Synonyms
FIPA syndrome
Definition
An inherited familial endocrine tumor syndrome characterized by the development of a pituitary adenoma, currently referred as “pituitary neuroendocrine tumor” in two or more members of the same family without clinical or genetic evidence of another hereditary endocrine tumor syndrome. When members of the same family are affected by the same tumor subtype, the disease is said to be “homogeneous.” In contrast, when tumors of different subtypes affect members in the same family, the disease is called “heterogeneous.”
Clinical Features
Incidence
The estimated prevalence of pituitary neuroendocrine tumors is approximately 17% (based on autopsy and radiological studies). Overall, pituitary neuroendocrine tumors arising in the context of familial isolated pituitary adenoma/pituitary neuroendocrine tumor syndrome probably represent approximately 2–5% of the overall patient population with pituitary neuroendocrine tumors.
Age
Patients affected by familial...
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References and Further Reading
Asa, S. L., & Mete, O. (2018). Immunohistochemical biomarkers in pituitary pathology. Endocrine Pathology, 29, 130–136.
Beckers, A., Aaltonen, L. A., Daly, A. F., & Karhu, A. (2013). Familial isolated pituitary adenomas (FIPA) and the pituitary adenoma predisposition due to mutations in the aryl hydrocarbon receptor interacting protein (AIP) gene. Endocrine Reviews., 34, 239–277.
Caimari, F., & Korbonits, M. (2016). Novel genetic causes of pituitary adenomas. Clinical Cancer Research, 22, 5030–5042.
Daly, A. F., Jaffrain-Rea, M. L., Ciccarelli, A., Valdes-Socin, H., Rohmer, V., Tamburrano, G., Borson-Chazot, C., Estour, B., Ciccarelli, E., Brue, T., Ferolla, P., Emy, P., Colao, A., De Menis, E., Lecomte, P., Penfornis, F., Delemer, B., Bertherat, J., Wémeau, J. L., De Herder, W., Archambeaud, F., Stevenaert, A., Calender, A., Murat, A., Cavagnini, F., & Beckers, A. (2006). Clinical characterization of familial isolated pituitary adenomas. The Journal of Clinical Endocrinology and Metabolism, 91, 3316–3323.
Daly, A. F., Tichomirowa, M. A., & Beckers, A. (2009). Genetic, molecular and clinical features of familial isolated pituitary adenomas. Hormone Research, 71(Suppl 2), 116–122.
Korbonits, M., Storr, H., & Kumar, A. V. (2012). Familial pituitary adenomas – Who should be tested for AIP mutations? Clinical Endocrinology, 77, 351–356.
Ozfirat, Z., & Korbonits, M. (2010). AIP gene and familial isolated pituitary adenomas. Molecular and Cellular Endocrinology, 326, 71–79.
Pepe, S., Korbonits, M., & Iacovazzo, D. (2019). Germline and mosaic mutations causing pituitary tumours: Genetic and molecular aspects. The Journal of Endocrinology, 240, R21–R45.
Villa, C., Lagonigro, M. S., Magri, F., Koziak, M., Jaffrain-Rea, M. L., Brauner, R., Bouligand, J., Junier, M. P., Di Rocco, F., Sainte-Rose, C., Beckers, A., Roux, F. X., Daly, A. F., & Chiovato, L. (2011). Hyperplasia-adenoma sequence in pituitary tumorigenesis related to aryl hydrocarbon receptor interacting protein gene mutation. Endocrine-Related Cancer, 18, 347–356.
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Hodgson, A., Pakbaz, S., Mete, O. (2022). Familial Isolated Pituitary Adenoma (FIPA) Syndrome. In: La Rosa, S., Uccella, S. (eds) Endocrine Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-62345-6_5221
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DOI: https://doi.org/10.1007/978-3-030-62345-6_5221
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