Association of primary hyperparathyroidism with pituitary adenoma and management issues

Abstract

The co-occurrence of primary hyperparathyroidism (PHPT) and pituitary adenomas (PAs) is often indicative of underlying genetic syndromes such as Multiple Endocrine Neoplasia type 1 (MEN1) and, less commonly, MEN4. Although both conditions can occur sporadically, their simultaneous presence warrants evaluation for genetic mutations, with MEN1 mutations being the most frequent cause. The management of concurrent PHPT and PAs, especially in MEN1 patients, presents unique challenges. Management complexities arise from the syndromic nature, involving both surgical and medical interventions tailored to each condition. PHPT often manifests earlier and more aggressively in MEN1, requiring surgical intervention. However, recurrence rates remain high due to multiglandular involvement. Pituitary adenomas in MEN1 are primarily prolactinomas, and treatment with dopamine agonists results in significant tumour control in most cases. Overall, PAs associated with MEN1 are generally responsive to medical therapy, but careful long-term monitoring is essential. The utility of genetic screening cannot be overstated, as it aids in early detection, risk stratification, and management of both the index case and affected family members by cascade screening. A multidisciplinary approach is crucial for optimizing outcomes, with ongoing surveillance to manage recurrence and associated complications. In summary, the co-occurrence of PHPT and PAs, particularly in the context of MEN1, necessitates an integrated management strategy. Genetic testing is key in confirming diagnosis and guiding treatment, while surgical and medical interventions should be tailored to the extent and nature of glandular involvement. Close monitoring for recurrence and proactive family screening are essential components of long-term care.