Context Germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene are responsible for a subset of familial isolated pituitary adenoma (FIPA) cases and sporadic pituitary neuroendocrine tumors (PitNETs). Objective To compare prospectively diagnosed AIP mutation-positive (AIPmut) PitNET patients with clinically presenting patients and to compare the clinical characteristics of AIPmut and AIPneg PitNET patients. Design 12-year prospective, observational study. Participants & Setting We studied probands and family members of FIPA kindreds and sporadic patients with disease onset ≤18 years or macroadenomas with onset ≤30 years (n = 1477). This was a collaborative study conducted at referral centers for pituitary diseases. Interventions & Outcome AIP testing and clinical screening for pituitary disease. Comparison of characteristics of prospectively diagnosed (n = 22) vs clinically presenting AIPmut PitNET patients (n = 145), and AIPmut (n = 167) vs AIPneg PitNET patients (n = 1310). Results Prospectively diagnosed AIPmut PitNET patients had smaller lesions with less suprasellar extension or cavernous sinus invasion and required fewer treatments with fewer operations and no radiotherapy compared with clinically presenting cases; there were fewer cases with active disease and hypopituitarism at last follow-up. When comparing AIPmut and AIPneg cases, AIPmut patients were more often males, younger, more often had GH excess, pituitary apoplexy, suprasellar extension, and more patients required multimodal therapy, including radiotherapy. AIPmut patients (n = 136) with GH excess were taller than AIPneg counterparts (n = 650). Conclusions Prospectively diagnosed AIPmut patients show better outcomes than clinically presenting cases, demonstrating the benefits of genetic and clinical screening. AIP-related pituitary disease has a wide spectrum ranging from aggressively growing lesions to stable or indolent disease course.

Objective. The aim of this study was to explore the long-term outcomes of surgery for transsphenoidal prolactinomas and the factors that influence them. Material and methods. Transsphenoidal approach for pituitary adenomas has been applied to 329 patients in the Department of Neurosurgery of Kaunas University of Medicine Hospital in the period of 1995 to 2006. Of these, 85 patients were operated for prolactinomas. Results. Of the 85 patients operated on for prolactinomas, 68 (80%) were females and 17 (20%) were males. Thirty-two microprolactinomas and 36 macroprolactinomas were diagnosed in women and 16 and 1, respectively, in men. Twenty (23.5%) patients (16 women and 4 men) had visual field defects before the operation. Dopamine agonist therapy was administered in 50 patients (38 women and 12 men) before the operation. Of 10 women, in whom microadenoma was diagnosed and no dopamine agonist therapy was prescribed, remission was achieved in 9 (90%) patients after the operation, while of 22 women, who was treated with dopamine agonists before the operation, remission was achieved only in 10 (45.5%) (P=0.01). Each year of age decreased the chance of remission by 8%. Conclusions. Remission after the surgical treatment was achieved in 11.8% of men and 47.1% of women hyperprowith prolactinomas. Remission rate was very high (90%) among women with microprolactinoma not treated with dopamine agonist before the surgical treatment. The probability of a good outcome of surgery among women with prolactinoma was related to younger age of the patient, noninvasive tumor growth, plasma prolactin level less than 2309 mU/L, and no use of dopamine agonist before the surgical treatment.

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