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Craniopharyngioma and Posttreatment Pituitary Dysfunction in Brain Tumors

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Book cover Hypothalamic-Pituitary Diseases

Part of the book series: Endocrinology ((ENDOCR))

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Abstract

Central nervous system tumors are a heterogeneous group of malignancies. They are rare diseases but represent a significant cause of mortality, especially in children and young adult patients. Craniopharyngioma, an embryogenic lesion of the sellar area with low-grade histological malignancy, represents approximately 3% of all intracranial tumors. Despite a relatively high rate of tumor recurrence, survival is generally good. Presenting symptoms are related to endocrine problems, mass effect, and cognitive dysfunction. Non-aggressive surgery followed by radiotherapy is currently the most widely used treatment, achieving the best long-term outcome. Other available treatments are intracystic irradiation, intracystic instillation of antineoplasmatic agents, and stereotactic radiotherapy. Complete tumor resection and potential cure should be balanced with a more conservative approach, aiming to avoid treatment-associated long-term morbidity as hypopituitarism and hypothalamic damage (obesity, adipsic or polidipsic diabetes insipidus, sleep disorders, neurocognitive impairment).

In the last decades, thanks to the improvement of anticancer therapies, the rate of patients that can be effectively cured is dramatically improved. The majority of patients with childhood and adolescent non-pituitary central nervous system tumors have a high survival expectancy. In these as in all cancer patients, the treatment choice should take into account the potential adverse effects of anticancer therapies. Any organ system can be damaged by oncological treatments, and endocrine disorders are the most common long-term effects in cancer survivors, with a cumulative incidence of about 50% after a 15-year follow-up from cancer recovery. Endocrine dysfunctions can play a role in the long-term health of these patients but also affect the short-term health, particularly in children. The main responsible for pituitary damage in survivors of central nervous system tumors is radiotherapy that, in combination with neurosurgery and/or chemotherapy, represents the mainstay for treatment of these tumors. Hypothalamic-pituitary dysfunction in survivors of central nervous system tumors is characterized by peculiar features that should be carefully kept in mind by endocrinologists who have to cope with these peculiar patients. The diagnosis, treatment, and clinical management of endocrinopathies in this context show some differences from those applied to patients in the routine endocrine practice. Therefore, the endocrinologist should play a prominent role in the multidisciplinary team that takes care of cancer survivors.

Francesco Felicetti and Nunzia Prencipe contributed equally to this work.

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Felicetti, F., Prencipe, N., Brignardello, E., Arvat, E. (2018). Craniopharyngioma and Posttreatment Pituitary Dysfunction in Brain Tumors. In: Casanueva, F., Ghigo, E. (eds) Hypothalamic-Pituitary Diseases. Endocrinology. Springer, Cham. https://doi.org/10.1007/978-3-319-44444-4_5

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