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Challenging Questions in the Management of Acromegaly in the Young

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Acromegaly

Part of the book series: Contemporary Endocrinology ((COE))

Abstract

Aryl hydrocarbon receptor-interacting protein (AIP) positive mutation patients are associated with an aggressive, inheritable form of acromegaly that responds poorly to first-generation somatostatin receptor ligands. We present a young female with AIP mutation-positive acromegaly with invasive pituitary macroadenoma that recurred after multiple transsphenoidal surgeries and failed to achieve biochemical remission with lanreotide therapy. Her disease was only controlled after three transsphenoidal surgeries, radiotherapy, and medical therapy that was switched over to pegvisomant. Following disease remission, she developed panhypopituitarism and worsening osteoporosis in her lumbar spine. Concurrently, she also desired pregnancy. Pegvisomant therapy was stopped after 3 years due to the gradual decline in IGF-I levels consistent with the development of post-acromegaly GH deficiency. Our case highlights the importance of careful and thoughtful evaluation of young AIP mutation-positive acromegaly patients by an expert multidisciplinary team so that a personalized treatment regimen can be implemented to achieve early disease remission and minimize tumor regrowth.

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  1. Department of Neuroendocrinology and Neurosurgery, Barrow Pituitary Center, Barrow Neurological Institute, University of Arizona College of Medicine and Creighton School of Medicine, Phoenix, AZ, USA

    Nancy Youssef & Kevin C. J. Yuen

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  1. Nancy Youssef
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  2. Kevin C. J. Yuen
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Correspondence to Kevin C. J. Yuen .

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  1. Department of Neurological Surgery, University of San Francisco, San Francisco, CA, USA

    Lewis S. Blevins Jr.

  2. Department of Neurological Surgery, University of San Francisco, San Francisco, CA, USA

    Manish K. Aghi

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Youssef, N., Yuen, K.C.J. (2022). Challenging Questions in the Management of Acromegaly in the Young. In: Blevins Jr., L.S., Aghi, M.K. (eds) Acromegaly. Contemporary Endocrinology. Springer, Cham. https://doi.org/10.1007/978-3-031-16258-9_15

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