The Endo-ERN will help to share information on diagnosis, treatment and chronic care of patient with a pituitary condition throughout life. We envision that the Endo-ERN will facilitate the knowledge and expertise exchange on these (ultra)rare diseases that require highly specialized interventions. The Endo-ERN will provide best practice documents and patient information for specific diseases. Future registries will improve knowledge on the phenotype/genotype relationship and on the long term outcome. Collaboration will be sought for multicenter trials.
Pituitary adenoma are rare benign tumors, from the pituitary gland. There are functioning tumors, resulting in hormone excess syndromes as prolactinoma, Cushings’disease and acromegaly, non-functioning pituitary adenoma or cystic lesions. Other tumors in the pituitary-hypothalamic region are for example craniopharyngeoma and meningioma. Pituitary function and visual function may be compromised, especially in larger tumors. Multidisciplinary management is needed to provide the optimal treatment and the team will include endocrinologists, neurosurgeons, ophthalmologist, radiation oncologists etc. Dedicated and centralized pituitary neurosurgical expertise is important for the best surgical results as well as clinical experience with radiotherapy and medical management including investigational drugs.
Congenital hypopituitarism results from insults in the developmental process of the pituitary and can result in the loss or reduction of the pituitary hormone-secreting cells. Phenotypes range from multiple pituitary hormone deficiencies to deficiencies in single hormones only, the most common isolated hormone deficiency being attributed to growth hormone. The condition can be life threatening or life-limiting, and needs careful and expert management. Given its midline location, and that the pituitary gland is derived from the same region of the neural plate as the hypothalamus, optic craniofacial/midline disorders affecting these structures too.
Aquired Hypopituitarism usually results from treatment of other pituitary or parasellar conditions, however, there are systemic diseases (sarcoidosis, histiocytosis), traumatic or vascular events and new drugs that result in specific ultrarare diseases.