Intracranial germ cell tumors: a view of the endocrinologist

J Pediatr Endocrinol Metab. 2023 Oct 30;36(12):1115-1127. doi: 10.1515/jpem-2023-0368. Print 2023 Dec 15.

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.

Keywords: endocrinologist; intracranial germ cell tumors; neuroendocrine function; pediatric; precocious puberty.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Brain Neoplasms* / diagnosis
  • Brain Neoplasms* / pathology
  • Brain Neoplasms* / therapy
  • Child
  • Diabetes Insipidus* / complications
  • Endocrinologists
  • Humans
  • Neoplasm Recurrence, Local
  • Neoplasms, Germ Cell and Embryonal* / complications
  • Neoplasms, Germ Cell and Embryonal* / diagnosis
  • Neoplasms, Germ Cell and Embryonal* / therapy