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Pubertal dysfunctions in intracranial germ cell tumors
Cristina Partenope1,2, Sabrina Criscuolo2,3, Fernando Carceller4
1Division of Pediatrics, Department of Health Science University of Piemonte Orientale, Ospedale Maggiore della Carità, Novara, Italy.
Insights
Pediatric intracranial germ cell tumors (IC-GCTs) frequently cause pubertal disorders like pseudo-precocious puberty (PPP) and hypogonadotrophic hypogonadism (HH). Early endocrine evaluation and long-term follow-up are crucial for managing these conditions in children.
Area of Science:
- Pediatric Endocrinology
- Neuro-oncology
- Oncology
Background:
- Pediatric intracranial germ cell tumors (IC-GCTs) are associated with significant endocrine complications, particularly pubertal dysfunctions.
- These disorders can manifest at diagnosis or during the follow-up period, impacting growth and development.
Purpose of the Study:
- To determine the prevalence and types of pubertal disorders in pediatric patients diagnosed with IC-GCTs.
- To analyze the patterns and evolution of these endocrine dysfunctions over time.
Main Methods:
- Retrospective analysis of clinical, radiological, histopathological, and hormonal data from 60 pediatric IC-GCT patients.
- Classification of pubertal dysfunctions into pseudo-precocious puberty (PPP), central precocious puberty (CPP), and hypogonadotrophic hypogonadism (HH).
Main Results:
- Pubertal disorders were identified in 27 patients (45%).
- Hypogonadotrophic hypogonadism (HH) was the most common (20 cases), followed by pseudo-precocious puberty (PPP) (5 cases).
- Central precocious puberty (CPP) developed in 3 patients, and some cases evolved over time.
Conclusions:
- Pubertal dysfunctions are a common complication of pediatric IC-GCTs, particularly those with suprasellar involvement.
- The spectrum of disorders includes PPP, CPP, and HH, with potential for evolution.
- Long-term specialist endocrine follow-up is essential for optimal management.
Introduction:
Pediatric intracranial germ cell tumors (IC-GCTs) are frequently associated with pubertal dysfunctions, either at diagnosis or during follow-up. This study aims to evaluate the prevalence and types of pubertal disorders in a cohort of pediatric patients with IC-GCTs.
Methods:
We collected clinical, radiological, histopathological, and hormonal data. Pubertal dysfunctions were classified as: (1) pseudo-precocious puberty (PPP, gonadotrophin-independent); (2) central precocious puberty (CPP, gonadotrophin-dependent); (3) hypogonadotrophic hypogonadism (HH).
Results:
Sixty patients with IC-GCTs (followed-up between 1996 and 2023 at Royal Marsden Hospital, UK) were included (median follow-up 22.9 months). Germinomas represented 70%, teratomas and mixed GCTs 15% each. Tumors were suprasellar (59%), pineal (30%), or bifocal (11%). Treatments included surgery (11%), chemotherapy (78%), and radiotherapy (90%). Pubertal disorders were found in 27 patients (16 males, 11 females). PPP was found in five patients at diagnosis; in three, it resolved with tumor treatment; one required treatment with bicalutamide/anastrozole and one progressed to CPP. Three patients in total developed CPP (one after PPP, one post-PPP with intervening treatment, and one post-cancer treatment completion). HH was diagnosed in 20 cases (mean age 17.5 years in males, 15.1 in females). Two had HH at diagnosis. One female had amenorrhea with polycystic ovary syndrome and responded to combined oral hormonal therapy. No cases of hypergonadotrophic hypogonadism from chemotherapy were observed.
Conclusion:
Pubertal dysfunctions are common in IC-GCTs, especially with suprasellar involvement. Patterns include PPP, CPP, and HH, which may evolve over time. Long-term specialist endocrine follow-up is essential.
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