipsia, voice deepening, and pubic hair development, without testicular growth.
- Elevated levels of human chorionic gonadotropin (HCG) and testosterone, with suppressed follicle-stimulating hormone (FSH), indicated abnormal hormonal activity.
- Diagnostic imaging revealed a pineal tumor (pinealocytoma type I) secreting HCG.
Findings:
- The pineal tumor was surgically removed, and the neoplastic tissue confirmed high HCG and beta-HCG activity.
- Post-operative radiation therapy was administered, leading to rapid initial recovery.
- Despite chemotherapy, the patient's condition deteriorated progressively after several months.
Implications:
- This case highlights the potential for HCG-secreting pineal tumors to cause precocious puberty exclusively in males due to ectopic gonadotropin secretion.
- The findings suggest a comparison between pinealomas and other HCG-secreting carcinomas that manifest sexual precocity in boys.
- Further research into the mechanisms of ectopic hormone secretion by tumors is warranted.
Abstract:
The present report concerns a six years nine months old boy in whom a HCG secreting pineal tumor (pinealocytoma type I) was responsible for precocious puberty. Features : polydipsia, deepened voice, pubic hair, penile enlargement without corresponding testicular growth. Serum basal concentrations of LH (150 mU/ml), testosterone (85.80 nanomole/l), HCG (115 ng/ml) and its beta subunit (15 ng/ml) were markedly elevated, FSH was low (3.9 mU/ml) ] dissociated hypopituitarism was also present. A surgical removal of the tumor was performed (RIA of the neoplastic tissue revealed high HCG and beta HCG activity) and after the operation the child had radiation therapy. Recovery for the foregoing procedure was rapid, but after some months, in spite chemotherapy his condition deteriorated progressively. The apparent limitation of pinealoma and precocious puberty to the male sex is discussed and supposed to be partly the result of the ectopic gonadotropin secretion. These tumors are compared to other HCG secreting carcinomas located elsewhere in the body and causing sexual precocity in boys only.