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Intracranial germ cell tumors: a view of the endocrinologist
1Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, Beijing, P.R. China.
Abstract:
Intracranial germ cell tumors (iGCTs) are rare malignant neoplasms that mainly affect children and adolescents. The incidence, clinical presentation, and prognosis of iGCTs exhibit high heterogeneity. Previous studies have primarily focused on eliminating tumors, reducing tumor recurrence, and improving survival rates, while neglecting the impact of the tumors and their treatment on neuroendocrine function. Throughout the entire course of the disease, neuroendocrine dysfunction may occur and is frequently overlooked by oncologists, neurosurgeons, and radiologists. Endocrinologists, however, are more interested in this issue and have varying priorities at different stages of the disease. From onset to the diagnostic phase, most patients with iGCTs may present with symptoms related to impaired neuroendocrine function, or even experience these symptoms as their first indication of the condition. Particularly, a minority of patients with sellar/suprasellar lesions may exhibit typical imaging features and elevated tumor markers long after the onset of initial symptoms. This can further complicate the diagnosis process. During the peritumor treatment phase, the neuroendocrine function shows dynamic changes and needs to be evaluated dynamically. Once diabetes insipidus and dysfunction of the hypothalamic-pituitary-adrenal and hypothalamic-pituitary-thyroid axes occur, hormone replacement therapy should be administered promptly to ensure successful tumor treatment for the patient. Subsequently, during the long-term management phase after the completion of tumor treatment, the evaluation of growth and development as well as corresponding hormone replacement therapy are the most concerning and complex issues. Thus, this paper reviews the interest of endocrinologists in iGCTs at different stages.
Insights
Intracranial germ cell tumors (iGCTs) impact neuroendocrine function, often overlooked by non-endocrinologists. This review highlights the critical role of endocrinologists throughout iGCT diagnosis and treatment.
Area of Science:
- Pediatric Oncology
- Neuroendocrinology
- Oncology
Background:
- Intracranial germ cell tumors (iGCTs) are rare, heterogeneous pediatric neoplasms.
- Focus has been on tumor elimination, often neglecting neuroendocrine impact.
- Neuroendocrine dysfunction is frequently overlooked by non-endocrinologists.
Purpose of the Study:
- To review the evolving interest of endocrinologists in iGCTs across disease stages.
- To emphasize the importance of neuroendocrine evaluation and management in iGCT patients.
- To highlight the diagnostic and therapeutic challenges posed by iGCTs.
Main Methods:
- Review of existing literature on iGCTs and neuroendocrine dysfunction.
- Analysis of the role of endocrinologists at different disease phases.
- Discussion of diagnostic and management strategies for neuroendocrine complications.
Main Results:
- Neuroendocrine dysfunction can be an initial symptom of iGCTs, complicating diagnosis.
- Dynamic neuroendocrine evaluation is crucial during peritumor treatment.
- Prompt hormone replacement is essential for successful iGCT treatment and long-term management.
Conclusions:
- Endocrinologists play a vital role in managing iGCTs from diagnosis through long-term survivorship.
- Addressing neuroendocrine sequelae is critical for improving patient outcomes.
- Multidisciplinary collaboration is essential for comprehensive iGCT care.
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