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Pituitary apoplexy (PA) is a clinical condition characterised by a sudden increase in pituitary gland volume secondary to ischaemia and/or necrosis. Most cases occur in non-functioning pituitary adenoma but can also occur in functioning adenoma. Certain predisposing factors can result in PA and the use of gonadotropin-releasing hormone (GnRH) agonists for prostate cancer (PCa) is one such condition. Once diagnosed, both surgical and conservative management has been used for the treatment of PA. We present a case of a man in his late 50s who developed PA following treatment of PCa with leuprolide. His symptoms developed insidiously and he presented 6 months after symptom onset. Anterior pituitary hormone workup along with pituitary MRI confirmed the diagnosis of PA and patient was subsequently treated with adequate replacement of pituitary hormone with significant improvement in his symptoms. It is very important to keep a high index of suspicion for PA, especially among elderly patients receiving GnRH agonist treatment for PCa.

Hyperprolactinemia has been proven to induce hypogonadism and metabolic derangements in both genders, while the consequences of prolactin (PRL) deficiency have been poorly investigated.To systematically review and analyze data from clinical studies focusing on the metabolic consequences of abnormally high prolactin levels (HPRL) and low prolactin levels (LPRL). In addition, data from preclinical studies about underlying pathophysiological mechanisms were summarized and discussed.PRL contributes to providing the correct amount of energy to support the mother and the fetus/offspring during pregnancy and lactation, but it also has a homeostatic role. Pathological PRL elevation beyond these physiological conditions, but also its reduction, impairs metabolism and body composition in both genders, increasing the risk of diabetes and cardiovascular events. Hence, hypoprolactinemia should be avoided as much as possible during treatment with dopamine agonists for prolactinomas. Patients with hypoprolactinemia, because of endogenous or iatrogenic conditions, deserve, as those with hyperprolactinemia, careful metabolic assessment.

Objective To profile clinically non-aggressive and aggressive pituitary adenomas (PAs)/pituitary neuroendocrine tumours (PitNETs) and pituitary carcinomas for somatic mutations and epigenetic alterations of genes involved in cell proliferation/differentiation, microRNAs (miRNA)/long noncoding RNA (LncRNA)-post-transcriptional regulators and therapy targets. Design Retrospective observational study. Patients and Measurements A total of 64 non-aggressive and 41 aggressive PAs/PitNETs and 6 pituitary carcinomas treated by endoscopic surgery with >= 1-year follow-up were included. Somatic mutations of 17 genes and DNA methylation of 22 genes were assessed. Ten normal pituitaries were used as control. Results We found at least one mutation in 17 tumours, including 6/64 non-aggressive, 10/41 aggressive PAs/PitNETs, and 1/6 pituitary carcinoma. AIP (N = 6) was the most frequently mutated gene, followed by NOTCH (4), and TP53 (3). Hypermethylation of PARP15, LINC00599, ZAP70 was more common in aggressive than non-aggressive PAs/PITNETs (p < .05). Lower levels of methylation of AIP, GNAS and PDCD1 were detected in aggressive PAs/PITNETs than non-aggressive ones (p < .05). For X-linked genes, males presented higher level of methylation of FLNA, UXT and MAGE family (MAGEA11, MAGEA1, MAGEC2) genes in aggressive vs. non-aggressive PAs/PITNETs (p < .05). In pituitary carcinomas, methylation of autosomal genes PARP15, LINC00599, MIR193 and ZAP70 was higher than in PAs/PITNETs, while X-linked genes methylation level was lower. Conclusions Somatic mutations and methylation levels of genes involved in cell proliferation/differentiation, miRNA/LncRNA-post-transcriptional regulators and targets of antineoplastic therapies are different in non-aggressive and in aggressive PAs/PitNETs. Methylation profile also varies according to gender. Combined genetic-epigenetic analysis, in association with clinico-radiological-pathological data, may be of help in predicting PA/PitNET behaviour.

BACKGROUND: Prolactin is a hormone of the pituitary gland whose main function is the production of milk. Hyperprolactinemia is defined as an increase in prolactin levels above 25 μg/L in women and 20 μg/L in men. Causes of hyperprolactinemia include pituitary tumors, especially prolactinomas. Hyperprolactinemia can manifest clinically with a variety of symptoms, including galactorrhea and menstrual irregularities in women and erectile dysfunction in men. There are limited data on the epidemiology of hyperprolactinemia in the Middle East region. OBJECTIVES: Description of the epidemiology and clinical features of hyperprolactinemia in a cohort from Saudi Arabia. DESIGN: Medical record review SETTING: Tertiary medical center in Riyadh PATIENTS AND METHODS: The study included adult patients with hyperprolactinemia in King Abdulaziz Medical City in Riyadh. The patients were treated in endocrinology clinics from 2015 to 2019. Patients of both sexes older than 14 years were enrolled in the study. Patients with insufficient follow-up were excluded. Data were collected on demographic characteristics, symptoms, prolactin level, cause of high prolactin level, and treatment. MAIN OUTCOME MEASURES: The frequency of different etiologies and symptoms in patients with hyperprolactinemia. SAMPLE SIZE: 295 patients RESULTS: The majority of patients with hyperprolactinemia were female 256 (86.8%). Hyperprolactinemia was diagnosed more frequently in patients in the age groups 21–30 years (42.6%) and 31–40 years (24.1%). The majority of the study population was obese or overweight: 136 (46.3%) and 74 (25.2%), respectively. Most of the cases were symptomatic (192, 65.1%). In women, the most common symptom was oligomenorrhea (35%). In men, infertility and erectile dysfunction were the most common clinical symptoms (50% and 44.7%, respectively). Idiopathic causes were the most common etiology (108, 36.6%), followed by pituitary adenomas (81, 27.5%). The majority of patients were treated (184,62.4%), with cabergoline being the most commonly used medication (173, 94.0%). CONCLUSION: The demographic and clinical presentations and causes of hyperprolactinemia in male and female Saudi patients were similar to that in studies in other populations. LIMITATIONS: Single-center retrospective chart review study. CONFLICT OF INTEREST: None.

Objective Since the outbreak of the COVID-19 pandemic, several cases of pituitary apoplexy (PA) following a SARS-CoV-2 infection have been described in several countries. Here, we describe a case series of PA occurring in the aftermath of a SARS-CoV-2 infection to alert physicians about possible neuro-endocrinological damage caused by the virus that can lead to visual sequelae and hypopituitarism. Design and methods We retrospectively identified all the adult patients treated at Amiens University Hospital between March 2020 and May 2021 for PA confirmed by cerebral imaging and following an RT-PCR-confirmed SARS-CoV-2 infection. Results Eight cases (six women, two men) occurred between March 2020 and May 2021 and were reviewed in this study. The mean age at diagnosis was 67.5 ± 9.8 years. Only one patient had a ‘known’ non-functional pituitary macroadenoma. The most common symptom of PA was a sudden headache. Brain imaging was typical in all cases. Only two patients required decompression surgery, whereas the others were managed conservatively. The clinical outcome was favorable for all patients but without recovery of their pituitary deficiencies. There was no diabetes insipidus. Conclusion This case series, the largest in the literature, reinforces the strength, consistency, and coherence of the association between SARS-CoV-2 infection and PA. Our study provides support for the hypothesis that SARS-CoV-2 may be a new precipitating factor for PA. It is essential that practitioners be alerted about possible pituitary disease due to the virus so that such patients are recognized and appropriately managed, hence improving their prognosis.

A cardiac myxoma is a rare tumor that could be incidental or present with common symptoms due to embolization. A minority of cases are attributed to carney complex, a rare inherited disease. A 73-year-old Asian male presented with acute left-side weakness, slurred speech, gait imbalance, and subacute constitutional symptoms. Left atrial myxoma was discovered by computed tomography and confirmed by echocardiography. Brain imaging revealed pituitary macroadenoma with subarachnoid and intraventricular hemorrages. The hormonal profile confirmed pituitary apoplexy, for which hormone replacement was initiated. Workup also revealed multiple endocrine tumors and excluded infection and malignancy. Myxoma resection could not be carried out, due to the patient's rapid clinical deterioration and death.Furthermore, the presence of cardiac myxoma, non-functioning pituitary macroadenomas, and pituitary apoplexy is extremely rare and rarely documented in the literature. Therefore, we emphasize clinical awareness of rare conditions with atypical presentations to improve outcomes.

To study the incidence and prognostic factors of patients with benign pituitary tumors based on the Surveillance, Epidemiology, and End Results (SEER) database.This was a retrospective cohort study. Patients with benign pituitary tumors reported in the Surveillance, Epidemiology, and End Results database from 2004 to 2016, who presented completed demographic and clinical data, were included in our study. The age-adjusted incidence rate was calculated and stratified by year at diagnosis, age, gender, and the pathological type of benign pituitary tumor. We used Kaplan-Meier curves and multivariable Cox regressions to determine the factors associated with overall survival.A total of 29,967 patients were included in the study, of whom 26,691 (89.07%) survived and 3276 (10.93%) died. The age-adjusted incidence rate increased from 3.15 per 100,000 person-years in 2004 to 4.66 per 100,000 person-years in 2011 (annual percent change = 5.51, P0.001), and the subsequent growth trend from 2011 to 2016 was not statistically significant (annual percent change = 0.26, P = 0.711). Most patients were female, aged 60-79 years, and pituitary adenomas accounted for the main proportion of the incidence of benign pituitary tumors. Surgery was associated with the overall survival on the multivariable Cox regression model (hazard ratio = 0.677, 95% confidential interval: 0.629-0.727) and Kaplan-Meier curves, especially in pituitary adenoma. Radiation was not associated with the overall survival of benign pituitary tumor.The overall incidence of benign pituitary tumors was low but showed an increasing trend. Surgery may be beneficial to the prognosis. It should be noted that benign pituitary tumors may not require excessive treatment, such as radiation.

Purpose:To study the baseline characteristics predicting hypogonadotropic hypogonadism (HH) persistence in men with macroprolactinoma that achieved prolactin normalization.Design:Retrospective cohort study.Methods:Male patients diagnosed with macroprolactinoma and HH that received cabergoline treatment with subsequent prolactin normalization were included: men that achieved eugonadism, and men that remained hypogonadal. Patient’s demographic, clinical and biochemical parameters, sellar imaging and visual fields tests were obtained. Univariate and multivariate models were used to identify predictors of HH persistence.Results:Fifty-eight male patients (age, 49.2±12.6 years) with a median baseline prolactin of 1154 ng/mL (IQR, 478-2763 ng/mL) and adenoma (maximal) diameter of 25.9±14.8 mm were followed for a median of 5.6 years (IQR, 3.0-10.7). Twelve men (21%) suffered from HH persistence at the end of follow-up and 46 men achieved eugonadism. Forty-two out of 46 men (91%) accomplished eugonadism within the first year following prolactin normalization.In a multivariate logistic regression model, hypopituitarism (OR=10.1; 95% CI 1.10 – 101.94), visual field defect (OR=9.9; 95% CI 1.07 – 92.33), and low baseline testosterone levels (OR=0.5; 95% CI 0.29 – 0.93) were independent predictors of HH persistence.Conclusion:In our cohort of men with macroprolactinoma that reached prolactin normalization with cabergoline treatment, 21% had HH persistence. Pituitary hormone deficiency, visual field defects, and low baseline testosterone levels were independently associated with HH persistence. 91% of men achieved eugonadism within the first year following prolactin normalization. These findings may support informed clinical decision-making regarding the initiation of testosterone replacement in men with macroprolactinomas.

Craniopharyngiomas involving the ventricular system are rare but pose significant surgical challenges. We systematically reviewed the literature on craniopharyngiomas invading the ventricles (CP-V).PubMed, EMBASE, Scopus, Web of Science, and Cochrane were searched to include studies reporting clinical data of patients with CP-Vs. Clinico-radiological features, management, and treatment outcomes were analyzed.We included 73 studies encompassing 407 patients. Patients were mostly male (61.5%), presenting with headache (57.9%) and/or endocrine disorders (52.1%). CP-Vs mostly involved the third ventricle (96.3%), followed by the lateral ventricles (2.9%), and the fourth ventricle (1%). Tumors had cystic components in 59% of cases and were mostly adamantinomatous (70.8%). Open resection was performed in 232 cases (57%), mostly with trans-lamina terminalis (36.6%) and trans-callosal (31.9%) approaches. Endoscopic resection was performed in 169 cases (41.5%), mostly with trans-sphenoidal (74.6%) and transventricular (24.9%) approaches. Gross-total tumor resection was obtained in most cases (62.9%). Adjuvant radiotherapy was delivered in 22.8% cases. A total of 178 patients experienced persistent complications, mostly including diabetes insipidus (47.1%) and panhypopituitarism (12.7%), not significantly different after open versus endoscopic resection (p=0.117). Symptom improvement was obtained in 88% of cases. CP-Vs recurrences were reported in 94 patients (23.1%), with median progression-free survival of 13.5 months (range=0.5-252.0 months). Fifty-nine patients died (14.5%), with median overall survival of 32.0 months (range=0.5-252.0 months), significantly longer after endoscopic resection than open resection (p=0.019).CP-Vs are uncommon and challenging entities. Surgical resection is feasible, but patient-tailored selection of open/endoscopic approaches is necessary to achieve optimal outcomes and minimize complication risks.

Purpose/Objective Multiple tumorous lesions in one pituitary gland are rare and mostly described in case reports. Their incidences and combinations are defined in larger collectives. Therefore, we analyzed our large collection for double tumors and combinations of tumors, cysts, and inflammation. Methods The German Registry of Pituitary Tumors, including cases from 1990 to 2018, served as the database. Our collection comprises a total of 16,283 cases up until the end of 2018. Of these cases, 12,673 originated from surgical and 3,610 from autopsy material. All specimens were fixed in formalin and embedded in paraffin. The sections were stained with hematoxylin–eosin and PAS. Monoclonal (prolactin, TSH, FSH, LH, and α subunit) or polyclonal (GH and ACTH) antibodies were used to detect pituitary hormones in the lesions. Since 2017, antibodies against the transcription factors Pit-1, T-Pit, and SF-1 have been used in difficult cases. The criteria of the 2017 WHO classification have been basic principles for classification since 2018 (Osamura et al. 2017). For differentiation of other sellar tumors, such as meningiomas, chordomas, or metastases, the use of additional antibodies was necessary. For these cases, it was possible to use a broad antibody spectrum. Autopsy pituitaries were generally studied by H&E and PAS sections. If any lesions were demonstrated in these specimens, additional immunostaining was performed. Results Multiple tumorous lesions with more than one pituitary neuroendocrine tumor (PitNET) respectively adenoma make up 1.4% (232 cases) in our collection. Within the selected cases, synchronous multiple pituitary neuroendocrine tumors (PitNETs) account for 17.3%, PANCH cases (pituitary adenoma with neuronal choristoma) for 14.7%, PitNETs and posterior lobe tumors for 2.2%, PitNETs and metastases for 5.2%, PitNETs and mesenchymal tumors for 2.6%, PitNETs and cysts for 52.2%, and PitNETs and primary inflammation for 6.0%. The mean patient age was 53.8 years, with a standard deviation of 18.5 years. A total of 55.3% of the patients were female and 44.7% were male. From 1990 to 2018, there was a continuous increase in the number of multiple tumorous lesions. Conclusion From our studies, we conclude that considering possible tumorous double lesions during surgeries and in preoperative X-ray analyses is recommended.

An 84-year-old man presented with a frontal headache and easy bruising. He had a background history of a pituitary macroadenoma, diagnosed incidentally a year earlier. Investigations showed haemorrhage into the pituitary macroadenoma leading to a diagnosis of pituitary apoplexy in the context of low platelet count secondary to immune thrombocytopaenia. He was treated with intravenous hydrocortisone, platelet transfusion, intravenous immunoglobulin and high-dose steroid. Neurosurgical intervention was not indicated initially. Five days into his admission, he developed bilateral ptosis and ophthalmoplegia. MRI confirmed further haemorrhage associated with compression of the optic chiasm. He was transferred to a tertiary neurosurgical centre where he underwent urgent surgical decompression. To date, there has been minor improvement in his neurological symptoms. Management of this patient required considerable multidisciplinary teamwork between the clinics of endocrinology, haematology, neurosurgery, ophthalmology and geriatrics.

Malignant prolactinomas are very rare and are diagnosed when a prolactin-producing pituitary adenoma has metastasized. We report on a 54-year-old man with a history of macroprolactinoma transforming into a pituitary carcinoma secreting both prolactin and growth hormone with metastases to the stomach, bone, lungs, retroperitoneum, and kidney. Reviewing the literature, this case is the first reporting of a pituitary carcinoma with biopsy-proven paraneoplastic cast nephropathy. Symptoms and renal function improved following a course of palliative chemotherapy and radiotherapy. After 2 years, his disease progressed requiring further palliative treatment that was complicated by severe chest sepsis. He was not fit for further chemotherapy, receiving symptomatic relief in a hospice, and died soon after. The case highlights the importance of considering a patient's past medical history in the context of persistent unexplained renal impairment and systemic metastases when unexplained systemic symptoms and multi-organ involvement is present. The importance of renal biopsy for definitive diagnosis and before using potentially nephrotoxic chemotherapy is also highlighted. Renal diagnosis helped inform the decision to give chemotherapy, with the importance of this evidenced by an improvement in renal function following chemotherapy.

Prolactin (PRL)-secreting tumors are the most common functional pituitary adenomas. They usually respond to dopamine agonist (DA) treatment, with PRL normalization and adenoma shrinkage. Our aim was to characterize patients with prolactinoma resistant to DA treatment.This retrospective case series included patients diagnosed with DA-resistant prolactinomas between 1993-2017 in three medical centers. Resistance was defined as PRL levels above three times the upper limit of normal (ULN) despite a weekly dose of ≥2 mg cabergoline (CAB). Clinical and biochemical information, and response to treatment, were retrieved from medical records.Twenty-six patients were identified; 20 males. Of 25 macroadenomas, three were giant tumors (40 mm) and 15 (57.7%) were invasive. The mean age at diagnosis was 31.8 ± 14.9 years (range: 13-62). The median maximal CAB dose was 3.5 mg/week (IQR, 2.5-5). Half the patients received only CAB in escalating doses, nine received CAB and underwent transsphenoidal surgery, and four underwent surgery and radiotherapy in addition to CAB treatment. PRL levels at baseline between patients treated only with CAB and those operated were (91.6 [51.1-296.7] vs. 73.1 [22.6-170.9] XULN p = 0.355), and under maximal CAB dose PRL levels between patients treated only with CAB and those operated were similar (5.77 [1.27-11.27] vs 5.27 (2.9-26) XULN p = 0.317). At the last visit patients who received combined therapy achieved lower PRL levels than those treated with DA only (5.22 [1.7-21.6] vs 1.1 [0.44-3.99] XULN p = 0.017) PRL normalization was attained in seven patients and levels below 3 × ULN in fourteen patients; the overall response was 56%.Resistant prolactinomas usually require a multi-modal treatment strategy. We were able to control 14/25 (56%) of resistant tumors.

The prognostic value of optical coherence tomography (OCT) of the macular ganglion cell layer (mGGL) versus peripapillary retinal nerve fibre layers (pRNFL) following chiasmal decompression is unclear. This study is the largest comparison of the two parameters to date and aims to clarify how their performance as covariates compare in predictive models of long-term visual outcomes following pituitary or parasellar tumour surgical resection.This was a prospective, two-year, longitudinal cohort study in a single centre tertiary hospital setting. Participants with MRI evidence of pituitary or parasellar tumour compression of the optic chiasm who underwent surgical decompression, were enrolled. Associations between pre-operative OCT parameters and long-term visual outcomes were assessed using multivariable generalised linear mixed models and an age matched normative database.Final analysis included 216 eyes of 108 participants with a mean age (standard deviation) of 51.6 (17.04) years, of whom 58 (49%) were female. The superior inner mGCL was the best predictor of long-term visual field recovery, with an area under the curve of 0.90, a sensitivity of 80%, specificity of 88%, positive predictive value of 86%, and negative predictive value of 83%.mGCL performed better in predicting long-term visual field recovery post-pituitary or parasellar surgical resection. The superior inner mGCL was the best specific measure which may provide clinical utility in pre-operative counselling. In this study we clarify previously variable comparisons of mGCL and pRNFL parameters in post-operative predictive modelling.

To evaluate effect of first-line dopamine agonist (DA) therapy as an alternative to surgery for visual field defect (VFD) recovery in giant and macro-prolactinoma.In this retrospective study, 125 patients with giant and macro-prolactinoma, except those with a history of previous surgery or radiotherapy, were evaluated. Those who underwent visual field examinations using the Humphrey Visual Field analyser upon initial assessment and after treatment were included for analysis. Twelve patients with VFD were included. The effects of DA therapy on both VFD and tumor size were evaluated within the first three months.There were twelve patients analysed: three females and nine males, five giant and seven macroprolactinomas; eight patients received cabergoline (CAB) and four patients received bromocriptine (BRC). The mean adenoma diameter was 35±13mm (range 15-60), and the mean PRL level was 3,523ng/dL (range 312-11,703). Eight patients (67%) complained of blurred vision, while four patients (33%) reported no visual symptoms. After a median duration of three weeks, the VFD completely resolved in ten patients (83%) but only partially improved in two (17%). The mean initial doses of CAB and BRC that provided VFD improvement were 0.5±0.2mg/week and 6.3±1.4mg/day, respectively. After a mean duration of 2.2±0.9months, the mean decrease in adenoma size was 43.6±24.5% (range 10-95%).The use of DA as a first-line treatment for at least one month before deciding on surgery is recommended in giant and macro-prolactinomas with VFD. Surgery should be considered only in cases with DA resistance or persistent visual impairment despite medical therapy.

A 68-year-old Chinese man was found to have a lobular mass in the sphenoid sinus which extended to the clivus and the roof of the nasopharynx on a staging MRI scan performed for his high-grade parotid salivary duct carcinoma. Further positron emission tomography scan showed that this lesion was fluorodeoxyglucose (FDG) avid. This proved to be a diagnostic dilemma. The patient underwent a total parotidectomy, left selective neck dissection and a transphenoidal biopsy of his nasal lesion. Final histology revealed that this lesion was a synchronous ectopic sphenoid sinus pituitary adenoma (ESSPA). Initial differential diagnoses that were considered included a chordoma, metastatic carcinoma and nasopharyngeal carcinoma. However, an important differential with a neoplastic appearance and a tendency for positive FDG uptake is an ESSPA. It requires dedicated immunohistochemical staining to diagnose, and its mainstay of treatment is surgical excision.

Growth of some pituitary tumors is driven by hormones which vary in concentration along the lines of patient socioeconomic status. Thus, pituitary tumors may exhibit disparities in incidence upon stratification by socioeconomic variables. Exploring for these disparities could provide direction in tumor etiology elucidation and identification of healthcare inequalities.To investigate pituitary adenoma and carcinoma incidence (per 100,000) with respect to sex, age, income, residence, and race/ethnicity, we searched the largest American administrative dataset (1997-2016), the National (Nationwide) Inpatient Sample (NIS), which surveys 20% of United States (US) discharges.Annual national incidence was 2.80 for adenomas and 0.046 for carcinomas. For adenomas, males had an incidence of 2.63, similar (p = 0.17) to females at 2.78; likewise, for carcinomas, males had a statistically equivalent (p = 0.24) incidence at 0.051 to females at 0.041. Amongst age groups, for adenomas incidence progressively rose, peaking 65-84 years old (6.12), before declining. For adenomas and carcinomas respectively, patients with low income had an incidence of 2.66 and 0.044, similar (p = 0.11; p = 0.72) to the 3.01 and 0.041 of middle/high income patients. Incidence was greatest for adenomas amongst urban centers (3.47), followed by rural (3.16) and suburban (3.01) communities. Examining race/ethnicity (p = 0.0000016), for adenomas, incidences amongst Blacks, Asian/Pacific Islanders, Hispanics, and Whites were as follows, respectively: 3.64, 2.57, 2.54, 2.44. Annually, incidence for adenomas was increasing (τ = 0.63, p = 0.00021), but decreasing (τ = -0.60, p = 0.00085) for carcinomas. Specifically, for carcinomas incidence was only decreasing for females and the middle/high income.In the US, time-enduring healthcare disparities were identified for pituitary adenomas and carcinomas, against the background of sociodemographic strata. For carcinomas, annual incidence was declining only for middle/high income patients and females, which supporting prior investigations that low income patients and males are experiencing barriers to definitive treatment for pituitary adenomas. Incidence was also found to be greatest Blacks and urban residents.

To describe cases diagnosed with pituitary macroadenoma during the follow-up of their primary ocular pathologies.Charts of patients followed in the Centre for Ophthalmic Specialized Care for diverse pathologies and who had subsequently developed a pituitary macroadenoma were retrieved. The primary pathologies were noted. The delay of the diagnosis after sufficiently compatible perimetric signs became available was calculated and the evolution after the neurosurgical intervention was reported.In total, from 2003 to 2020, 16/14 966 (0.1%) pituitary macroadenoma patients were recorded. In 10 patients, the disease was noted in their history. In 6 patients (2 females, 4 males; 0.04%), macroadenoma occurred during the follow-up for their primary ocular pathologies. Mean age at first presentation was 65.16 ± 8.52 years. Primary pathologies included amblyopia (1), glaucoma (2), cataract (4), and uveitis (2). Mean duration of symptoms was 18.17 ± 13.11 months. Mean delay from first suspicious visual field signs to diagnosis was 125 ± 207.93 days. All patients underwent one surgical treatment with or without radiotherapy except one where a second intervention was required. All patients have seen their visual field improve after surgical intervention. Mean preoperative Mean defect (MD) was 13.43 ± 8.68 dB OD and 13.4 ± 5.07 dB OS. Mean postoperative MD was 8.2 ± 10.27 dB OD and 5.42 ± 4.12 dB OS.Pituitary macroadenomas are prone to be missed or diagnosed with delay when ophthalmic patients are already followed for another pathology that prevents the clinician from diligently evoking the diagnosis. Despite profound visual field loss, visual recovery was almost complete in 4/6 patients, indicating that even diagnostic delay did not preclude recovery in our series.Beschreibung von Fällen mit Hypophysen-Makroadenom während des Follow-ups ihrer primären okulären Pathologien diagnostiziert.Im „Centre for Ophthalmic Specialised Care“ (COS) wurden Patientenakten analysiert von Fällen, die für verschiedene Pathologien verfolgt wurden und die sekundär zu ihrer Basispathologie ein Makroadenom der Hypophyse entwickelten. Die primären Pathologien wurden festgestellt. Die Verzögerung der Diagnose nach Vorliegen hinreichend kompatibler perimetrischer Zeichen wurde berechnet und die Entwicklung nach dem neurochirurgischen Eingriff berichtet.Insgesamt wurden von 2003 bis 2020 16/14 966 (0,1%) Hypophysen-Makroadenom-Patienten registriert. Bei 10 Patienten wurde die Krankheit in ihrer Anamnese festgestellt. Bei 6 Patienten (2 Frauen, 4 Männer; 0,04%) traten Makroadenome während der Nachbeobachtung für ihre primären Augenpathologien auf. Das mittlere Alter bei der ersten Präsentation betrug 65,16 ± 8,52 Jahre. Zu den primären Pathologien gehörten Amblyopie (1) Glaukom (2) Katarakt (4) und Uveitis (2). Die mittlere Dauer der Symptome betrug 18,17 ± 13,11 Monate. Die mittlere Verzögerung vom ersten verdächtigen Gesichtsfeld bis zur Diagnose betrug 125 ± 207,93 Tage. Alle Patienten wurden einer einzigen chirurgischen Behandlung mit oder ohne Strahlentherapie unterzogen, mit Ausnahme einer 2. Behandlung bei einem Patienten. Alle Patienten haben gesehen, wie sich ihr Gesichtsfeld nach dem chirurgischen Eingriff verbesserte. Der mittlere präoperative Mean Defect (MD) betrug 13,43 ± 8,68 dB OD und 13,4 ± 5,07 dB OS. Der mittlere postoperative MD betrug 8,2 ± 10,27 dB OD und 5,42 ± 4,12 dB OS.Hypophysen-Makroadenome neigen dazu, verpasst oder mit Verzögerung diagnostiziert zu werden, wenn Augenpatienten bereits für eine andere Pathologie behandelt und nachbeobachtet werden. Trotz tiefgreifender Gesichtsfeldausfälle erholte sich das Sehvermögen bei 4/6 Patienten fast vollständig, was darauf hindeutet, dass selbst eine verzögerte Diagnose in unserer Serie eine Erholung nicht ausschloss.

The epidemiology of male hypogonadism has been understudied. Of the known causes of endogenous androgen deficiency, only Klinefelter syndrome is common with a likely population prevalence of > 5:10,000 men (possibly as high as 10–25:10,000). Mild traumatic injury might also be a common cause of androgen deficiency (prevalence 5–10:10,000 men), but large, long-term studies must be completed to confirm this prevalence estimation that might be too high. The classic causes of male androgen deficiency—hyperprolactinemia, pituitary macroadenoma, endogenous Cushing syndrome and iron overload syndrome are rare (prevalence < 10,000 men). Of the iatrogenic causes of male androgen deficiency, androgen deprivation therapy for prostate cancer, radiation and chemotherapy for testicular cancer, lymphoma and leukemia, and radiation therapy for primary brain tumors and head and neck cancers are common (prevalence > 5:10,000 men).

Male prolactinoma treatment by dopamine agonists (DA) restores sexual function. However, excessive DA dose can lead to impulse control disorder.The aim of this retrospective study was to determine the level of testosterone that eliminates symptoms and provides fertility in male macroprolactinoma, without causing these adverse effects.Twenty-seven male patients with macroprolactinoma were included. There were 16 macro (≥1-2.8cm), 7 large macro (≥2.9-3.9cm) and 4 giant (≥4cm) adenomas. Prolactin (PRL) and testosterone (T) levels were evaluated. A timeline was created to analyze improvement in symptoms of hypogonadism and infertility. Testosterone levels were compared with age-matched controls.Mean PRL, basal tumor diameter and shrinkage were 2846±3415ng/mL, 27.2±10.2mm and 63.4%, respectively. Basal T levels were 1.6±1.0ng/mL for patients and 4.4±1.5ng/mL for controls (P0.001). Mean T level in the asymptomatic period was significantly lower than in controls (3.2±0.4ng/mL vs. 4.4±1.5ng/mL, respectively; P=0.002), while mean PRL was 27.2ng/mL. Fertility was achieved in 6 of the patients seeking fertility, and there was no difference in T level between these patients and controls (3.7±0.8ng/mL and 4.4±1.5ng/mL, respectively; P=0.14); when fertility was achieved, mean PRL was 26.9±23ng/mL.Patients should be carefully questioned regarding complaints at each consultation, and DA dose should not be increased unnecessarily, to avoid possible serious adverse effects.

Endoscopic techniques have been widely applied for challenging cranial base surgeries in recent years. In this study, we evaluated the safety and efficacy of using the endoscopic endo-nasal route for various skull base pathologies in terms of postoperative complications.A total of 584 consecutive patients who underwent endoscopic skull base surgery were studied. Peri- and post-operative complications and risk factors affecting the occurrence of these complications were evaluated.648 endoscopic skull base surgical procedures were performed on 584 patients (47.8% females and 52.2% males) with the mean age of 41.2 years. Pituitary adenoma (69.3%) was the most common pathology. Post-operative mortality was 2.0%. The rates of post-operative permanent neurological deficit (one case of 6th nerve injury, two 12th nerve injuries and one hemiparesis) and visual deterioration were 0.6% and 1.5%, respectively. Ten patients (1.7%) were complicated with meningitis and it was the cause of death in 3. Systemic complications not directly attributable to skull base surgical access occurred in 2% (11 patients) with 5 mortalities. The rate of intra-operative vascular injury was 1% and among them one patient died due to PCA injury. The most common post-operative complications were diabetes insipidus (12.5%), anterior pituitary dysfunction (10.6%) and CSF leak (3.6%), respectively. In general, reoperation, malignant lesions, and level IV of surgical complexity were associated with a higher incidence of complications.Endoscopic endo-nasal approach can be a safe and less-morbid first-line treatment of patients with various skull base lesions.

BACKGROUND: The association between alcohol intake and incidence of pituitary adenoma has not been reported previously. We examined this association in three large, prospective cohort studies. METHODS: Using data from the Nurses’ Health Study (NHS), Nurses’ Health Study II (NHSII), and Health Professionals Follow-Up Study (HPFS), we computed multivariable-adjusted hazard ratios (MVHR) and 95% confidence intervals (CI) for pituitary adenoma by levels of alcohol intake using Cox proportional hazards regression. RESULTS: We identified 292 incident cases of pituitary adenoma (225 among women, 67 among men) among 235,973 participants with 6,548,732 person-years of follow up. Compared with intake of ≤0.5 grams/day, cumulative average alcohol intake in all categories was associated with reduced risk of pituitary adenoma (MVHR=0.60, 95%CI:0.43-0.83 for 0.5-≤2 g/d, MVHR=0.57, 95%CI:0.41-0.79 for >2.0-≤8.0, MVHR=0.70, 95%CI:0.47-1.04 for >8.0-≤15.0, and MVHR=0.51, 95%CI:0.32-0.83 for >15.0 g/d). Significant inverse findings were present in women and were similar but non-significant in men. For specific alcoholic beverages, inverse associations were statistically significant for total wine (MVHR=0.58, 95%CI:0.43-0.79 comparing 0.5-≤2 to ≤0.5 g/d), red wine (MVHR=0.65, 95%CI:0.46-0.92 comparing 0.5-≤2 to ≤0.5 g/d), and white wine (MVHR=0.72, 95%CI:0.53-0.97 comparing 0.5-≤2 to ≤0.5 g/d). Results were consistent using baseline intake, recent intake, and with an 8 year lag. CONCLUSION: In three prospective cohorts, compared to almost no consumption, alcohol consumption was associated with reduced risk of pituitary adenoma. Sensitivity analyses suggest that these results are unlikely to be the result of reverse causation or diagnostic bias.

Background and Objective The literature on the use of intraoperative computed tomography (iCT) is sparse. We provide our experience of the usefulness of iCT in extent of resection in large and giant pituitary adenomas. Methods A retrospective review was performed of cases using the endonasal endoscopic technique in which iCT was used. Demographic factors, number of scans, and impact on the extent of resection are reported, with visual acuity and field changes. Tumors were graded according to the Hardy classification. Patients with cavernous sinus invasion were excluded. Results All patients received a perioperative computed tomography scan with our iCT scanner. Thirty patients are reported, including 14 large and 16 giant pituitary adenomas, including 14 nonfunctional and 16 growth hormone–secreting tumors. The overall gross total resection (GTR), near-total resection, and subtotal resection rates were 83.3%, 16.7%, and 3.3%, respectively. iCT scanning detected residual in 13 of 30 patients, including 4 with 14 large (29%) and 9 with 16 (56.3%) giant adenomas promoting further surgery. iCT use improved GTR from 43.8% to 81.3% in giant adenomas and from 71% to 86% in large adenomas. Of the 13 patients in whom iCT detected residual disease, none required >2 iCT scans. No intraoperative complications were observed. Conclusions iCT can improve extent of resection in large and giant pituitary adenomas and facilitate maximum safe resection such as GTR or near-total resection in patients where such should be attempted. iCT use may reduce iatrogenic complications and has select financial benefits in our patients' socioeconomic demographics. However, further prospective controlled studies are required to affirm our conclusions.

This case report concerns a 31-year-old male with an aggressive pituitary tumor who presented initially with bitemporal hemianopsia and slightly elevated prolactin. On magnetic resonance imaging of the brain, there was a sellar mass with parasellar invasion to the lateral aspects of the internal carotid arteries, compressing the optic chiasm. On histopathological analysis, the diagnosis was made of a densely granulated lactotroph pituitary tumor with a Ki67 proliferation rate of 15%, a mitotic count of 6/10 high-power fields, and p53 positivity. Based on these features, the tumor was classified as a grade 2b tumor according to the Trouillas classification, and a more aggressive behavior of the tumor could be expected. In order to anticipate a future need for alternative drug treatments, the following analyses were undertaken: MGMT methylation (present) as well as the expression of estrogen receptor (negative), programmed-death ligand 1 (60 - 70% positive tumor cells), vascular endothelial growth factor-A and somatostatin receptor 2 (both positive). There was regrowth of residual tumor tissue, and the treatment consisted thus far of repeat surgery, cabergoline, pasireotide, and radiotherapy. Chemotherapy with temozolomide could not yet be initiated due to a concurrent infertility treatment. This case is unique because the tumor displays atypical characteristics, both in terms of morphology and behavior. It also illustrates how pathologists can play an important role in determining the diagnosis, prognosis, and possibilities for targeted therapy.

Context Endocrine complications are common in pediatric brain tumor patients. Objective We aimed to describe the endocrine follow-up of patients with primary brain tumors. Methods This is a noninterventional observational study based on data collection from medical records of 221 patients followed at a Pediatric Endocrinology Department. Results Median age at diagnosis was 6.7 years (range, 0-15.9), median follow-up 6.7 years (0.3-26.6), 48.9% female. Main tumor types were medulloblastoma (37.6%), craniopharyngioma (29.0%), and glioma (20.4%). By anatomic location, 48% were suprasellar (SS) and 52% non-suprasellar (NSS). Growth hormone deficiency (GHD) prevalence was similar in both groups (SS: 83.0%, NSS: 76.5%; P = 0.338), appearing at median 1.8 years (−0.8 to 12.4) after diagnosis; postradiotherapy GHD appeared median 1.6 years after radiotherapy (0.2-10.7). Hypothyroidism was more prevalent in SS (76.4%), than NSS (33.9%) (P < 0.001), as well as ACTH deficiency (SS: 69.8%, NSS: 6.1%; P < 0.001). Early puberty was similar in SS (16%) and NSS (12.2%). Hypogonadotropic hypogonadism was predominant in SS (63.1%) vs NSS (1.3%), P < 0.001, and postchemotherapy gonadal toxicity in NSS (29.6%) vs SS (2.8%), P < 0.001. Adult height was lower for NSS compared to target height (−1.0 SD, P < 0.0001) and to SS patients (P < 0.0001). Thyroid nodules were found in 13/45 patients (28.8%), including 4 cancers (4.8-11.5 years after radiotherapy). Last follow-up visit BMI was higher in both groups (P = 0.0001), and obesity incidence was higher for SS (46.2%) than NSS (17.4%). Conclusion We found a high incidence of early-onset endocrine disorders. An endocrine consultation and nutritional evaluation should be mandatory for all patients with a brain tumor, especially when the tumor is suprasellar or after hypothalamus/pituitary irradiation.

OBJECTIVE Youth with craniopharyngioma experience weight gain, fragmented sleep, excessive daytime sleepiness (EDS), fatigue, and psychosocial problems that negatively impact their overall health-related quality of life (HRQoL). Greater hypothalamic tumor involvement (HI) may be associated with higher rates or severity of these impairments; however, the direct and indirect impact of HI on the physical and psychosocial consequences associated with pediatric craniopharyngioma remain unclear. The purpose of the current study was to examine relations between HI, body mass index (BMI), fragmented sleep, EDS, fatigue, psychosocial problems, and HRQoL among youth with craniopharyngioma. METHODS Eighty-four youth with craniopharyngioma (Mage = 10.27 ± 4.3 years, 53.6% female, 64.3% White) were assessed with actigraphy, nocturnal polysomnography, and multiple sleep latency tests prior to proton therapy, when indicated. Caregivers completed measures of fatigue, psychosocial functioning, and HRQoL. RESULTS Hypothalamic tumor involvement was associated with greater BMI (Est. = 2.97, p = 0.003) and daytime sleepiness (Est. = 2.53, p = 0.01). Greater fatigue predicted more psychosocial problems (Est. = 0.29, p

Objective Improving the gross total resection (GTR) rate of suprasellar pituitary macroadenomas (SPMAs) using the pure endoscopic endonasal transsphenoidal approach (EETA) has been a long-standing focus of neurosurgeons. This study was aimed at evaluating the influences of the removal of the tuberculum sellae bone (TSB) without opening the dura of the tuberculum sellae on the GTR rate of SPMAs via the EETA. Methods We retrospectively analyzed medical reports of patients with SPMAs who underwent EETA between February 2015 and November 2020. Data on clinical manifestations, endocrinologic types, imaging features (Hardy classification, morphology, and texture), clinical outcomes, and TSB removal status were collected. All patients were followed up for 6 months postoperatively. Results Seventy-eight patients were enrolled in our study. The GTR rates of the TSB removal group (45/78, 57.7%) and nonremoval group (33/78, 42.3%) were 80.0% (36/45) and 57.6% (19/33), respectively. Univariate logistic regression analysis found that the removal of TSB, rounded morphology, and low Hardy classification were correlated with higher GTR rates. Multiple logistic regression analysis indicated that even after adjusting for tumor types and imaging features, the removal of TSB had an independent effect on the GTR rate (odds ratio, 7.6; 95% confidence interval, 1.8–31.6; P = 0.005). The incidence rates of postoperative cerebrospinal fluid leakage and diabetes insipidus were not significantly different between the TSB removal group and TSB nonremoval group. Conclusions TSB removal using EETA without opening the tuberculum sellae dura improves the GTR rate of SPMAs without increasing the incidence of postoperative complications.

Resume La remission spontanee de la maladie de Cushing est un evenement extremement rare. Nous decrivons un cas representatif et proposons une revue systematique des cas precedemment rapportes dans la litterature. Presentation de cas : une femme de 51 ans diagnostiquee avec la maladie de Cushing a subi un traitement unique avec de la metyrapone pendant neuf mois. Deux mois apres l’arret du traitement, elle a ete admise pour une insuffisance renale aigue. Apres encore quatre mois, en juin 2020, il n’y avait aucun signe d’hypercortisolisme, que ce soit cliniquement ou biochimiquement, ou d’hypocortisolisme, chez notre patient. Un an plus tard, elle est toujours en remission. Cas rapportes dans la litterature : sur 23 patients (dont notre cas), 87 % etaient des femmes avec un âge median de 32 ans. Parmi ceux qui avaient des tumeurs radiologiquement visibles, 10 patients avaient des microadenomes (44 %), tandis que 7 patients avaient des macroadenomes (30 %). Le delai moyen entre le diagnostic et la remission spontanee etait de 5 mois, plus court dans les macroadenomes (1 mois) que dans les microadenomes (13,5 mois). Avant la remission spontanee, les traitements etaient : l’absence de traitement (65 %), les inhibiteurs steroidiens (22 %), la surrenalectomie bilaterale et l’autotransplantation surrenale (5 %), la surrenalectomie bilaterale partielle (4 %), la chirurgie hypophysaire incomplete (4 %). L’apoplexie tumorale hypophysaire etait l’evenement incrimine le plus frequent (91 %), documente radiologiquement chez 43 % des patients. La periode moyenne de remission au cours du suivi etait de 28 mois, les remissions allant de 6 a 130 mois. Une recidive de la maladie de Cushing est survenue chez 39 % (n = 9) des patients. Bien que plusieurs mecanismes responsables de ce phenomene aient ete proposes, l’apoplexie tumorale hypophysaire cliniquement manifeste ou infra-clinique, cette derniere se presentant parfois de maniere atypique, semble etre l’evenement incrimine le plus frequent. Les medecins doivent en etre conscients, et un suivi regulier est obligatoire en raison de son imprevisibilite.

Supplemental digital content is available in the text., Purpose This study aimed to investigate the value of magnetic resonance (MR) characteristics in differentiating the subtypes of growth hormone pituitary adenomas. Materials and Methods The clinical and MR imaging data of 70 patients with growth hormone pituitary adenoma confirmed by surgery and pathology were retrospectively analyzed. The tumors were divided into dense granular (DG; 36 cases) and sparse granular subtypes (SG; 34 cases). The tumors' MR features were analyzed, including the mean and maximum diameters, T2 signal intensity, T2 relative signal intensity (rSI), homogeneity, enhancement degree, and invasiveness (Knosp grade). Mann-Whitney U test and χ2 test were used to analyze MR characteristics between the 2 groups. The independent predictors and predictive probabilities of tumor subtypes were obtained via a logistic regression model, and the efficacy was compared by receiver operating characteristic curve. Results The mean and maximum diameters of growth hormone adenoma in DG and SG were 1.77 versus 2.45 and 1.95 versus 3.00 cm (median, P < 0.05), respectively. There was a significant difference between the 2 groups in T2 signal intensity and rSI (P values were 0.02 and 0.001, respectively). Most DG adenomas (86.1%) appeared as hypointense on T2 images, and 38.2% of SG adenomas were hyperintense. There was no significant difference in tumor homogeneity (P = 0.622). A significant difference was found in the Knosp grade between the 2 subtypes (P = 0.004). In addition, the enhancement degree of SG adenomas was significantly higher than that of DG adenomas (P = 0.001). Logistic regression analysis showed that high T2 rSI value and marked contrast enhancement were independent predictors of the 2 subtypes, and the odds ratios were 4.811 and 4.649, respectively. The multivariate logistic model obtained relatively high predicting efficacy, and the area under the curve, sensitivity, and specificity were 0.765, 0.882, and 0.500, respectively. Conclusions There are significant differences in tumor size, T2 signal intensity, T2 rSI, enhancement degree, and invasiveness between DG and SG adenomas. The logistic model based on the marked contrast enhancement and high T2 rSI value has an important value in predicting the subtype of growth hormone adenoma.

Background This report describes the case of a patient whose pituitary microadenoma resolved after he contracted coronavirus disease 2019. To our knowledge, this is one of the first reported cases of pituitary tumor resolution due to viral illness. We present this case to further investigate the relationship between inflammatory response and tumor remission. Case presentation A 32-year-old man in Yemen presented to the hospital with fever, low blood oxygen saturation, and shortness of breath. The patient was diagnosed with coronavirus disease 2019. Past medical history included pituitary microadenoma that was diagnosed using magnetic resonance imaging and secondary adrenal insufficiency, which was treated with steroids. Due to the severity of coronavirus disease 2019, he was treated with steroids and supportive care. Three months after his initial presentation to the hospital, brain magnetic resonance imaging was performed and compared with past scans. Magnetic resonance imaging revealed changes in the microadenoma, including the disappearance of the hypointense lesion and hyperintense enhancement observed on the previous scan. Conclusions Pituitary adenomas rarely undergo spontaneous resolution. Therefore, we hypothesized that tumor resolution was secondary to an immune response to coronavirus disease 2019.