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Rationale: Multiple endocrine neoplasia type 1 (MEN1) is a rare tumor syndrome with an autosomal dominant inheritance, and genetic testing for MEN1 gene is important for both affected individuals and their relatives. We present a 2-person family affected by a germline c.1546dupC MEN1 mutation, and one of them had a full-spectrum of MEN-related endocrine tumors., Patient Concerns: A female patient aged 32 years presented with jejunal ulcer perforation due to gastrinoma., Diagnoses: We conducted genetic analysis and extensive biochemical/radiological evaluation for detecting other endocrine tumors. Multiple pancreatic neuroendocrine tumors (NETs), prolactinoma and primary hyperparathyroidism were diagnosed, and a frame-shift mutation, NM_130799.1:c.1546dupC (p.Arg516Profs∗15), was detected. One daughter of the proband, aged 12 years, had the same mutation for MEN1., Intervention: She underwent pancreatic surgery for pancreatic NETs and total parathyroidectomy for primary hyperparathyroidism., Outcomes: After pancreatic surgery, long-term symptoms of epigastric soreness, acid belching, sweating, and palpitation in fasting were improved. Hypercalcemia was improved after parathyroidectomy and she was supplemented with oral calcium and vitamin D. Her daughter showed normal biochemical surveillance until 15 years of age., Lessons: We report 2 people in a family affected by MEN1 with the heterozygous germline c.1546dupC mutation, a variant that should be surveilled for early development of full-blown MEN1-associated endocrine tumors., Competing Interests: Conflict of Interest: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The authors report no conflicts of interest., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objective: The aim of this study was to evaluate cognitive functions in patients with prolactin (PRL)-secreting pituitary adenoma., Methods: We conducted a hospital-based case-control study. The effect of PRL overproduction on cognitive processes was assessed with a comprehensive battery of neuropsychological tests to measure verbal memory, nonverbal memory, attention, visuospatial skills, verbal fluency, and executive functions. The data were gathered from 40 participants matched for age, sex, handedness, and education (20 with pituitary adenoma, 20 healthy controls). The patients were examined on the first day of their hospitalization in the Department of Neurosurgery of Medical University of Warsaw. MRI as well as blood test of pituitary hormone level and perimetry test of the visual field were conducted., Results: The group of patients had significantly lower scores on verbal memory, nonverbal memory, and attention tests compared with healthy volunteers. Their results in memory and visuospatial tests were significantly negatively correlated with the level of PRL but there was no marked relationship between cognitive functioning and the size of tumor., Conclusions: This study demonstrated a significant relationship between PRL overproduction and worsening of cognitive processes, especially in the domains of memory and attention in hospitalized patients with pituitary adenomas. Greater hyperprolactinemia was associated with a larger decrease in cognitive performance. There was no effect of tumor size., (© 2015 American Academy of Neurology.)

This is the first reported case of pineal lymphoma with concomitant prolactin-producing pituitary adenoma.A 51-year-old male experienced worsening headaches accompanied by nausea, diplopia, and memory loss for 1 month. Cranial nerve examination revealed bilateral upward gaze limitation with convergence impairment, which is known as Parinaud syndrome. Magnetic resonance images revealed a mass in the pineal gland with a coexisting mass within the enlarged sella fossa. Hormone analysis revealed hyperprolactinemia. The pineal mass was removed without injuring the hypothalamus, brain stem, or any neighboring vessels. Pathology examination confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL) involving the pineal gland. After further studies, the pineal lymphoma was determined to be a secondary tumor from a gastric primary tumor. The patient died 6 months after diagnosis due to systemic progression of DLBCL.Although the mechanistic link between hyperprolactinemia and lymphoma progression has not been clarified on a clinical basis, high prolactin levels may contribute to the rapid progression and therapeutic resistance of the lymphoma.

Prolactin-secreting adenomas represent nearly 40% to 60% of all pituitary adenomas. Prolactin inhibits the secretion of gonadotropin-releasing hormone that is responsible for the synthesis and secretion of gonadotropins. Sex steroids have an important effect on the regulation of bone metabolism in men. Decreased libido and impotence are the most common presenting symptoms of hyperprolactinemia in males. These symptoms are easily neglected by both patients and some physicians. We present a 22-year-old man with multiple osteoporotic fractures associated with prolactinoma despite the use of teriparatide for 18 months. We emphasize and highlight the importance of hyperprolactinemia and fractures caused by high prolactin levels.

Purpose: Elevated prolactin levels may have several etiologies, one of which is a prolactinoma. Prolactinomas, the most prevalent pituitary tumors, may be more common than once described. These tumors can result in ocular complications such as vision loss and visual field defects. Prolactinomas are easily treated but need to be identified early in order to prevent more serious systemic complications., Case Report: A 24-year-old Pacific Islander male presented with sudden blurred vision and an associated visual field defect in the right eye for 4 to 6 months. After performing a magnetic resonance imaging and consultation with an endocrinologist, the patient was diagnosed with a prolactinoma. The patient was treated and demonstrated a complete recovery of visual acuity and visual field defect., Conclusions: Prolactinomas are a common pituitary tumor characterized by an increase in prolactin levels. This review describes how prolactinomas are classified, their etiology, subsequent complications, and treatments.

Purpose of Review: To review physiology of prolactin (PRL), cause and managment of hyperprolactinemia, and discuss evolving diverse roles of PRL in men's health., Recent Findings: Hyperprolactinemia can be physiologically found after sexual activities, exercise, lactation, during pregnancy, and after stressful venipuncture. Elevated PRL can be caused by medications use, renal failure, hypothyroidism, and by prolactinoma - PRL secreting tumors. Symptomatic hyperprolactinemia and prolactinomas should be treated to lower PRL levels, decrease tumor size, and restore gonadal function. Three modes of treatment are typically utilized: pharmacological, radiosurgery with gamma radiation, and external beam radiation. Pharmacological treatment of prolactinomas is mainly based on dopamine agonists. The most frequently used dopamine agonists are bromocriptine and cabergoline. Cabergoline becoming the preferred drug in the treatment of prolactinomas because of higher response rate and less side-effects. Bromocriptine has been recently approved to improve glycemic control in diabetes mellitus., Summary: PRL plays a diverse role in men's reproduction and health. Detecting and treating elevated PRL may not only improve infertility and hypogonadism but also have a positive effect on the metabolic profile of patient and control of glycemic control and metabolic profile - an important advantage considering dramatic and worldwide increase in obesity and diabetes.

Objective: Impulse control disorders (ICDs) such as pathological gambling, compulsive shopping, compulsive eating, and hypersexuality are a matter of growing interest, especially in patients with Parkinson disease who are on dopamine replacement therapy. It was recently reported that ICDs are associated with other disorders also treated with dopaminergic drugs (dopamine agonists) such as restless legs syndrome, multiple system atrophy, progressive supranuclear palsy, and fibromyalgia. The aim of this study was to determine the prevalence of ICDs in patients with pituitary adenomas who take dopamine agonists (DAs)., Methods: Twenty consecutive patients with pituitary adenomas (mostly prolactinomas) taking DAs were assessed. All participated in a structured interview focused on ICDs, which was conducted by a physician., Results: Two (10%) of 20 subjects had a condition diagnosed as ICD. The first patient is a 35-year-old man with giant macroprolactinoma who was alternately treated with different types of DAs (cabergoline, bromocriptine, and quinagolide). He developed compulsive eating and pathological gambling. The second patient is a 53-year-old man with macroprolactinoma who suffered from severe hypersexuality after cabergoline was begun., Conclusions: This study demonstrates the importance of systematic screening for ICDs in patients taking dopaminergic medication regardless of their primary condition.

We report a rare case of prolactinoma with deposition of amyloid and calcifications. Magnetic resonance imaging and/or computed tomography findings have been described in only a few cases. Possible mechanisms of amyloid deposition and calcification were discussed, and literatures were reviewed. In pituitary adenomas, particularly in prolactinomas, amyloid deposits should be included in the differential diagnosis, when hypointense areas are noted on T2-weighted images. Stippled calcifications in the tumor seem to be another characteristic finding.

A patient who had achieved marked improvement in vision and shrinkage of a prolactinoma following treatment with pergolide (0.1 mg/day) suffered a marked worsening of vision 7 months after continued treatment at the same dose. Brain magnetic resonance imaging (MRI) at the time of visual loss showed further shrinkage of the tumor and prolapse of the chiasm into the pituitary fossa. The dose of pergolide was cut in half (0.05 mg/day); 12 months later, vision had completely recovered. Brain MRI at the time of visual recovery showed no change in the position of the prolapsed chiasm. This is the 11th reported case of delayed visual loss following dopaminergic treatment of prolactinoma. Recovery of vision always occurs with reduction of the medication dosage. Many patients whose prolactinomas are treated in this fashion display chiasmal prolapse, and few suffer visual loss. Considering that visual recovery occurs without a visible change in the position of the chiasm, traction is an unlikely cause of delayed visual loss. Therefore, the term chiasmal traction syndrome, used to describe visual loss with prolapsed chiasm following surgical and radiation treatment of sellar tumors, should not be applied in this setting lest it prompt consideration of surgical chiasmapexy. The proper management is reduction of the dopaminergic agonist dosage.

Pituitary apoplexy is a severe and potentially life-threatening condition that may be highly variable in its clinical presentation. We report a 37-year-old man presenting to the emergency department with diplopia that abruptly developed while he was eating canned and bottled food prepared at home. A computed tomography scanning revealed an isodense mass within the sellar region and, subsequently, a magnetic resonance imaging showed a pituitary apoplexy causing a compression of the right III and VI oculomotor nerves. There was no improvement with hydrocortisone therapy and the patient underwent a transsphenoidal excision of the mass with an uneventful course. Pituitary apoplexy may raise in the appropriate setting the suspicion of botulism. The abrupt-onset paralysis of oculomotor nerves has been described as the chief presenting sign of pituitary apoplexy in only few cases including this. A pathophysiology, differential diagnosis with botulism and other causes of multiple cranial nerve paralysis, and treatment are described.

Background and Purpose: We report the first case of pituitary apoplexy caused by the rupture of an intracavernous carotid artery aneurysm embedded in a pituitary adenoma., Case Description: A 46-year-old man presented with clinical and CT findings typical of pituitary apoplexy. MRI showed an unusual flow-void protrusion into the intratumoral hematoma, which, however, was not diagnosed as a ruptured aneurysm until severe intraoperative bleeding occurred. Angiography after surgery revealed an intracavernous carotid artery aneurysm., Conclusions: The possible association of adenoma and aneurysmal rupture should be kept in mind when assessing any case of pituitary apoplexy.

Objective: The case of a psychotic woman is described in which risperidone use was found to correspond with an increase in the size of a prolactinoma and prevented the return of serum prolactin level to baseline., Methods: Although the patient had been treated with a high dose of bromocriptine, her prolactin level remained elevated, causing persistent galactorrhea. The patient later was treated with olanzapine and carbamazepine successfully., Results: This case report highlights the role of risperidone on prolactin and discusses alternative methods of treating psychosis when the etiology is unclear, especially in younger patients., Conclusions: The authors recommend that additional studies regarding the relationship between the growth of prolactinoma and atypical antipsychotics would be worthwhile.

We reviewed the clinical features, essential laboratory data, pituitary imaging findings (computerized tomography and magnetic resonance imaging), management, and outcome of 353 consecutive patients with the presumptive diagnosis of pituitary tumor investigated from January 1984 through December 1997 at University Hospital, Lausanne, Switzerland. In 18 cases primary empty sella turcica was diagnosed, and in 13 cases of pseudacromegaly there were no endocrine abnormalities. The remaining 322 patients disclosed abnormal pituitary masses, including 275 pituitary adenomas, 18 craniopharyngiomas, 6 cases of primary pituitary hyperplasia, 6 intrasellar meningiomas, 6 cases of distant metastases, 4 intrasellar cysts, 2 chordomas, 1 primary lymphoma, and 1 astrocytoma. Biologic data and immunohistochemical analysis of the excised tissues demonstrated that prolactinomas and nonsecreting adenomas (NSAs) were the most frequent pituitary tumors (40% and 39%, respectively), followed by somatotropic adenomas with acromegaly (11%) and Cushing disease (6%). In contrast with the vast majority of NSAs, which significantly expressed glycoprotein hormones in tissue without secreting them, there was a small group of glycoprotein hormone-secreting adenomas (2%), which had a more severe clinical course after surgery. Thirty-eight pituitary masses were incidentally discovered, most of them NSAs. The expansion of pituitary adenomas into the right cavernous sinus was twice as frequent as to the left cavernous sinus. For the differential diagnosis of hyperprolactinemia, basal prolactin (PRL) levels above 85 micrograms/L, in the absence of renal failure and PRL-enhancing drugs, and a PRL increment of less than 30% after thyrotropin-releasing hormone (TRH) accurately ruled out functional hyperprolactinemia due to NSA, and were typical of prolactinomas. For screening and follow-up of acromegaly, basal growth hormone (GH) and insulin-like growth factor 1 (IGF-1) levels, as well as the paradoxical GH response to TRH (present in 2/3 acromegalic patients), could be used as convenient tools, but the most accurate test for diagnosis and prediction of outcome after therapy was GH (lack of) suppression during oral glucose tolerance test. In Cushing disease, single evening plasma cortisol was as good as the overnight dexamethasone suppression test for screening, and a combined dexamethasoneovine corticotropin-releasing hormone (oCRH) test was as accurate as the long dexamethasone suppression test to confirm the diagnosis. Bilateral inferior petrosal sinus catheterization coupled with oCRH test confirmed the pituitary origin of excess adrenocorticotropic hormone (ACTH) in all patients, including those with normal pituitary on magnetic resonance imaging (50% of the cases). However, this procedure failed to predict tumor localization correctly within the pituitary in 21% of patients. Pituitary cysts, meningiomas, and craniopharyngiomas with an intrasellar component were correctly diagnosed based on pituitary imaging in 75%, 67%, and 44% of cases, respectively. The remainder, as well as the cases of pituitary hyperplasia, metastases, and other less frequent pathologies, were initially diagnosed as NSAs or as masses of unknown nature. When surgery was indicated, pituitary adenomas and other intrasellar masses were operated on by the transsphenoidal route, with the exception of 100% of meningiomas, 83% of craniopharyngiomas, and 10% of NSAs, which were operated on by the transcranial route. Favorable late surgical outcome of prolactinomas could be predicted by a restored PRL response to TRH. However, dopamine agonist (DA) therapy, usually resulting in satisfactory control of PRL levels and in tumor shrinkage, progressively displaced surgery as primary treatment for prolactinomas throughout the study period. After full-term pregnancy, the size of prolactinoma decreased in 7 of 9 patients, and PRL was normal in 2. Surgery was the first treatment for NSAs, with a tumor rela

Rationale and Objectives: The authors investigated whether hormonally active and inactive pituitary adenomas can be discriminated in vitro by magnetic resonance (MR) imaging-related data., Methods: 1/T1 nuclear magnetic relaxation dispersion profiles were measured for 39 fresh surgical specimens of secreting and nonsecreting adenomas, classified using clinical criteria or preoperative serum hormone levels. Nonsecreting adenomas were subdivided into hormone-producing and nonhormone-producing by immunostains. At five fields (0.00024 to 1.2 tesla [T]), mean 1/T1 was analyzed for statistically significant differences among these three tumor categories., Results: Mean 1/T1 was significantly higher (P < 0.02) for hormone-secreting than for nonsecreting adenomas at fields below 0.24 T; no significant difference existed at typical MR imaging fields (0.5 to 1.5 T). Mean 1/T1 for hormone-producing and nonhormone-producing, nonsecreting adenomas were not significantly different at any field., Conclusions: Because 1/T1 at low fields is related to 1/T2 at imaging fields, it may be possible to detect hormone secretion of pituitary adenomas noninvasively by MR imaging.

This review focuses on recent developments in the treatment of pituitary adenomas. Current treatments include both transcranial and transsphenoidal surgery, the medical treatment specifically for endocrinopathies, and finally, radiation treatments including conventional radiation as well as stereotactic radiosurgery. All these modalities are reviewed.

Objective: We present two patients with ectopic prolactinoma in the sphenoid sinus who were examined by MRI., Materials and Methods: Endocrinological evaluations including spontaneous and drug-induced secretion of prolactin (PRL) and MRI features of two patients with ectopic prolactinoma were studied., Results: Both patients had elevated serum PRL levels, and the endocrinological evaluations were similar to those in ectopic prolactinoma. Magnetic resonance of the head revealed a mass occupying the sphenoid sinus with partially empty sella in Case 1 and with normal pituitary gland in Case 2. Ectopic PRL secretion was confirmed by immunocytochemical examination of the ectopic tumor. Case 1 was treated with bromocriptine, leading to marked decrease in serum PRL levels and reduction of tumor size. Transsphenoidal surgery was performed in Case 2, resulting in normalization of serum PRL levels., Conclusion: It is necessary to consider ectopic prolactinoma for the differential diagnosis of the sphenoid sinus tumors.

We report a case in a 6-year-old boy of orbital invasion by prolactinoma in which the cavernous sinus was involved with direct spread into the superior ophthalmic vein, as seen with CT and MRI.

A phantom study was performed to assess the accuracy of MRI in volume determination of pituitary macroadenomas, using the summation of areas technique. Five phantoms, 3-4 cm in diameter, simulating pituitary macroadenomas of various shapes, were evaluated. The volume could adequately and reproducibly be determined, regardless of minor rotational variations of the phantom position. Using a three-dimensional SE sequence with 2.5 mm slices, the error was independent of the shape of the phantom and did not differ significantly from zero (less than 5%); this sequence was found to be the best of those tested. The coefficient of variation between examinations with different tilts was 2%. In a clinical part, the volume of pituitary macroadenomas was determined with the same technique on coronal two-dimensional 5 mm images and was used as a "gold standard" to which the largest transverse, sagittal, vertical, and oblique diameters, a central tumor area, and the product of the three orthogonal diameters were correlated. In interpatient comparisons the largest diameter was found to be useful, but only as a rough measure of the tumor size. The formula 0.5 x width x length x height provided a fairly adequate estimation of the tumor volume, except in the largest tumors. In intrapatient comparisons during bromocriptine treatment, the height was found to be the best of the diameters, but the central tumor area or the product of the orthogonal diameters was preferable.

The main advances in the diagnostic evaluation of pituitary tumors and prolactinomas have been in the areas of improved magnetic resonance techniques and in the use of inferior petrosal sinus sampling. New dynamic techniques of rapid acquisition magnetic resonance imaging during bolus contrast infusion have improved the sensitivity for the diagnosis of the small microadenoma. The development of three-dimensional volume imaging has also led to a further improvement in sensitivity to small lesions of the sella. The measurement of adrenocorticotropin levels in the inferior petrosal sinus in patients with Cushing's syndrome assists in the differentiation of adrenocorticotropin-secreting pituitary tumor from other peripheral causes of the syndrome. The use of corticotropin-releasing hormone concomitant with sampling has proven to be of value in improving sensitivity and specificity. Elevated levels of growth hormone in petrosal sinus sampling have also been shown to be valuable in the early diagnosis of acromegaly when peripheral hormone levels and imaging are nondiagnostic.