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Growth hormone (GH) activity might be implicated in male reproductive function. One previous study has suggested significantly reduced semen quality in untreated acromegalic patients due to both reduced sperm counts and sperm motility. A retrospective study comprising ten uncontrolled hypogonadal acromegalic patients (median age 29 years) who delivered semen for cryopreservation before initiation of testosterone therapy. Semen variables and hormone concentrations were compared to those of ten non-acromegalic hypogonadal men with pituitary disease (age 31 years) and those of young healthy men. Acromegalic patients vs. non-acromegalic patients had a higher percentage of progressive motile spermatozoa (62 vs. 47%, p = 0.04). Eight of ten acromegalic patients and 82% of controls had total sperm counts above 39 million and progressive motile spermatozoa above 32% (p = 0.55), corresponding to the WHO 2010 reference levels for expected normal fertility for these variables. Non-acromegalic patients vs. healthy controls had reduced percentage of progressive motile spermatozoa (47 vs. 57%, p = 0.02) and only five of ten patients had semen quality above the WHO reference level, which was significantly lower than observed in healthy controls (p = 0.022). Total sperm counts were similar between both patient groups and controls. There were no differences in reproductive hormone levels between acromegalic patients vs. non-acromegalic patients (p-values between 0.10 and 0.61). Compared to healthy controls both patient groups had severely reduced serum testosterone, calculated free testosterone. Despite severe hypoandrogenism acromegalic patients had semen quality similar to healthy controls based on determination of the number of progressively motile spermatozoa. By contrast non-acromegalic pituitary patients had reduced sperm motility. Our data do not support reduced semen quality in acromegaly.

Pituitary gigantism is a condition caused by excessive secretion of Growth Hormone (GH). The GH is the most commonpituitary hormone-deficient in pituitary disease. Chordoma is a bone primary tumor that grows slowly and is rarely found.Hypothyroidism is a pathological condition due to thyroid hormone deficiency. Symptoms of hypogonadism arenon-specific including libido disorders, erectile dysfunction, and decreased muscle mass and no hair growth in the head orbody. A 24-year-old male came with pain in the knee. Physical examination showed increased growth of natural and bodyparts as well as the loss of body hair. Laboratory investigations revealed pancytopenia, increased prolactin; decreased GH,Insulin-Like Growth Factor-1 (IGF-1) and testosterone; increased Thyroid-Stimulating Hormone (TSH), decreased FreeTriiodothyronine (FT3) and Free Thyroxine (FT4). Ahead MRI demonstrated the presence of a mass in the clivus. In this case,the patient presented with clinical gigantism. However, laboratory examination showed decreased GH and IGF-1 whichmight be due to the suppressive effect of mass on the clivus bone to the pituitary. Further examinations were needed to clearthe suspicion of hypothyroid. Hypogonadism can result from suppression in the pituitary. Pancytopenia can be caused by adeficiency of GH or from hypothyroidism. Gigantism may occur with GH and IGF-1 deficiency due to suppressed pituitarycaused by chordoma.

The pituitary is an endocrine gland with ability to uptake diverse radiopharmaceuticals and, therefore, susceptible to be investigated by nuclear medicine diagnostic procedures. Although this topic has been scarcely scrutinized, we have data indicating that somatostatin receptor scintigraphy with111In-DTPA-D-Phe-octreotide or 99mTc-EDDA/HYNIC-TOC may be of clinical utility in the diagnosis of some pituitary adenomas (PA). Only a few studies have evaluated the diagnostic performance of 99mTc-MIBI and 99mTc (V)-DMSA scintigraphy in pituitary disease. Scintigraphy using 123I-methoxybenzamide (123I-IBZM) might be useful in macroprolactinomas expressing dopamine D2 receptors. Pituitary gland does not usually accumulate 2-deoxy-2-[18F]fluoro-d-glucose (18F-FDG) and, therefore, it is not visualized on positron emission tomography (PET) imaging studies with this radiotracer. The pituitary uptake on18F-FDG PET/CT scans performed in the follow-up of oncological patients are uncommon. However, 60% of these incidental findings are due to PA, mainly non-functioning pituitary macroadenomas, and a small percentage to metastases or other pituitary lesions. Interestingly, 18F-FDG PET/CT may identify hypophysitis induced by different immunotherapeutic agents used in cancer patients. Positive 18F-FDG uptake has been reported in a high percentage of patients with PA, mainly macroadenomas and it seems that there is correlation between tumor size and SUVmax. 68Ga-DOTA-TATE PET/CT may identify functioning and non-functioning PA, although this technique is more useful in the detection of remaining normal pituitary tissue after transsphenoidal adenomectomy, and in the confirmation of recurrence of functioning PA, such as thyrotroph-secreting PA. Furthermore, 68Ga-DOTA-TATE uptake has potential therapeutic implications on molecular-targeted therapy. Lastly, other radiopharmaceuticals that have shown to be taken up in some patients with pituitary disease include 18F-DOPA (prolactinoma), 11C-methionine (residual or recurrent PA), O-(2-18F-fluoroethyl)-l-tyrosine (metastasis), 18F-choline (silent adenoma, ectopic corticotropinoma), and 13N-ammonia (hypopituitarism).

The pituitary is involved in the regulation of endocrine homeostasis. Therefore, animal models of pituitary disease based on a thorough knowledge of pituitary anatomy are of great importance. Accordingly, we aimed to perform a qualitative and quantitative description of polypeptide hormone secreting cellular components of the Göttingen minipig adenohypophysis using immunohistochemistry and stereology. Estimates of the total number of cells immune-stained for adrenocorticotrophic hormone (ACTH), prolactin (PRL), and growth hormone (GH) were obtained with the optical fractionator technique using Stereo Investigator software. Moreover, 3D reconstructions of cell distribution were made. We estimated that the normal minipig adenohypophysis contains, on average, 5.6 million GH, 3.5 million PRL, and 2.4 million ACTH producing cells. The ACTH producing cells were widely distributed, while the PRL and GH producing cells were located in clusters in the central and lateral regions of the adenohypophysis. The morphology of the hormone producing cells also differs. We visualized a clear difference in the numerical density of hormone producing cells throughout the adenohypophysis. The relative proportions of the cells analyzed in our experiment are comparable to those observed in humans, primates, and rodents; however, the distribution of cells differs among species. The distribution of GH cells in the minipig is similar to that in humans, while the PRL and ACTH cell distributions differ. The volume of the pituitary is slightly smaller than that of humans. These data provide a framework for future large animal experimentation on pituitary function in health and disease.

Hypophysitis is defined as inflammation of the pituitary gland that is primary or secondary to a local or systemic process. Differential diagnosis is broad (including primary tumors, metastases, and lympho-proliferative diseases) and multifaceted. Patients with hypophysitis typically present with headaches, some degree of anterior and/or posterior pituitary dysfunction, and enlargement of pituitary gland and/or stalk, as determined by imaging. Most hypophysitis causes are autoimmune, but other etiologies include inflammation secondary to sellar tumors or cysts, systemic diseases, and infection or drug-induced causes. Novel pathologies such as immunoglobulin G4-related hypophysitis, immunotherapy-induced hypophysitis, and paraneoplastic pituitary-directed autoimmunity are also included in a growing spectrum of this rare pituitary disease. Typical magnetic resonance imaging reveals stalk thickening and homogenous enlargement of the pituitary gland; however, imaging is not always specific. Diagnosis can be challenging, and ultimately, only a pituitary biopsy can confirm hypophysitis type and rule out other etiologies. A presumptive diagnosis can be made often without biopsy. Detailed history and clinical examination are essential, notably for signs of underlying etiology with systemic manifestations. Hormone replacement and, in selected cases, careful observation is advised with imaging follow-up. High-dose glucocorticoids are initiated mainly to help reduce mass effect. A response may be observed in all auto-immune etiologies, as well as in lymphoproliferative diseases, and, as such, should not be used for differential diagnosis. Surgery may be necessary in some cases to relieve mass effect and allow a definite diagnosis. Immunosuppressive therapy and radiation are sometimes also necessary in resistant cases.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the viral strain that has caused the coronavirus disease 2019 (COVID-19) pandemic, has presented healthcare systems around the world with an unprecedented challenge. In locations with significant rates of viral transmission, social distancing measures and enforced ‘lockdowns’ are the new ‘norm’ as governments try to prevent healthcare services from being overwhelmed. However, with these measures have come important challenges for the delivery of existing services for other diseases and conditions. The clinical care of patients with pituitary disorders typically involves a multidisciplinary team, working in concert to deliver timely, often complex, disease investigation and management, including pituitary surgery. COVID-19 has brought about major disruption to such services, limiting access to care and opportunities for testing (both laboratory and radiological), and dramatically reducing the ability to safely undertake transsphenoidal surgery. In the absence of clinical trials to guide management of patients with pituitary disease during the COVID-19 pandemic, herein the Professional Education Committee of the Pituitary Society proposes guidance for continued safe management and care of this population.

Context IGF-I is important for postnatal growth and may be of diagnostic value in infants suspected of pituitary disease; however, little is known about the impact of IGF-I and its determinants on infant growth. Importantly, detailed reference ranges for IGF-I and IGF binding protein-3 (IGFBP-3) concentrations during infancy are lacking. Objective To evaluate the rapid changes in weight and length as well as their determinants in healthy infants, and to establish age- and sex-specific reference curves for IGF-I and IGFBP-3 in children aged 0 to 1 years. Design Prospective longitudinal study. Setting Cohort study. Participants A total of 233 healthy children (114 girls) with repeated blood samples during the first year of life. Main Outcome Measure(s) Serum concentrations of IGF-I and IGFBP-3, length velocity, weight velocity, and PAPPA2 (rs1325598) genotype. Results Individual trajectories of length and weight velocities were sex specific. We provide detailed reference curves based on longitudinal data for IGF-I and IGFBP-3 during infancy. In both girls and boys, IGF-I decreased during infancy, whereas IGFBP-3 remained stable. IGF-I and IGFBP-3, but not PAPPA2 genotype, were positively associated with weight gain, but not with longitudinal growth. When stratified by sex, the association between weight gain and IGF-I only remained significant in girls. Conclusions Interestingly, we found a significant association between IGF-I and infant weight gain in girls, but not with longitudinal growth in the first year of life. Our findings highlight the role of IGF-I as an important anabolic hormone that is not limited to linear growth.

The pituitary gland embodies our endocrine hub and rigorously regulates hormone balances in the body, thereby ruling over vital developmental and physiological processes. Pituitary dysfunction and disease strongly impact the organism's biology. Physical damage, tumour development and ageing all negatively affect pituitary state and functionality. On top of its hormone-producing cells, the pituitary contains a population of stem cells. Not only their physiological role is still largely unknown, also whether or how these stem cells are involved in pituitary disease and recovery from defective functionality remains enigmatic. Here, we summarize what is known on the phenotypical and functional behaviour of pituitary stem cells in diseased or dysfunctional gland, as particularly caused by injury, tumourigenesis and ageing. ispartof: MOLECULAR AND CELLULAR ENDOCRINOLOGY vol:525 ispartof: location:Ireland status: published

Objective: Abnormalities of water and sodium balance, including diabetes insipidus and the syndrome of inappropriate antidiuretic hormone (SIADH), are common complications of transsphenoidal surgery. Postoperative practice patterns vary among clinicians, and no consensus guidelines exist to direct their monitoring and management. We aimed to identify and compare practice patterns regarding the evaluation and management to these postoperative complications. Methods: A questionnaire was utilized to capture demographic data and practice habits in the postoperative setting. Respondents were members of the Pituitary Society, an international organization comprised of clinicians and researchers dedicated to the study of pituitary disease. Results: Eighty-six respondents completed at least part of the survey. The geographic distribution of respondents was roughly even between American and non-American practitioners. The majority of respondents practiced at academic institutions (67.4%), worked in multidisciplinary teams (88.4%), and possessed significantly greater than 10 years of clinical experience (71%). Compared to non-American practitioners, American practitioners described a shorter length of stay postoperatively (P

Objective: The inflammation of the pituitary gland is known as hypophysitis. It is a rare disease accounting for approximately 0.24%-0.88% of all pituitary diseases. The natural course of hypophysitis is variable. Main forms are histologically classified as lymphocytic, granulomatous, IgG4 related and xanthomatous. We aim to present our patients with hypophysitis and compare clinical, laboratory and radiological features. Subjects and methods: We retrospectively reviewed our database of 1.293 patients diagnosed with pituitary diseases between 2010 and 2017. Twelve patients with hypophysitis were identified. Demographical data, clinical features, endocrinological dysfunction, imaging findings, treatment courses and follow-up periods were evaluated. Results: The frequency of hypophysitis was found 0.93% in all cases of the pituitary disease. Twelve patients (nine females and three males), ages between 17-61 years, were evaluated. The characteristic features of our patients tended to be predominantly female and young. Diagnosis of hypophysitis was made after pituitary biopsy in four patients, and in eight patients after pituitary operation due to adenoma. Headache (67%) and visual problems (33%) were the most frequent nonendocrine symptoms. Anterior pituitary hormone deficiencies (63.7%) and/or diabetes insipidus (17%) were seen among patients. According to histopathological forms, four had lymphocytic, seven had granulomatous and one had xanthogranulomatous types. Contrast enhancement heterogeneous and thickened pituitary stalk were the most common radiological alterations. Conclusion: Hypophysitis should be considered in the differential diagnosis of sellar masses. It can mimic pituitary adenomas in radiological and endocrinological aspects. The different patterns of pituitary hormone deficiencies may be seen in the course of the disease.

Objective and design A clinicopathological score has been proposed by Trouillas et al. to predict the evolution of pituitary adenomas. Aim of our study was to perform an independent external validation of this score and identify other potential predictor of post-surgical outcome. Methods The study sample included 566 patients with pituitary adenomas, specifically 253 FSH/LH-secreting, 147 GH-secreting, 85 PRL-secreting, 72 ACTH-secreting and 9 TSH-secreting tumours with at least 3-year post-surgical follow-up. Results In 437 cases, pituitary adenomas were non-invasive, with low (grade 1a: 378 cases) or high (grade 1b: 59 cases) proliferative activity. In 129 cases, tumours were invasive, with low (grade 2a: 87 cases) or high (grade 2b: 42 cases) proliferative activity. During the follow-up (mean: 5.8 years), 60 patients developed disease recurrence or progression, with a total of 130 patients with pituitary disease at last follow-up. Univariate analysis demonstrated a significantly higher risk of disease persistence and recurrence/progression in patients with PRL-, ACTH- and FSH/LH-secreting tumours as compared to those with somatotroph tumours, and in those with high proliferative activity (grade 1b and 2b) or >1 cm diameter. Multivariate analysis confirmed tumour type and grade to be independent predictors of disease-free-survival. Tumour invasion, Ki-67 and tumour type were the only independent prognostic factors of disease-free survival. Conclusions Our data confirmed the validity of Trouillas’ score, being tumour type and grade independent predictors of disease evolution. Therefore, we recommend to always consider both features, together with tumour histological subtype, in the clinical setting to early identify patients at higher risk of recurrence.

Introduction: Heterozygous mutations or haploinsufficiency of NKX2-1 are associated with the brain-lung-thyroid syndrome incorporating primary hypothyroidism, respiratory distress, and neurological disturbances. Case Presentation: We report a patient presenting in the neonatal period with multiple pituitary hormone deficiency including central hypothyroidism and hypoadrenalism, growth hormone deficiency, undetectable gonadotrophins, and a small anterior pituitary on MRI. CGH microarray revealed haploinsufficiency for NKX2.1 and during subsequent follow-up, she has exhibited the classic triad of brain-lung-thyroid syndrome with undetectable tissue on thyroid ultrasonography. Whilst the role of NKX2-1 is well described in murine pituitary development, this report constitutes the first description of multiple pituitary dysfunction in humans associated with the syndrome and haploinsufficiency NKX2-1. Conclusion: The report highlights a potential need for pituitary screening in patients with established brain-lung-thyroid syndrome and implicates NKX2.1 in human pituitary disease.

Summary IgG4-related hypophysitis is an important diagnostic consideration in patients with a pituitary mass or pituitary dysfunction and can initially present with headaches, visual field deficits and/or endocrine dysfunction. Isolated IgG4-related pituitary disease is rare, with most cases of IgG4-related disease involving additional organ systems. We report the case of a teenage female patient with isolated IgG4-related hypophysitis, diagnosed after initially presenting with headaches. Our patient had no presenting endocrinologic abnormalities. She was treated with surgical resection, prednisolone and rituximab with no further progression of disease and sustained normal endocrine function. This case, the youngest described patient with isolated IgG4-related hypophysitis and uniquely lacking endocrinologic abnormalities, adds to the limited reports of isolated pituitary disease. The use of rituximab for isolated pituitary disease has never been described. While IgG4-related hypophysitis has been increasingly recognized, substantial evidence concerning the appropriate treatment and follow-up of these patients is largely lacking. Learning points: IgG4-related hypophysitis most often occurs in the setting of additional organ involvement but can be an isolated finding. This diagnosis should therefore be considered in a patient presenting with pituitary abnormalities. Most patients with IgG4-related hypophysitis will have abnormal pituitary function, but normal functioning does not exclude this diagnosis. Corticosteroids have been the mainstay of therapy for IgG4-related disease, with other immunosuppressive regimens being reserved for refractory cases. Further research is needed to understand the effectiveness of corticosteroid-sparing regimens and whether there is utility in using these agents as first-line therapies.

Ca2+ influx through voltage-gated Ca2+ channels (VGCCs) plays a key role in GH secretion. In this review, we summarize the current state of knowledge regarding the physiology and molecular machinery of VGCCs in pituitary somatotrophs. We next discuss the possible involvement of Ca2+ channelopathies in pituitary disease and the potential use of Ca2+ channel blockers to treat pituitary disease. Various types of VGCCs exist in pituitary cells. However, because L-type Ca2+ channels (LTCCs) contribute the major component to Ca2+ influx in somatotrophs, lactotrophs, and corticotrophs, we focused on these channels. An increasing number of studies in recent years have linked genetic missense mutations in LTCCs to diseases of the human cardiovascular, nervous, and endocrine systems. These disease-associated genetic mutations occur at homologous functional positions (activation gates) in LTCCs. Thus, it is plausible that similar homologous missense mutations in pituitary LTCCs can cause abnormal hormone secretion and underlying pituitary disorders. The existence of LTCCs in pituitary cells opens questions about their sensitivity to dihydropyridines, a group of selective LTCC blockers. The dihydropyridine sensitivity of pituitary cells, as with any other excitable cell, depends primarily on two parameters: the pattern of their electrical activity and the dihydropyridine sensitivity of their LTCC isoforms. These two parameters are discussed in detail in relation to somatotrophs. These discussions are also relevant to lactotrophs and corticotrophs. High dihydropyridine sensitivity may facilitate their use as drugs to treat pituitary oversecretion disorders such as acromegaly, hyperprolactinemia, and Cushing disease.

BACKGROUND Perioperative steroid protocols for patients undergoing transsphenoidal surgery (TSS) for pituitary pathology vary by institution. OBJECTIVE To assess the safety of withholding glucocorticoids in patients undergoing TSS. METHODS Patients with an intact hypothalamic-pituitary-adrenal (HPA) axis undergoing TSS for a pituitary tumor at the same academic institution between 2012 and 2015 were randomized to either receive 100 mg of intravenous hydrocortisone followed by 0.5 mg of intravenous dexamethasone every 6 h for 4 doses (STER, n = 23) or to undergo surgery without steroids (NOSTER, n = 20). Postoperative cortisol levels were then used to determine the need for glucocorticoids after surgery. Data regarding postoperative cortisol levels, hospital stay length, and complications were collected. RESULTS Mean postoperative 8 am cortisol levels were higher in the NOSTER group compared to the STER group (745 ± 359 nmol/L and 386 ± 193 nmol/L, respectively, P = .001) and more patients were discharged on glucocorticoids in the STER group (42% vs 12%, P = .07). There was no difference in the incidence of postoperative complications, including hyperglycemia, diabetes insipidus, or permanent adrenal insufficiency. Permanent adrenal insufficiency occurred in 8% of patients. CONCLUSION Perioperative steroids can be safely withheld in patients with an intact HPA axis undergoing TSS. Although administration of perioperative glucocorticoids does not appear to increase the risk of complications, it may interfere with assessment of the HPA axis after surgery.

Background Pituitary lesions are present in >10% of the population. Approximately one in 1000 people has a symptomatic pituitary tumour, which may cause clinical problems from mass effect, hormonal hypersecretion and impairment of normal pituitary function. Objective The aim of this article is to outline the potential causes of a sellar and parasellar mass, with an emphasis on the presenting clinical features and screening investigations that are applicable to doctors working in the primary care setting. Discussion There is a broad range of causes of a sellar/parasellar mass. Pituitary adenomas and Rathke's cleft cysts are the most frequently encountered. Prolactinomas are the most common functioning tumour and tend to present as macroadenomas in men, while hyperprolactinaemia is associated with 15% of secondary amenorrhoea in women. Acromegaly and Cushing's disease are rare but important diagnoses to detect. Pituitary disease is optimally managed in a specialist centre in the context of an established multidisciplinary team.

Guidelines recommend adults with pituitary disease in whom GH therapy is contemplated, to be tested for GH deficiency (AGHD); however, clinical practice is not uniform. Aims (1) To record current practice of AGHD management throughout Europe and benchmark it against guidelines; (2) To evaluate educational status of healthcare professionals about AGHD. Design Online survey in endocrine centres throughout Europe. Patients and methods Endocrinologists voluntarily completed an electronic questionnaire regarding AGHD patients diagnosed or treated in 2017–2018. Results Twenty-eight centres from 17 European countries participated, including 2139 AGHD patients, 28% of childhood-onset GHD. Aetiology was most frequently non-functioning pituitary adenoma (26%), craniopharyngioma (13%) and genetic/congenital midline malformations (13%). Diagnosis of GHD was confirmed by a stimulation test in 52% (GHRH+arginine: 45%; insulin-tolerance: 42%, glucagon: 6%; GHRH alone and clonidine tests: 7%); in the remaining, ≥3 pituitary deficiencies and low serum IGF-I were diagnostic. Initial GH dose was lower in older patients, but only women Conclusion Despite guideline recommendations, GH replacement in AGHD is still not available or reimbursed in all European countries. Knowledge among professionals and health administrators needs improvement to optimise the care of adults with GHD.

The pituitary is the master gland that conducts the endocrine orchestra of the human body. Manifestations of pituitary toxicity are usually expressed as functional and structural changes in target endocrine glands under its control. Although several drugs can affect the pituitary gland, relatively low exposure of the pituitary cells to xenobiotics as the vascular supply and concentrating ability of the gland are less than the major organs of excretion and metabolism such as the liver and the kidneys. Pituitary apoplexy, originally described as postpartum ischemic necrosis of the anterior pituitary due to spasm of the infundibular arteries, is now known to be due to several causes including drugs. Case reports in the literature are limited and include bromocriptine, chlorpromazine, leuprorelin, clomiphene, and goserelin. Other complications include CSF leakage through the nose due to shrinking of macroprolactinomas as an effect of bromocriptine therapy. Hypopituitarism or pituitary insufficiency in anterior-pituitary hormone secretion may result from pituitary disease, and manifestations may be due to deficiency of corticotrophin, gonadotropins, androgen, or growth hormone. Drugs such antineoplastics, interferon, and corticosteroids may cause hypopituitarism. Several drugs have a secondary effect on the pituitary via induction of changes in the other endocrine function.

IgG4-related hypophysitis (IgG4-RH) is increasingly being reported as an isolated entity or, less frequently, as a manifestation of a multiorgan IgG4-related disease (IgG4-RD), in which typical histopathology is a cornerstone for the diagnosis. We aimed to describe the histopathological changes in the surgical specimens from patients with clinical signs of pituitary disease that fulfilled the current diagnostic criteria for IgG4-RH. Histopathological features were correlated with clinical and radiological findings. Of 19 patients with pituitary dysfunction and inflammatory changes in the surgical pituitary specimen operated on during 2011-2019, we identified five patients with typical IgG4-related pathology (lymphoplasmacytic infiltration with more than 10 IgG4-positive plasma cells per one high power microscopic field, representing at least 40% of all plasma cells and at least focal storiform fibrosis). One patient with diabetes insipidus and pachymeningitis with IgG4-related changes in a biopsy from the dura was also included. Additional histopathological changes that typically are not part of the IgG4-RH were observed: Rathke's cleft cyst in four and granulomatous changes in two patients. One patient had an elevated serum IgG4 level and systemic manifestations that could be associated with the systemic IgG4-RD. Our findings indicate that pure IgG4-RH is uncommon. All patients with pituitary dysfunction, beyond typical IgG4-related pathology, had other pathological findings that could trigger the secondary IgG4-response. Both primary pathology and secondary IgG4-related features should be reported in patients with pituitary dysfunction because their co-occurrence may cause atypical clinical and imaging features, and unexpected response to surgical and pharmacological treatment. The current criteria for the diagnosis of IgG4-RH can lead to overdiagnosis of IgG4-RH if additional pathological changes are not taken into consideration. The classification criteria of IgG4-RD proposed by the American College of Rheumatology/European League Against Rheumatism could help classify patients more properly as IgG4-RH if applied to the pituitary gland.

After treatment, patients with pituitary diseases often still experience complaints or persistent morbidity (physical or psychological), which tend to impair their everyday life and limit their professional, personal, or social activities. In other words, their health-related quality of life is affected. This is especially so in Cushing's disease (in fact, in both pituitary- and adrenal-dependent Cushing's syndrome), hypopituitarism (and especially GH deficiency), and acromegaly. Surgical and/or medical therapy and control of pituitary dysfunction are usually followed by improvement of quality of life, but it is still impaired when compared with healthy matched controls. Understanding of the potential health hazards after being diagnosed with a pituitary disease through patient education and understanding by the involved health professionals help to adapt to the new situation and tend to prevent depression and anxiety. Additionally, support by family, friends, or patient associations all help to improve patients’ perception of health and outcome, improving their quality of life.

Magnetic resonance imaging is the preferred method for diagnosis and follow-up of secreting and nonfunctioning pituitary adenomas. However, the frequency and duration of serial imaging follow-up varies depending on the adenoma type, pathology, biochemical status of the pituitary disease, and other factors. Clinical practice guidelines recommend performing early follow-up imaging in patients with a new or worsening neurological deficit and patients with a suspected cerebrospinal fluid leak, postoperatively. Otherwise imaging should be performed approximately 3 months after surgery; further imaging should be guided by clinical, biochemical, and pathological data. Recent data show that some gadolinium-based contrast agents could have a potential for long-term tissue deposition; thus macrocyclic agents are preferred until definitive safety evidence of cumulative use is available. Long-term imaging follow-up should be individualized and assessed in conjunction with patient's clinical and biochemical status and pituitary adenoma pathology.

Hypopituitarism after moderate or severe traumatic brain injury (TBI) is usually underdiagnosed and therefore undertreated. Its course can be divided in an acute phase during the first 14 days after TBI with 50 to 80% risk of hypopituitarism, and a chronic phase, beginning three months after the event, with a prevalence of hypopituitarism that ranges from 2 to 70%. Its pathophysiology has been addressed in several studies, suggesting that a vascular injury to the pituitary tissue is the most important mechanism during the acute phase, and an autoimmune one during chronic stages. In the acute phase, there are difficulties to correctly interpret pituitary axes. Hence, we propose a simple and cost-effective algorithm to detect and treat a potential hypothalamic-pituitary-adrenal axis impairment and alterations of sodium homeostasis, both of which can be life-threatening. In the chronic phase, post-concussion syndrome is the most important differential diagnosis. Given the high prevalence of hypopituitarism, we suggest that all pituitary axes should be assessed in all patients with moderate to severe TBI, between 3 to 6 months after the event, and then repeated at 12 months after trauma by a specialized team in pituitary disease.

Introduction The low prevalence of pituitary diseases makes patient autonomy crucial, and self-management programs should be more common. Objectives To assess the efficacy of an education program for patients with pituitary diseases in terms of patients’ quality of life, satisfaction and goal attainment. Design and methods Adult patients with pituitary disorders were recruited in a tertiary referral center and chose at least three of eight possible sessions on various topics, from disease management to psychosocial issues. Patients were included if they attended at least three sessions between 2012 and 2016 and completed the initial, final, and follow-up questionnaires. Data on quality of life (SF36), satisfaction and goal attainment were analyzed. Results Fifty-three patients were included (33 women; mean age, 53.5 years). There were a significant quality of life improvements in terms of physical and psychic limitation scores at the final assessment that persisted at follow-up evaluation. Most patients reached their objectives, especially those on sharing experiences and improving autonomy and self-confidence. More than half set new objectives at the end of the program, the most popular one being to reinforce their knowledge of their pituitary disease, its evolution and treatment (17.1% of patients). The mean overall satisfaction score was 3.75/4. At follow-up evaluation, patients reported improved self-management of pituitary disease (3.6/5) and improved self-efficacy (3.8/5). Conclusion Individualizing the educational objectives of patients with pituitary disease improves the way they live with their disease. If confirmed in other cohorts, this approach could become the gold standard for education programs in rare endocrine diseases.

Purpose The coronavirus disease 2019 (COVID-19) pandemic is widely believed to have had a major impact on the care of patients with pituitary disease. The virus itself may directly result in death, and patients with adrenal insufficiency, often a part of hypopituitarism, are thought to represent a particularly susceptible subgroup. Moreover, even in patients that do not contract the virus, the diversion of resources by healthcare institutions to manage the virus may indirectly result in delays in their management. To this end, the aim of this study was to determine the direct and indirect impact of the COVID-19 pandemic on patients with pituitary disease. Methods A cross-sectional study design was adopted, with all adult patients seen by our pituitary service in the year prior to the nationwide lockdown on March 23rd 2020 invited to participate in a telephone survey. Results In all, 412 patients (412/586; 70.3%) participated in the survey. 66 patients (66/412; 16.0%) reported having suspected COVID-19 infection. Of the 10 patients in this group tested for COVID-19 infection, three received a positive test result. No deaths due to COVID-19 were identified. 267 patients (267/412; 64.8%) experienced a delay or change in the planned care for their pituitary disease, with 100 patients (100/412; 24.3%) perceiving an impact to their care. Conclusions Whilst only a small percentage of patients had confirmed or suspected COVID-19 infection, over half were still indirectly impacted by the pandemic through a delay or change to their planned care.

Background: Dopamine agonists (DA) are the first line therapy for prolactinoma and symptomatic hyperprolactinemia; use as an adjuvant treatment for acromegaly and Cushing's disease is rare. Some patients develop de novo psychiatric symptoms or have exacerbation of pre-existing conditions during DA therapy. A practical, clinically sensitive depression and impulse control disorders (ICD; particularly hypersexuality and gambling disorders) detection tool is important for identifying at risk patients. The Barratt Impulsivity Scale (BIS-11) and the 9-item Patient Health Questionnaire (PHQ-9) are sensitive in identifying impulsivity and depression.Objective: Detail use of the BIS-11 and PHQ-9 as screening tools for depression and ICD in patients with pituitary disease at a high-volume academic pituitary center.Methods: DA-treated and naïve patients with pituitary disease were included. Patients with a known history of depression or psychiatric disorder were excluded. PHQ-9 standardized interpretation criteria were utilized to classify depression severity. For BIS-11, threshold was established based on previous studies. Statistical analysis was with SPSS version 25.Results: Seventy-six DA-treated and 27 naïve patients were included. Moderate and moderately severe depression were more prevalent in DA-treated patients; severe depression only found in DA-treated patients. A normal BIS-11 score was noted in 76.69%; higher scores (not significant) were noted in DA-treated patients. There was a positive correlation between higher BIS-11 and PHQ-9 scores; higher in DA-treated patients (r = 0.52, p < 0.001) than DA-naïve patients. Patients with BIS-11 scores ≥60 were younger and received lower cumulative DA doses compared to patients with BIS scores

Xanthogranuloma of the sellar region has been reported in both humans and animals. The lesion is rare, and its aetiology and pathogenesis are not fully understood. The association of sellar xanthogranuloma with an adenoma, known as xanthogranulomatous pituitary adenoma (XPA), is an extremely rare condition in humans, and is usually associated with anterior pituitary insufficiencies, headache, vomiting and visual deficits. We present the first report of XPA in an animal. A 7-year-old male neutered Labrador Retriever was presented for investigation of progressive lethargy, vomiting and hyporexia, having been previously diagnosed with chronic kidney disease, pituitary-dependent hyperadrenocorticism and hypoparathyroidism. The dog was euthanized due to lack of response to medical treatment and post-mortem examination revealed XPA. Although rare, xanthogranulomatous lesions should be considered in patients with pituitary disease.

To evaluate the prevalence of differentiated thyroid cancer (DTC) in patients with non-GH secreting pituitary adenomas [NGHPA group: non-functioning (NFPA), prolactin (PRL) and corticotropin (ACTH)-secreting adenomas] compared to patients with acromegaly, a pituitary disease that has been associated with increased risk for thyroid cancer.Prospective, cross-sectional study involving consecutive outpatients followed in our institution with diagnosis of acromegaly (n = 71; 43 women, median age 57 yrs) and NGHPA (n = 57; 38 women, median age 48 yrs.; PRL (n = 35), ACTH (n = 7), NFPA (n = 15). All participants were subjected to thyroid ultrasound (US) by the same examiner, and US-guided fine needle aspiration (FNA) biopsy when indicated.Thyroid volume was higher in acromegaly than in NGHPA (median 12.5 ml vs 6.3 ml; p 0.0001), and thyroid nodules were present in 27/71 (38.0%) of acromegaly patients and in 14/57 (24.6%) of NGHPA group. FNA was indicated in 15/27 (55.5%) of acromegaly patients [Bethesda I (n = 1); II (n = 11), III (n = 1), two patients refused FNA], and in 8/14 (57.1%) of the NGHPA group [Bethesda I (n = 2); II (n = 4); V (n = 1); VI (n = 1)]. The two patients of NGHPA group with Bethesda V and VI were operated and papillary carcinoma was confirmed histologically.DTC was not detected in our acromegaly patients and its presence in patients with NGHPA suggests that DTC predisposition is not related to GH excess.

Background: Adult growth hormone deficiency (GHD) is considered a rare condition. Current guidelines state that GH provocative test is indicated in patients affected by organic hypothalamic/ pituitary disease or with a history of head injury, irradiation, hemorrhage or hypothalamic disease with multiple pituitary deficiencies. Nevertheless, the clinical picture related to GHD may be subtle. Objective: We have retrospectively evaluated the indication to GHRH+arginine test in our monocentric cohort of patients treated with hrGH in order to assess whether other conditions had been considered as a rationale for provocative testing. Methods: Ninety-six patients (51 females and 45 males), aged 19-67 years were included. The GHRH+arginine test had been performed in 29 patients with organic hypothalamic/pituitary disease and in 4 patients for Childhood onset-GHD (CoGHD). In other patients, the diagnosis was suspected for “non classical” reasons in the clinical picture suspected for GHD. Results: Classical indications included previously known primary empty sella (n=15), pituitary surgery (n=14), pituitary cyst (n=1), non-secreting pituitary tumors (n=3) but more than half of the patients (57.3%) had been studied for “non classical” indications: metabolic syndrome (n=25), asthenia (n=13), heart failure (n=4), osteoporosis (n=6), unexplained hypoglycaemia (n=1) and infertility (n=6). The latter represented a significant percentage in the male subgroup under 45 ys. IGF-1 levels were lower than 50th percentile in 63% of patients. Finally, among non-classical reasons, organic pituitary disease was discovered in 22 patients. Conclusions: Idiopathic GHD may be unrecognized due to its subtle manifestations and that an extended use of dynamic GH tests may reveal such conditions. A potential field of investigation could be to identify subsets of patients with clinical conditions caused or worsened by underlying unrecognized GHD.

Purpose Highlight and characterize manifestations, diagnostic/management approaches and outcomes in a contemporary cohort of patients with pituitary metastases (PM) from a large European pituitary center—over 10 years. Methods Retrospective review of PM cases between 1/2009 and 12/2018. Clinical, laboratory, imaging data at PM detection and during follow-up were analysed. Results 18 cases were identified (14 females; median age at diagnosis 61.5 years). Most common primary malignancies were lung (39%) and breast (32%). Most frequent presenting manifestation was visual dysfunction (50%). Gonadotrophin, ACTH, TSH deficiency were diagnosed in 85%, 67%, 46% of cases, respectively; diabetes insipidus (DI) was present in 17%. 33% of cases were detected during investigation for symptoms unrelated to PM. PM management included radiotherapy (44%), transsphenoidal surgery (17%), transsphenoidal surgery and radiotherapy (6%) or monitoring only (33%). One-year survival was 49% with median survival from PM detection 11 months (range 2–47). Conclusions In our contemporary series, clinical presentation of PM has evolved; we found increased prevalence of anterior hypopituitarism, decreased rates of DI and longer survival compared with older literature. Increased availability of diagnostic imaging, improvements in screening and recognition of pituitary disease and longer survival of patients with metastatic cancer may be contributing factors.

Pregnancy results in a significant change in both pituitary gland size and function. Due to this physiological adaptation, the diagnosis and management of pituitary diseases during pregnancy represents a particularly complex challenge. The presence of a functioning pituitary adenoma may be harmful to the health of the mother and fetus, and scientific evidence regarding the safety of drugs normally used to control hormone excess during pregnancy is scarce. In addition, pregnancy may be associated with the risk of the growth of a pre-existing pituitary adenoma. This review focuses on the diagnostic challenges in pregnant women with adenomas secreting prolactin, growth hormone, or adrenocorticotropic hormone. Some evidence-based recommendations for the treatment of these conditions during pregnancy are provided, and algorithms that could help monitor a pituitary adenoma during pregnancy are examined. Mention is also made of how hormone replacement therapy can be optimised in pregnant women with hypopituitarism. Finally, differential diagnosis between Sheehan's syndrome and lymphocytic hypophysitis, two pituitary disorders that may occur during pregnancy or delivery, is discussed.

Context Clinically nonfunctioning pituitary adenomas (CNFPAs) typically remain undetected until mass effect symptoms develop. However, currently, head imaging is performed commonly for many other indications, which may increase incidental discovery of CNFPAs. Since current presentation and outcome data are based on older, retrospective series, a prospective characterization of a contemporary CNFPA cohort was needed. Objective To determine the prevalence of incidental presentation and hypopituitarism and its predictors in a CNFPA cohort that spanned 6 to 9 mm micro- to macroadenoma included observational and surgical therapy. Methods At enrollment in a prospective, observational study, 269 patients with CNFPAs were studied by history, examination, blood sampling, and pituitary imaging analysis and categorized into incidental or symptoms presentation groups that were compared. Results Presentation was incidental in 48.7% of patients and due to tumor symptoms in 51.3%. In the symptoms and incidental groups, 58.7% and 27.4% of patients had hypopituitarism, respectively, and 25% of patients with microadenomas had hypopituitarism. Many had unappreciated signs and symptoms of pituitary disease. Most tumors were macroadenomas (87%) and were larger in the symptoms than incidental and hypopituitary groups than in the eupituitary groups. The patients in the incidental group were older, and males were older and had larger tumors in both the incidental and symptoms groups. Conclusions Patients with CNFPAs commonly present incidentally and with previously unrecognized hypopituitarism and symptoms that could have prompted earlier diagnosis. Our data support screening all large micro and macro-CNFPAs for hypopituitarism. Most patients with CNFPAs still have mass effect signs at presentation, suggesting the need for more awareness of pituitary disease. Our ongoing, prospective observation of this cohort will assess outcomes of these CNFPA groups.

Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis through clinical criteria and non-invasive methods and predicting the patients’ clinical outcome. The aim of this review is to describe the neuroradiological findings of this rare disease, providing some information regarding the possible differential diagnosis in order to avoid unnecessary surgery. Gadolinium-enhanced pituitary magnetic resonance imaging (MRI) is considered the neuroradiological investigation of choice. The features suggestive for HP include an enlarged triangular- or dumbbell-shaped gland with a thickened and not obviously deviated stalk, further supported by the absence of posterior pituitary bright spot on T1weighted images, particularly in patients presenting with diabetes insipidus. Contrast enhancement pattern is quite variable; dural enhancement has been reported in some cases after intravenous contrast administration. The characterization of the unusual sellar mass is not straightforward and generally results in a wide differential. HP should be primarily differentiated from pituitary adenomas (including pituitary apoplexy), from pituitary metastases, and from other sellar and parasellar tumors, e.g., craniopharyngiomas, germinomas, gliomas, lymphomas, meningiomas, pituicytomas, chordomas, teratomas, dermoids and epidermoids, Rathke’s cleft cysts, and abscesses. In patients suspected for secondary forms related to systemic pathology, additional imaging is helpful in identifying other involved sites. Neuroradiologists need to know MRI appearance of this rare disease, as well as its typical symptoms and serological markers. A strict collaboration with endocrinologists and neurosurgeons is mandatory in order to reach a definitive diagnosis, allowing to promptly initiating an appropriate treatment.

The presence of a pituitary adenoma may affect the course of pregnancy, as the hormonal changes related to these tumors may lead to early termination of pregnancy due to failure to implant or maintain the conceptus or early embryo. The age-adjusted incidence rate of pituitary adenomas is estimated to be 3.4 cases per 100,000 inhabitants per year. They are usually benign adenomas, with a peak incidence in young women of childbearing age. The management of pituitary adenomas during pregnancy depends on its clinical presentation and should be adapted to the individual case. Most pregnant women with pituitary adenomas can be safely observed with frequent neuro-ophthalmologic assessments and MRI, if needed. Among women with pituitary adenomas, prolactin (PRL)-secreting pituitary adenomas (prolactinomas) are the most common. Dopamine agonists (DAs) are the gold standard treatment for prolactinomas; they normalize serum PRL levels, leading to tumor shrinkage in more than 80% of cases and restoration of eugonadism. In micro- and intrasellar macroprolactinomas, DA is usually withdrawn when pregnancy is confirmed. In pregnant women with acromegaly, hormonal control is often achieved in most patients, allowing the withdrawal of clinical treatment. Due to similar clinical features and changes in the hypothalamic-pituitary-adrenal (HPA) axis during pregnancy, the diagnosis of Cushing disease (CD) during gestation can be difficult. Similar to nonpregnant women, surgery is the first treatment option for CD during pregnancy, if complications develop. Overall, pregnancy in women harboring clinically nonfunctioning tumors is a rare event. Causes of hypopituitarism that are most specific to pregnancy include lymphocytic hypophysitis and postpartum pituitary infarction (Sheehan’s syndrome). During pregnancy, the priority for hormonal replacement should be glucocorticoid, followed by thyroid hormone. Doses should be adjusted throughout pregnancy based on the severity and nature of the condition. For this reason, it is necessary to follow these patients regularly and closely during pregnancy. Central or nephrogenic diabetes insipidus (DI) can also be observed during pregnancy. Therefore, a detailed medical history is essential for the differential diagnoses of DI. Primary polydipsia and head trauma should be excluded. Ingestion of drugs such as lithium, mannitol, diuretics, and anticholinergic drugs should be questioned. Management of pituitary disease during pregnancy in otherwise healthy women poses difficult challenges from various perspectives. However, a multidisciplinary approach, involving the endocrinologist, obstetrician, neurosurgeon, and anesthesiologist, will allow a better outcome for both mother and fetus during pregnancy.

Several different proteins regulate, directly or indirectly, the production of growth hormones from the pituitary gland, thereby complex genetics is involved. Defects in these genes are related to the deficiency of growth hormones solely, or deficiency of other hormones, secreted from the pituitary gland including growth hormones. These studies can aid clinicians to trace the pattern of the disease between the families, start early treatment and predict possible future consequences. This paper highlights some of the most common and novel genetic anomalies concerning growth hormones, which are responsible for various genetic defects in isolated growth and combined pituitary hormone deficiency disease.

A recent publication described pathological findings in the pituitary gland incidentally discovered during routine necropsies of the brain of dogs and cats; however, imaging characteristics of these lesions were not reported. Aims of this retrospective, observational study were to characterize MRI variants and incidental lesions in pituitary glands of dogs with no clinical signs of pituitary disease. Cranial MRIs from dogs with no suspicion of pituitary disease, based on history and presenting clinical signs, were retrieved from a veterinary teleradiology database during the period of January 2014 to January 2016. Images were reinterpreted by two observers and pituitary lesions were described based on consensus. A total of 580 scans were evaluated and pituitary lesions were detected in 78 dogs (13.44%). Pituitary cystic lesions were the most common finding and occurred in 31 dogs (5.34%). Of these 31 dogs, the majority (74%) were of toy or brachycephalic breed. Partial or total empty sella lesions were detected in 14 dogs (2.41%), and all of these were small or toy breeds. A significantly increased incidence of the partial empty sella lesion was found in male dogs (P = .034). Pituitary lesions greater than 1 cm occurred rarely (0.69%). There was a significant association between low-field (LF) MRI strength and detection of a partial or total empty sella lesion (P = .0112), and detection of a pituitary lesion greater than 1 cm (P = .0125). A significant difference was present between the MRI field strength (FS) that identified pituitary cysts and the FS that detected an empty sella (P = .0068), with the former being a high FS and the latter a LF strength. The findings from this study indicated that up to 13% of dogs with no presenting clinical signs of pituitary disease may have MRI pituitary lesions.

People with pituitary disease report impairments in quality of life. The aim of this study was to elucidate the impact of the pituitary condition on the lives of partners. Four focus groups of partners of people with pituitary disease (Cushing’s disease, non-functioning adenoma, acromegaly, prolactinoma) were conducted. Partners mentioned worries related to the pituitary disease and negative beliefs about medication, coping challenges, relationship issues, social issues and unmet needs regarding care. This study emphasizes the importance of not only paying attention to psychosocial well-being of people with pituitary disease but also to their partners.

BACKGROUND Diagnosis of Cushing syndrome requires a multistep process that includes verification of hypercortisolism followed by identification of the cause of adrenocortical hyperfunction. This study assessed whether pituitary, ectopic, and adrenal subtypes of Cushing syndrome were characterized by distinct plasma steroid profiles that might assist diagnosis. METHODS In this retrospective cross-sectional study, mass spectrometric measurements of a panel of 15 plasma steroids were applied to 222 patient samples tested for Cushing syndrome. Disease was excluded in 138 and confirmed in 51 patients with pituitary Cushing syndrome, 12 with ectopic adrenocorticotropin secretion, and 21 with adrenal disease. Another 277 age- and sex-matched hypertensive and normotensive volunteers were included for comparison. RESULTS Compared with patients without disease, the largest increases in plasma steroids among patients with Cushing syndrome were observed for 11-deoxycortisol (289%), 21-deoxycortisol (150%), 11-deoxycorticosterone (133%), corticosterone (124%), and cortisol (122%). Patients with ectopic disease showed the most prominent increases, but there was considerable variation for other steroids according to subtype. Patients with adrenal disease had the lowest concentrations of androgens, whereas those with ectopic and pituitary disease showed the lowest concentrations of aldosterone. Plasma 18-oxocortisol was particularly low in ectopic disease. With the use of 10 selected steroids, subjects with and without different Cushing syndrome subtypes could be discriminated nearly as closely as with the use of salivary and urinary free cortisol, dexamethasone-suppressed cortisol, and plasma adrenocorticotropin (9.5% vs 5.8% misclassification). CONCLUSIONS Patients with different subtypes of Cushing syndrome show distinctive plasma steroid profiles that may offer a supplementary single-test alternative for screening purposes.

Background: Growth hormone (GH) treatment may unmask central hypothyroidism (CeH). This was first observed in children with GH deficiency (GHD), later also in adults with GHD due to acquired “organic” pituitary disease. We hypothesized that newly diagnosed CeH in children after starting GH treatment for nonacquired, apparent isolated GHD points to congenital “organic” pituitary disease. Methods: Nationwide, retrospective cohort study including all children with nonacquired GHD between 2001 and 2011 in The Netherlands. The prevalence of CeH, hypothalamic-pituitary (HP) abnormalities, and neonatal congenital hypothyroidism screening results were evaluated. Results: Twenty-three (6.3%) of 367 children with apparent isolated GHD were prescribed LT4 for presumed CeH within 2 years after starting GH treatment. Similarly to children already diagnosed with multiple pituitary hormone deficiency, 75% of these 23 had structural HP abnormalities. In children not prescribed LT4, low pre- or post-GH treatment FT4 concentrations were also associated with structural HP abnormalities. Neonatal screening results of only 4 of the 23 children could be retrieved. Conclusion: In children with nonacquired, apparent isolated GHD, a diagnosis of CeH after, or a low FT4 concentration around the start of GH treatment, is associated with congenital structural HP abnormalities, i.e., “organic” pituitary disease. Neonatal values could not be judged reliably.

Patients with growth hormone deficiency (GHD) have an increased risk of atherosclerosis and vascular mortality. Evidence suggests that endothelial dysfunction is involved in all stages of atherogenesis. This study examined the effect of growth hormone (GH) replacement therapy on diacron-reactive oxygen metabolites (d-ROMs) and endothelial function in Japanese patients with GHD, using peripheral arterial tonometry. This was an open-label, prospective, case-control study. Nine patients with GHD who had not previously received any GH replacement therapy were enrolled. The following parameters were evaluated at baseline (before treatment), and after 24 weeks of GH replacement therapy: endothelial function using the reactive hyperemia index (RHI; EndoPAT® system), d-ROMs, blood pressure, and fasting lipid levels. Plasma GH and insulin-like growth factor-1 (IGF-1) levels were measured at baseline and after 24 weeks of GH replacement therapy. We also enrolled eight controls with pituitary disease but no GH deficiency. Over 24 weeks of GH replacement therapy, the serum IGF-1 levels normalized with significant improvement in the RHI (from 1.65 ± 0.33 to 1.92 ± 0.26, p < 0.05) and decreased d-ROM levels (from 356.8 ± 64.1 to 303.1 ± 43.3 U.CARR, p < 0.05). There were no significant improvements in the RHI or d-ROM levels in controls. GH replacement therapy in Japanese patients with GHD may be mediated by the reduced oxidative stress and the d-ROMs associated with the treatment.

Measuring IGF-1, a biomarker for GH activity, is critical to evaluating disordered hypothalamic-pituitary GH axis. Inconsistent IGF-1 measurements among different immunoassays are well documented. We switched from Immulite 2000 immunoassay to narrow-mass-extraction, high-resolution liquid chromatography mass-spectrometry (LC-MS) compliant with recent consensus recommendations on assay standardization. Comparability of these two assays in patients with pituitary disease in a clinical practice setting is not known. We sought to compare IGF-1 levels on Immulite 2000 and LC-MS in samples from naive and treated patients with secretory and non-secretory pituitary masses. We prospectively collected serum samples from 101 patients treated at the Cedars-Sinai Pituitary Center between February 2012 and March 2014. We intentionally recruited more patients with acromegaly or GH deficiency to ensure a clinically representative cohort. Samples were classified as in or out of the respective reference ranges. Bland–Altman analysis was used to assess agreement between assays. Twenty-four percent of samples were classified differently as below, in, or above range. Agreement between the assays was poor overall, with a significant bias for immunoassay reporting higher values than LC-MS. This pattern was also observed in patients with acromegaly and those with ≥ 2 pituitary hormone deficiencies. IGF-1 results may differ after switching from an older immunoassay to a consensus-compliant assay such as LC-MS. Clinicians should consider the potential impact of assay switching before altering treatment due to discrepant results, particularly in patients monitored over time, such as those with acromegaly and GH deficiency.

Central nervous system (CNS) disease is an uncommon but significant complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and affects 3 primary areas of the CNS: the pituitary, the pachymeninges, and the CNS vasculature. Pituitary disease in uncommon, but hormonal deficiencies can be long lasting even in the face of excellent disease response. Chronic hypertrophic pachymeninigitis occurs in anti-proteinase 3-positive patients with systemic GPA and in anti-myeloperoxidase-positive patients with a milder and more limited form of the disease. Cerebral and spinal vasculitis due to GPA and MPA presents with focal and general neurologic abnormalities.

Background The use of combined positron emission tomography/computed tomography for staging in patients with cancer and the widespread use of magnetic resonance imaging has led to increased detection of incidental sellar masses. The imaging findings can be suggestive of a benign pituitary tumor, but metastasis can never be completely ruled out with noninvasive work-up. Appropriate diagnosis of sellar masses is critical, as the treatment paradigm might change in the presence of a pituitary metastasis. Definitive tissue diagnosis might prevent unnecessary radiotherapy to the skull base or the need for systemic treatment when benign pituitary disease is confirmed. Methods A retrospective chart review from 2010 to 2015 of all patients with recently diagnosed cancer and undergoing surgery for sellar region masses was performed. Results There were 9 patients (3 female and 6 male) identified. Lung cancer was the primary condition in 4 patients; the remaining 5 patients had breast cancer, follicular thyroid cancer, cutaneous melanoma, colorectal carcinoma, and renal cell carcinoma. On final pathology, the sellar mass was a benign pituitary adenoma in 5 patients, metastatic cancer in 3 patients, and a granular cell tumor in 1 patient. Conclusions Surgical resection of a sellar mass in patients with known cancer helps in the definitive diagnosis, relieves compressive symptoms, and avoids unnecessary empiric radiotherapy in cases of confirmed benign pituitary disease.