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(1) Background: Pituitary adenomas are benign tumors comprising about 18% of all intracranial tumors, and they often require surgical intervention. Differentiating pituitary tissue from adenoma during surgery is crucial to minimize complications. We hypothesized that using ICG dye would reduce the hormonal complication rates. (2) Methods: A prospective randomized study (February 2019–October 2023) included 34 patients with non-functional macroadenomas of the pituitary gland randomly assigned to receive intraoperative ICG or be in the control group. All underwent endoscopic endonasal transsphenoidal surgery. Pituitary function was assessed preoperatively, immediately postoperatively, and 3–6 months postoperatively. Adenohypophysis function was evaluated with hormonal tests (Cosyntropin stimulation test, TSH, fT3, fT4, prolactin, IGF-1, FSH, LH, and testosterone in men) and neurohypophysis function with fluid balance, plasma and urine osmolality, and serum and urinary sodium. (3) Results: Of the 34 patients (23 men, 11 women; average age 60.9 years), 5.9% in the ICG group developed diabetes insipidus postoperatively, compared to 23.5% in the control group. Adenohypophysis function worsened in 52.9% of the ICG group and in 35.3% of the control group. (4) Conclusions: Our study did not confirm the benefits of using ICG in these surgeries. Further research with a larger sample is needed. [ABSTRACT FROM AUTHOR]

The caudal neurosecretory system (CNSS) of teleost is intricately linked to reproduction. This study sought to explore the association between the CNSS and the reproductive cycle of Pampus argenteus. Utilizing microscopy and transmission electron microscopy, we observed the morphological alterations within the CNSS. Furthermore, we quantified the levels of urotensin-I (U-I), urotensin-II (U-II), cortisol, and corticotropin-releasing hormone (CRH) throughout the reproductive process. Findings revealed that Dahlgren cells, distributed along the spinal cord adjacent to the terminal 6 vertebrae of the silver pomfret's tail, were primarily concentrated within the final 3 vertebrae. The neurohypophysis was characterized by an elongated oval form when viewed laterally and an almost spherical configuration in ventral perspective. Postbreeding season, the average diameters of Type I and Type II Dahlgren cells decreased by 21.8% and 15.44%, respectively (P < 0.05), accompanied by a reduction in neurosecretory granules to merely 60% of their initial prereproductive state (P < 0.05). Variations in the serum and gonadal concentrations of U-I, U-II, cortisol, and CRH indicated a significant correlation with gonadal maturation. In summary, the CNSS plays a crucial role in gonadal development, directly or indirectly influencing and modulating the reproductive activities of P. argenteus. [ABSTRACT FROM AUTHOR]

We report the case of a 61-year-old male with spindle cell oncocytoma of the hypophysis. On presentation to the Department of Neurosurgery at the German Armed Forces Hospital of Ulm, the patient reported a history of several years of left sixth nerve palsy, right ptosis, increased sensitivity to light, and a bilateral retrobulbar pressure sensation. Pituitary function was normal. A chromophobe non-functioning pituitary adenoma was initially suspected. The diagnosis was established on the basis of examination at a histopathology reference laboratory using immunohistochemistry to identify cell surface markers. During two years of follow-up, there were two clinical recurrences requiring surgery. To our knowledge, this is the 35th documented case of spindle cell oncocytoma of the pituitary gland and the first that was immunohistochemically negative for epithelial membrane antigen (EMA) and S100; and the first that displayed haematogenous metastasis to the right sphenoparietal sinus. The three surgical procedures were associated with massive intraoperative bleeding and thus resulted in subtotal tumor resection. Following surgery for the recurrences, the patient underwent radiotherapy. [ABSTRACT FROM AUTHOR]

Pituicytoma is a rare benign tumor that arises from pituicytes in the infundibulum and posterior pituitary gland. Pituicytomas are rarely encountered in clinical practice and have no characteristic radiological features and, thus, can be misdiagnosed as more commonly encountered lesions such as pituitary adenoma, meningioma, and craniopharyngioma. Clinically, patients can present with hormonal disturbances and headaches. However, visual field defects are the most commonly reported clinical complaint. Here, we report a 42‑year‑old male who presented with symptoms suggestive of hypogonadism for the past 2 years. Upon clinical assessment, the patient’s ophthalmic examination was unremarkable, and hormonal evaluation revealed low testosterone levels. Pituitary magnetic resonance imaging (MRI) showed a suprasellar homogeneous enhancing mass causing chiasmatic displacement. Elective transnasal transsphenoidal resection was performed. Postoperatively, the patient developed diabetes insipidus, and was managed accordingly. Histopathology showed patterns consistent with pituicytoma, one of the uncommon intracranial tumors. Total resection remains the goal of treatment; however, this is not easily achievable because of the high vascularity of the tumor and its adhesive nature to adjacent structures. The diagnosis of pituicytoma is established by histopathology and immunohistochemistry. Clinical and radiological follow-up is advisable, especially with subtotal resection, due to the likelihood of recurrence. [ABSTRACT FROM AUTHOR]

Objective: We aimed to investigate the magnetic resonance imaging (MRI) findings and clinical significance of position and changes in morphology of the pituitary stalk following pituitary adenoma (PA) resection using a transsphenoidal approach. Methods: We collected clinical and MRI data of 108 patients with PA after transsphenoidal surgery. Diameter, length, and coronal deviation of the pituitary stalk were measured pre-, post-, and mid-term post-operatively, to observe pituitary stalk morphology. Results: Of 108 patients, 53 pituitary stalks were recognisable pre-operatively. The angle between the pituitary stalk and the median line was 7.22◦-50.20◦ (average, 25.85◦ ) in 22 patients with left-sided pituitary stalks and 5.32◦-64.05◦ (average, 21.63◦ ) in 20 patients with right-sided pituitary stalks. Of 42 patients with preoperative pituitary stalk deviation, 41 had an early postoperative recovery and 1 had increased deviation. In the mid-term postoperative period, 21 of 42 patients had pituitary stalks located centrally. In 53 patients, the pituitary stalk length was 1.41–11.74 mm (mean, 6.12 mm) pre-operatively, 3.61–11.63 mm (mean, 6.93 mm) in the early postoperative period, and 5.37–17.57 mm (mean, 8.83 mm) in the mid-term postoperative period. In the early postoperative period, 58 (53.70%) patients had posterior pituitary bright spots (PPBS) and 28 (25.92%) had diabetes insipidus (DI). Conclusion: Pre-operatively, the pituitary stalk was compressed and thinned. Post-operatively, it could be stretched to a “normal state”, and its position showed a gradual centring trend. Post-operatively, the length of the pituitary stalk gradually increased. The PPBS in the early postoperative period negatively correlated with postoperative DI. [ABSTRACT FROM AUTHOR]

Attention has recently been focused on central nervous system neuropeptides as potential mediators of the symptom profile of obsessive–compulsive disorder (OCD). OCD includes a range of cognitive and behavioral symptoms that bear some relationship to dimensions of behavior associated with oxytocin (OT). Increased cerebrospinal fluid levels of the anxiolytic neuropeptide OT have been reported in OCD. OT is a neurosecretory nonapeptide synthesized in hypothalamic cells, which project to widely distributed sites in the central nervous system as well as the neurohypophysis. Central OT affects a variety of cognitive, grooming, affiliative, sexual, and reproductive behaviors in animals. OT is associated with the regulation of complex sociocognitive processes such as attachment, social exploration, social recognition, anxiety, and other stress-related behaviors. Based on these data, we hypothesized that OCD is mediated by OT. The aim of this review is to define possible involvements of OT in the pathophysiology of OCD. [ABSTRACT FROM AUTHOR]

Between 1996 and 2020, 12,565 cases were enrolled in the German Registry of Pituitary Tumors including 10,084 PitNETs (10,067 adenomas and 19 carcinomas obtained surgically and 193 adenomas diagnosed at autopsy) as well as 69 spindle cell tumors of the neurohypophysis (64 surgical specimens and 5 autopsies). In six patients (1 post mortem and 5 surgical specimens), PitNETs as well as posterior lobe tumors were found in the specimens. Two of the PitNETs were sparsely granulated prolactin-producing tumors, combined in one case with a granular cell tumor and in one case with a pituicytoma. One of the PitNETs revealed that autopsy was a sparsely granulated GH tumor combined with a neurohypophyseal granular cell tumor. Two PitNETs were null cell adenomas combined with a pituicytoma and a spindle cell oncocytoma, respectively. Further, one Crooke cell tumor was combined with a spindle cell oncocytoma. In five cases, the PitNETs were larger than the posterior lobe tumors and accounted for the clinical symptoms. Previously, four cases of co-existing pituitary anterior and posterior lobe tumors were described in the literature, comprising two ACTH PitNETs, one gonadotrophic PitNET and one null cell PitNET, each in combination with a pituicytoma. PitNETs and concomitant granular cell tumor or spindle cell oncocytoma, as observed in our cohort, have not been reported before. [ABSTRACT FROM AUTHOR]

Magnocellular neurosecretory cells that release vasopressin (MNCVP) from axon terminals in the neurohypophysis display a unique pattern of action potential firing termed phasic firing. Under basal conditions, only a small proportion of MNCVP display spontaneous phasic firing. However, acute and chronic conditions that stimulate vasopressin release, such as hemorrhage and dehydration, greatly enhance the number of MNCVP that fire phasically. Phasic firing optimizes VP neurosecretion at axon terminals by allowing action potential broadening to promote calcium‐dependent frequency‐facilitation, at the same time as preventing the secretory fatigue caused by spike inactivation that occurs during prolonged continuous stimulation. This review provides an update on our mechanistic understanding of these processes and highlights important gaps in our knowledge that must be addressed in future experiments. [ABSTRACT FROM AUTHOR]

Background: Pituicytoma is an extremely rare low-grade glial tumor that is closely related to the neurohypophysis axis. Most studies of pituicytomas include only several cases. To better understand this disease, we reviewed a series of cases of pituicytomas. The diagnosis and treatment of pituicytoma must be further elucidated. Methods: Eleven patients with pituicytoma admitted to Beijing Tiantan Hospital from 2012 to 2019 were selected. The clinical features, including radiological and histological examination, surgical records and prognosis were reviewed. Sixty-eight other previously published cases of pituicytoma also were used to analyze the predictive factors for the results. The Cox regression model was used for univariate and multivariate analyses. Results: Our patients included 5 males (45.5%) and 6 females (54.5%), with a mean age of 49.3 years. The tumor was located in the suprasellar region in 5 patients (45.5%), intrasellar region in 4 patients (36.4%), and intrasellar-suprasellar region in 2 patients (18.2%). All patients were misdiagnosed with other common tumors in the sellar region before the operation. During the operation, gross total resection (GTR) of the tumor was achieved in 6 patients (54.5%), and subtotal resection (STR) was achieved in 5 patients (45.5%). The mean progression-free survival (PFS) time was 29.82 months. Tumor progression after surgical resection occurred in 4 patients (36.4%). Among them, 60.0% of the patients (cases 4, 5, 7) with STR experienced progression, while 16.7% of the patients (case 2) with GTR experienced progression. Combined with the 68 cases in the literature, GTR was an independent risk factor for PFS time (P < 0.05). Conclusions: Pituicytomas are more common in middle-aged people and the sellar region. The clinical manifestations of pituicytomas are different, but no diagnostic clinical features have been identified other than an abnormally abundant blood supply. Currently, GTR is the best approach for the treatment of pituicytomas. More patients and longer follow-up periods were needed to further elucidate the biological features of pituicytomas. [ABSTRACT FROM AUTHOR]

Introduction: Spindle cell oncocytoma (SCO) is an extremely rare sellar neoplasm. No observational studies have been reported so far to investigate the prognostic factors of this tumor entity. This systematic review aimed to elucidate the risk factors for tumor recurrence/progression of SCO. Methods: We searched for relevant articles in PubMed and Web of Science. Studies providing individual patient data with follow-up information of SCO cases were included. Pearson's Chi square and Fisher's exact test were used for categorical variables while t test or Mann–Whitney tests were applied for continuous variables, if applicable. We used the Cox regression model to assess the effects of suspected variables on progression-free survival (PFS). Results: A total of 38 case reports and case series comprising of 67 SCOs were included for final analyses. Recurrent/progressive tumors were noted in 38.8% of cases. Among the clinicopathological factors, only the extent of surgery was a significant risk factor for tumor recurrence/progression. SCO patients with a subtotal resection had a significantly higher risk for tumor relapse in comparison with complete removal (HR 7.51; 95% CI 1.75–32.31; p = 0.007). Conclusion: Our study demonstrated the characteristic clinicopathological features of SCOs with a high recurrence/progression rate and outlined the predictor for tumor relapse. Failure to achieve gross total resection is the only risk factor for tumor recurrence/progression. [ABSTRACT FROM AUTHOR]

Copyright of Revista de la Facultad de Medicina de la UNAM is the property of UNAM, Facultad de Medicina and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Childhood-onset lymphocytic infundibuloneurohypophysitis (LINH) due to infiltration of autoimmune lymphocyte in the neurohypophysis is rarely reported. Its definitive diagnosis requires a pituitary biopsy, which is an invasive procedure. Recently, anti-rabphilin-3A antibody has been reported as a potential diagnostic marker for LINH in adults; however, only a few cases have been reported in children. Here, we present a case of childhood-onset LINH in a 10-yr-old boy identified as anti-rabphilin-3A antibody positive during chronic phase, 9 yr post-onset of central diabetes insipidus (CDI). T1-weighted magnetic resonance imaging (MRI) revealed pituitary stalk thickening and absence of posterior pituitary bright signal spot, and the hormonal responses of the adenohypophysis to GHRH, TRH, CRH, and LHRH revealed no abnormalities during the first admission. MRI at 5 mo post-onset indicated reduced stalk swelling; however, replacement treatment with intranasal desmopressin was continued to counter unimproved CDI. Additionally, GH replacement therapy was also initiated to counter its deficiency. Pituitary re-enlargement was not observed in the subsequent routine MRI, and no increase was observed in the levels of tumor markers during follow-up, which was considered clinically consistent with LINH. Our case study suggests that anti-rabphilin-3A antibody may be considered as a useful diagnostic marker for LINH in children. [ABSTRACT FROM AUTHOR]

Background: Granular cell tumor (GCT) of the sellar region is a rare tumor of the sellar and suprasellar regions that originate from the neurohypophysis. This tumor is very difficult to differentiate from other pituitary neoplasms, such as pituitary adenoma, pituicytoma, and spindle cell oncocytoma. We report a rare case of GCT arising from the posterior pituitary of the sellar region and suggest a useful indicator for accurate diagnosis and pitfalls for surgical procedures. Case Description: A 42-year-old woman was admitted to our hospital with bitemporal hemianopsia. Neuroimaging showed a large pituitary tumor in the sellar and suprasellar regions with a hypointense part on T2-weighted magnetic resonance imaging, and the enhanced anterior pituitary gland was displaced anteriorly. Laboratory findings showed mild hyperprolactinemia. Subtotal resection of the tumor was achieved using an endoscopic endonasal transsphenoidal approach. Histological findings showed round or polygonal cells with abundant granular eosinophilic cytoplasm staining strongly for thyroid transcription factor 1. The tumor was, therefore, diagnosed as a GCT of the sellar region, belonging to tumors of the posterior pituitary. After surgery, visual impairment and anterior pituitary function were improved. Follow-up neuroimaging after 1 year showed no signs of recurrence. Conclusion: GCT of the sellar region is difficult to diagnose on routine neuroimaging. Therefore, accurate diagnosis requires careful identification of clinical signs, magnetic resonance imaging including hypointensity on T2-weighted imaging, and analysis of combined morphological and immunohistochemical studies. [ABSTRACT FROM AUTHOR]

Although sociological studies affirm the importance of parental care in the survival of offspring, maltreatment—including child neglect—remains prevalent in many countries. While child neglect is well known to affect child development, the causes of maternal neglect are poorly understood. Here, we found that female mice with a deletion mutation of CAST (a presynaptic release-machinery protein) showed significantly reduced weaning rate when primiparous and a recovered rate when multiparous. Indeed, when nurturing, primiparous and nulliparous CAST knock out (KO) mice exhibited less crouching time than control mice and moved greater distances. Contrary to expectations, plasma oxytocin (OXT) was not significantly reduced in CAST KO mice even though terminals of magnocellular neurons in the posterior pituitary expressed CAST. We further found that compared with control mice, CAST KO mice drank significantly less water when nurturing and had a greater preference for sucrose during pregnancy. We suggest that deficiency in presynaptic release-machinery protein impairs the facilitation of some maternal behaviours, which can be compensated for by experience and learning. [ABSTRACT FROM AUTHOR]

The pituitary gland is noted through distinct particularities to those of the other vertebrates. Within this class there are morphological variations in form, the size and lobulation of the pituitary gland. Thus, in some species, adenohypophysis is not anatomically divided but it is consisting of a single lobe. In many species of Teleostei adenohypophysis it consists of two lobes, one anterior or distally subdivided into two zones and the other intermediate. The present paper aims to highlight the structure of the pituitary gland and cell types present in cyprinids lobes. The studies were performed on microscopic preparations containing sagittal sections through carp pituitary. The sagittal histological sections made of 5µm reveal the presence of two distinct parts: neurohypophyses of nervous origin, and the adenohypophysis of epithelial origin. Considering the scientific importance and the practice of pituitary hormones in stimulating the maturation of gonads in fish also followed the changes of the pituitary structure, especially of the middle gonadotropic lobe. The histological study of the pituitary gland in the H. molitrix species at different times of the year has given us the possibility to track the seasonal variations of the basophil gonadotropic lobe structure. In the present study demonstrated that during the annual cycle, GTH cells go through different stages of activity that are not synchronous in all cells GTH. We were able to draw conclusions regarding the best period for harvesting pituitary glands used for pituitary injections under artificial reproduction conditions. [ABSTRACT FROM AUTHOR]

Copyright of Neotropical Ichthyology is the property of Neotropical Ichthyology and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

To maintain body homeostasis, endocrine systems must detect and integrate blood-borne peripheral signals. This is mediated by fenestrae, specialized permeable pores in the endothelial membrane. Plasmalemma vesicle-associated protein (Plvap) is located in the fenestral diaphragm and is thought to play a role in the passage of proteins through the fenestrae. However, this suggested function has yet to be demonstrated directly. We studied the development of fenestrated capillaries in the hypophysis, a major neuroendocrine interface between the blood and brain. Using a transgenic biosensor to visualize the vascular excretion of the genetically tagged plasma protein DBP-EGFP, we show that the developmental acquisition of vascular permeability coincides with differential expression of zebrafish plvap orthologs in the hypophysis versus brain. Ultrastructural analysis revealed that plvapb mutants display deficiencies in fenestral diaphragms and increased density of hypophyseal fenestrae. Measurements of DBP-EGFP extravasation in plvapb mutants provided direct proof that Plvap limits the rate of blood-borne protein passage through fenestrated endothelia. We present the regulatory role of Plvap in the development of blood-borne protein detection machinery at a neuroendocrine interface through which hormones are released to the general circulation. [ABSTRACT FROM AUTHOR]

Copyright of Medicina Neotloznyh Sostoanij is the property of Zaslavsky O.Yu and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

In 2017, the World Health Organization established that pituicytoma, granular cell tumor (GCT), spindle cell oncocytoma (SCO) and sellar ependymomas (SE) are posterior pituitary tumors (PPT). They probably arise from the pituicytes and may constitute a unique histopathological entity. We carried out a systematic review using PubMed's database. A total of 266 patients with pathological diagnosis of PPT (135 pituicytomas, 69 GCT, 47 SCO, 8 SE and 7 mixed histology tumors) were analyzed. Gender distribution was identical and median age at diagnosis was 48 ± 21.8 years. Main presentation symptoms were visual disorders (n = 142; 58.1%), headache (n = 99; 40.5%), hypopituitarism (n = 84; 34.4%), hypercortisolism (n = 10; 4.1%), polyuriapolydipsia (n = 6; 2.4%) and acromegaly features (n = 5; 2.0%). On MRI, 122 (47.6%) patients showed sellar with suprasellar extension masses, 67 (23.1%) were suprasellar and 63 (24.6%) exclusively sellar. Median tumor size was 22.0 ± 14.2 mm. Two hundred sixty four patients underwent surgery, transphenoidal access was selected in 132 (64.4%) and craniotomy in 58 (28.3%). Complications were hypopituitarism (n = 70; 42.1%), diabetes insipidus (n = 55; 33.1%) and hemorrhage (n = 50; 30.1%). Tumor persisted in 93 patients (45.6%) and recurred in 13 (6.4%). Regarding comparison between main types of PPT, SCO patients were diagnosed later (60.0 vs 47.0 vs 47.0 years, p = 0.023), the tumor was larger 25.0 mm [10.8] vs 20.0 mm [14.2] vs 2.0 mm [15.0] and they were frequently sellar with suprasellar extension tumors (71.7% vs 46.2% vs 32.8%, p = 0.003) compared to pituicytoma and GCT. In conclusion, PPT are rare tumors and have been misdiagnosed mainly as non-functioning pituitary adenomas. Different types of PPT share similar epidemiology, clinical manifestations and surgical outcomes. Surgery is the only curative option but complications and subtotal resection are common. [ABSTRACT FROM AUTHOR]

In order to conduct this study, eight adult turkey heads were obtained. Pituitary glands were harvested following cranial bones removal and examined morphologically and anatomically as well as topographically. Then, tissue sections were prepared and stained using Hematoxylin and Eosin, Alcian blue, orange G and periodic acid-Schiff staining techniques. The results showed that turkey pituitary gland as a pea-sized structure is located in the ventral part of the cerebrum and composed of adenohypophysis and neurohypophysis parts. Moreover, histological analyses revealed that sinusoids are well-developed at the distal part of the adenohypophysis and irregular masses of endocrine cells exist among them. Distributions of basophilic cells in the distal part of adenohypophysis were significantly higher than those of other endocrine cells, while the acidophilic cells had the lowest distribution. Lower and higher numbers of chromophobe cells were also found compared to those of basophilic and acidophilic cells, respectively. These findings were mostly similar to the other birds' pituitary gland anatomical and histological features, but there were also differences in cellular elements distributions along with infundibular cavity topography. [ABSTRACT FROM AUTHOR]

Oxytocin (OT) has drawn the attention of researchers since 1930. Since then, many aspects of oxytocin have been uncovered, such as reproductive functions, dampening anxiety, enhancing socioemotional behavior, or regulating genomic effects on a cellular level. Here, we want to focus on the interaction between the OT system and the stress/corticotropin-releasing factor (CRF)-system of the brain. Depending on the nature of the stressor, OT is released simultaneously or directly after the stress from the neurohypophysis into the periphery and/or via somato-dendritic release in stress-sensitive brain areas. This stress-induced OT release might serve to modulate or dampen the stress response; however, the functional relevance is not yet fully understood. In this review, we will describe the effects of OT and discuss the interplay between OT and CRF on a cellular, physiological, and behavioral level. [ABSTRACT FROM AUTHOR]

Introduction: Granular cell tumors (GCTs) of the infundibulum are rare in practice and literature, resulting in a lack of evidence-based standard of care. We present two characteristic cases from our institution and perform a systematic review of the existing literature to further elucidate the presentation of this tumor and guide management.Methods: A systematic literature search was conducted according to PRISMA guidelines, yielding 42 total individual reported GCTs suitable for evaluation. Available clinical presentation, magnetic resonance imaging (MRI) characteristics, pathology, surgical approaches, and outcomes were charted. We measured frequencies of clinical characteristics and performed an outcome comparison of open versus endoscopic surgical treatment.Results: In this pooled dataset, GCT incidence was higher in females than males (3:1). Clinical presentation peaked in the fourth decade with tumor-related symptoms. MRI appearance was characterized by T1 isointensity (50%) and T2 hypointensity or isointensity (52%) with gadolinium contrast enhancement (74%). Histopathology demonstrated positive staining for PAS, PAS-D, S100, CD68, and TTF1. In a simple uncontrolled analysis, patients who underwent endoscopic surgery experienced more symptom improvement (p = 0.006) and lower incidence of new diabetes insipidus postoperatively (p = 0.047) versus patients who underwent open microsurgery.Conclusions: This first comprehensive review of GCTs of the infundibulum corroborates existing data and adds significant new MR-radiological information to the literature, notably a typical tumor appearance of T1 isointensity, T2 iso- to hypointensity, and gadolinium contrast enhancement. Future prospective studies should be conducted to validate our findings. [ABSTRACT FROM AUTHOR]

Vasopressin (AVP) and oxytocin (OT) are essential for the control of extracellular fluid osmolality and volume. The secretion of these hormones is modulated by several mechanisms, including NMDA and AMPA l‐glutamate receptors in magnocellular cells of paraventricular (PVN) and supraoptic (SON) hypothalamic nuclei. Thus, to better understand the participation of l‐glutamate on the neuroendocrine control of AVP and OT, the present study evaluated the effects of i.c.v. NMDA and AMPA receptor antagonists on plasma AVP and OT levels induced by extracellular volume expansion (EVE). Cannulated rats received i.c.v. NMDA (amino‐5‐phosphonovalerate; AP5) and AMPA (2,3‐dihydroxy‐6‐nitro‐7‐sulphamoyl‐benzo[f]quinoxaline; NBQX) antagonists in doses of 10 and 30 nmol (5 μL per rat) and were subjected to either isotonic (0.15 mol L‐1 NaCl, 2 mL per 100 g) or hypertonic (0.30 mol L‐1 NaCl, 2 mL per 100 g) EVE. Blood samples were collected for plasma AVP and OT determination. Isotonic EVE did not change plasma AVP and OT levels, although hypertonic EVE increased both AVP and OT plasma levels. AP5 reduced plasma AVP, although it did not change the OT level induced by hypertonic EVE. On the other hand, NBQX reduced plasma OT but did not alter the AVP plasma level. Our data show that l‐glutamate controls the secretion of neurohypophyseal hormones via the NMDA receptor for AVP release, as well as via the AMPA receptor for OT release, both in response to hypertonic EVE. [ABSTRACT FROM AUTHOR]

Introduction: Persistent craniopharyngeal canal (PCC) is a rare anomaly of the base of the skull. PCC is defined as a well-corticated osseous canal, extending from the roof of the nasopharynx to the base of the sella over the course of the sphenoid corpus. We reported a PCC case with unique associations. The magnetic resonance imaging findings are discussed.Case presentation: We report a case of a 2-year-old boy with persistent craniopharyngeal canal, bilateral microphtalmia with large colobomatous cyst, and ectopic adenohypophysis with Rathke cleft cyst. He also has ectopic neurohypophysis and optic atrophy.Conclusion: The presence of orbital and optic tract malformations, craniofacial and intracranial anomalies, and tumors can accompany craniopharyngeal canals (CCs). MRI is helpful in the evaluation of PCC and associated anomalies in these patients. [ABSTRACT FROM AUTHOR]

The pituitary stalk (PS) is crucial to endocrine function and water-electrolyte equilibrium. Preservation of the PS during craniopharyngioma (CP) surgery is critical; however, in a pathological state, it is difficult to identify. The hypothalamo-hypophyseal tract (HHT) connects the hypothalamus and the posterior pituitary gland and projects through the PS. Thus, visualization of the HHT can help locate the PS. Preoperative visualization of the neural fasciculus has been widely achieved using diffusion tensor imaging (DTI) tractography. Therefore, this study evaluated the use of DTI tractography to identify and characterize the human HHT. We used DTI tractography to track the HHT in 10 patients with CP and compared the location of the tract with the intraoperative view of the PS in these patients. We successfully tracked the HHT in nine patients, indicating that delineating and quantifying the tracked HHT using this method is feasible. In addition, we found that the tract was consistent with the intraoperative view of the PS in seven out of eight patients (87.50%). Finally, we found that the mean number of tracts was 7.11 ± 12.28, the mean fractional anisotropy (FA) was 0.11 ± 0.04, and the mean tract length was 24.22 ± 9.39 mm. Taken together, our results demonstrate that the HHT can be visualized and characterized with DTI even in a clinical application, which may aid in preoperative identification of the PS. Characterization of the tracked HHT with this technique could also be used to advance our understanding of the HHT. [ABSTRACT FROM AUTHOR]

Granular cell tumors of the neurohypophysis are rare tumors with a WHO grade of I. Symptomatic tumors are even more rare. In this case, we present a 50‐year‐old patient with a sellar and suprasellar granular cell tumor of the neurohypophysis, who reported headaches, blurred vision and unsteady gait. CT imaging showed a sellar and suprasellar mass approximately 2.9 cm in diameter with clear boundaries. Histologically, the tumor lacked any obvious atypia and contained densely arranged polygonal tumor cells with abundant granular eosinophilic cytoplasm. Staining for Alpha‐1 AntiChymotrypsin (AACT), TTF‐1 and PAS was diffusely positive, and S‐100 staining was focally positive in the tumor cells. CD34, CK, EMA, GFAP and HMB45 staining were negative. The Ki‐67 index was < 1%. According to these findings, the tumor was diagnosed as a symptomatic granular cell tumor of the neurohypophysis. We suggest that identifying the location of the tumor with imaging is helpful for understanding the granular cell tumor of the neurohypophysis. Prompt diagnosis and treatment are critical for patients. [ABSTRACT FROM AUTHOR]

In the pituitary gland, S100β-positive cells localize in the neurohypophysis and adenohypophysis but the lineage of the two groups remains obscure. S100β is often observed in many neural crest-derived cell types. Therefore, in this study, we investigate the origin of pituitary S100β-positive cells by immunohistochemistry for SOX10, a potent neural crest cell marker, using S100β-green fluorescence protein-transgenic rats. On embryonic day 21.5, a SOX10-positive cell population, which was also positive for the stem/progenitor cell marker SOX2, emerged in the pituitary stalk and posterior lobe and subsequently expanded to create a rostral-caudal gradient on postnatal day 3 (P3). Thereafter, SOX10-positive cells appeared in the intermediate lobe by P15, localizing to the boundary facing the posterior lobe, the gap between the lobule structures and the marginal cell layer, a pituitary stem/progenitor cell niche. Subsequently, there was an increase in SOX10/S100β double-positive cells; some of these cells in the gap between the lobule structures showed extended cytoplasm containing F-actin, indicating a feature of migration activity. The proportion of SOX10-positive cells in the postnatal anterior lobe was lower than 0.025% but about half of them co-localized with the pituitary-specific progenitor cell marker PROP1. Collectively, the present study identified that one of the lineages of S100β-positive cells is a SOX10-positive one and that SOX10-positive cells express pituitary stem/progenitor cell marker genes. [ABSTRACT FROM AUTHOR]

Oxytocin is a neurohypophysial peptide linked to a wide range of biological functions, including milk ejection, temperament and reproduction. Aims of the present study were a) the characterization of the OXT (Oxytocin-neurophysin I) gene and its regulatory regions in Old and New world camelids; b) the investigation of the genetic diversity and the discovery of markers potentially affecting the gene regulation. On average, the gene extends over 814 bp, ranging between 825 bp in dromedary, 811 bp in Bactrian and 810 bp in llama and alpaca. Such difference in size is due to a duplication event of 21 bp in dromedary. The main regulatory elements, including the composite hormone response elements (CHREs), were identified in the promoter, whereas the presence of mature microRNAs binding sequences in the 3’UTR improves the knowledge on the factors putatively involved in the OXT gene regulation, although their specific biological effect needs to be still elucidated. The sequencing of genomic DNA allowed the identification of 17 intraspecific polymorphisms and 69 nucleotide differences among the four species. One of these (MF464535:g.622C>G) is responsible, in alpaca, for the loss of a consensus sequence for the transcription factor SP1. Furthermore, the same SNP falls within a CpG island and it creates a new methylation site, thus opening future possibilities of investigation to verify the influence of the novel allelic variant in the OXT gene regulation. A PCR-RFLP method was setup for the genotyping and the frequency of the allele C was 0.93 in a population of 71 alpacas. The obtained data clarify the structure of OXT gene in domestic camelids and add knowledge to the genetic variability of a genomic region, which has received little investigation so far. These findings open the opportunity for new investigations, including association studies with productive and reproductive traits. [ABSTRACT FROM AUTHOR]

Neurohormones vasopressin and oxytocin are synthesized in the hypothalamus and are transported along the axons to the neurohypophysis as a part of equimolar complexes with hormone-specific neurophysins. The tumors of epithelial origin synthesize ectopic vasopressin and have an ability to express all types of receptors of neurohypophysis hormones. Vasopressin and oxytocin receptors provide the transduction of signals to protein kinases A, B, and C and activate intracellular cascades of the CREB, MDM2, and TORC1/2 proteins and mitogen-activated protein kinases. Central endocrine and autocrine neurohormonal contours are involved in the regulation of proliferative, migration, and angiogenic processes accompanied by tumor progression. Tumor growth and development occur in close cooperation with the supporting stroma. The interstitial tissue is involved in signal communication of tumor cells by integrins and integral CD44 glycoproteins formulating hyaluronic acid. Hyaluronic acid metabolites modulate the effect of neurohormones and peptide growth factors; intermediate hyaluronan fragments with molecular weight of approximately 20 kDa elicit the most significant angiogenic effect. Platelets expressing AVPR1 vasopressin receptors are an important source of hyaluronidase 2 hydrolyzing macromolecular hyaluronan to fragments of intermediate length. The AVPR2 receptors localized in endothelium and AVPR1-AVPR2 vasopressin receptors expressing themselves in the tumor cells are involved in the mechanisms controlling local hemostasis. Neurohormonal regulatory contours are involved in optimization of the balance of inducing and inhibiting signals generated by the tumor and stroma in the process of progressive growth. [ABSTRACT FROM AUTHOR]

Angiotensin (1-7) peptide has been considered for a long time as an inactive product of the renin-angiotensin system. This concept changed with the studies of demonstrating that Ang (1-7) was equally potent to Angiotensin II for releasing vasopressin from neurohypophysial decreases in arterial blood pressure upon low doses of Ang (1-7) microinjection into the nucleus tractus solitarii. Because of that, Ang (1-7) has been suggested as functional peptides in the renin-angiotensin system (RAS), which is generated by the action of many angiotensin converting enzyme enzymes(ACE)-independent from ANG I. Therefore, Ang (1-7) peptide is one part of alternative rennin-angiotensin system which includes angiotensin-converting enzyme-2 (ACE2), mas receptors as well as Ang (1-7) peptide. ANG-(1-7) has many actions that are mainly counteracting ANG II vasoconstriction roles. New findings reveal depressor, vasodilator, and antihypertensive actions that may be more apparent in hypertensive animals or humans because of the actions of Ang (1-7) peptide. The accumulating evidence suggests that Ang-(1-7) may oppose the actions of Ang II either directly or by stimulation of prostaglandins and nitric oxide. Studies in humans and animals showed that the antihypertensive action of converting enzyme inhibitors correlated with increases in plasma levels of Ang-(1-7). We strongly believe that this review would be very helpful in highlighting the mechanistic roles of Ang (1-7) peptide in blood pressure regulation. [ABSTRACT FROM AUTHOR]

We present a pediatric case of a rapidly expanding third ventricle germ cell tumor (GCT). A 14-year-old boy suffered from gradual-onset central diabetes insipidus (DI) and received desmopressin treatment. Magnetic resonance imaging (MRI) showed nonspecific findings of the pituitary-hypothalamic axis. Nine months after the initial DI diagnosis, he developed progressively worsening headache. MRI demonstrated a third ventricle tumor causing noncommunicating hydrocephalus, although an MRI 16 weeks before admission did not show the lesion. We performed gross total resection (GTR) of the tumor in 2 stages: a translamina terminalis approach and an extended transsphenoidal approach. The lesion was histologically diagnosed as immature teratoma with some germinoma. His noncommunicating hydrocephalus resolved after surgery. Through postoperative radiochemotherapy (whole ventricle: 23.4 Gy/13 fractions, tumor bed: 27.0 Gy/15 fractions, and 3 courses of carboplatin-etoposide), he has was in complete remission at the 3-year follow-up and has continued his high school program. This case suggests the following: (1) a mixed GCT originating from the neurohypophysis/infundibulum can show rapidly expansive growth in a child with central DI; (2) GTR and adjuvant radiochemotherapy can result in a good therapeutic outcome in rapidly expanding GCT; and (3) the extended transsphenoidal approach is a complementary approach to transcranial resection of anterior third ventricle GCTs. [ABSTRACT FROM AUTHOR]

Granular cell tumor (GCT) rarely involves the central nervous system, and fewer than 100 cases have been reported in English literatures. We herein report a case of a 36-year-old Chinese man with GCT of the neurohypophysis. Magnetic resonance imaging showed one mass located in the hypophysis with heterogeneous contrast enhancement. Pathological examination showed a neoplasm comprising densely packed polygonal cells of ample cytoplasm with abundant eosinophilic granules inside. The nuclei were small with inconspicuous nucleoli and yet without any mitoses. The tumor was positive for S-100, CD68, CD163, lysosome, and vimentin. Translocation factor E-3 (TFE-3) was diffusely nuclear positive although ASPSCR1-TFE-3 fusion was not detected by fluorescence in situ hybridization. GCT of the neurohypophysis is supposed to be considered under differential diagnosis with neoplasms or lesions of histiocytic origin and others such as pituitocytoma and spindle cell oncocytoma. A group of markers such as GFAP, EMA, CD68, S-100, and PAS staining are useful in complementary diagnosis and TFE-3 may be an alternative marker. [ABSTRACT FROM AUTHOR]

The protective effect in rainbow trout ( Oncorhynchus mykiss) of an experimental subunit vaccine targeting antigens in the parasite Ichthyophthirius multifiliis has been evaluated and compared to effects elicited by a classical parasite homogenate vaccine. Three recombinant parasite proteins (two produced in E. coli and one in insect cells) were combined and injected i.p., and subsequently, protection and antibody responses were analysed. Both the experimental and the benchmark vaccine induced partial but significant protection against I. multifiliis when compared to control fish. Specific antibody responses of vaccinated trout (subunit vaccine) were raised against one neurohypophysial n-terminal domain protein #10 of three recombinant proteins, whereas the benchmark vaccine group showed specific antibody production against all three recombinant proteins. The immunogenic parasite protein #10 may be a potential vaccine candidate supplementing the protective I-antigen in future vaccine trials. [ABSTRACT FROM AUTHOR]

Background Mutations in PROP1, HESX1 and LHX3 are associated with combined pituitary hormone deficiency ( CPHD) and orthotopic posterior pituitary lobe ( OPP). Objective To identify mutations in PROP1, HESX1 and LHX3 in a large cohort of patients with CPHD and OPP (35 Brazilian, two Argentinian). Design and Methods We studied 23 index patients with CPHD and OPP (six familial and 17 sporadic) as well as 14 relatives. PROP1 was sequenced by the Sanger method in all except one sporadic case studied using a candidate gene panel. Multiplex ligation-dependent probe amplification ( MLPA) was applied to one familial case in whom PROP1 failed to amplify by PCR. In the 13 patients without PROP1 mutations, HESX1 and LHX3 were sequenced by the Sanger method. Results We identified PROP1 mutations in 10 index cases. Three mutations were novel: one affecting the initiation codon (c.1A>G) and two affecting splicing sites, c.109+1G>A and c.342+1G>C. The known mutations, c.150delA (p.Arg53Aspfs*112), c.218G>A (p.Arg73His), c.263T>C (p.Phe88Ser) and c.301_302del AG (p.Leu102Cysfs*8), were also detected. MLPA confirmed complete PROP1 deletion in one family. We did not identify HESX1 and LHX3 mutations by Sanger. Conclusion PROP1 mutations are a prevalent cause of congenital CPHD with OPP, and therefore, PROP1 sequencing must be the first step of molecular investigation in patients with CPHD and OPP, especially in populations with a high frequency of PROP1 mutations. In the absence of mutations, massively parallel sequencing is a promising approach. The high prevalence and diversity of PROP1 mutations is associated with the ethnic background of this cohort. [ABSTRACT FROM AUTHOR]

The hypothalamic neurosecretory system synthesizes neuropeptides in hypothalamic nuclei and releases them from axonal terminals into the circulation in the neurohypophysis (NH) and median eminence (ME). This system plays a crucial role in regulating body fluid homeostasis and social behaviors as well as reproduction, growth, metabolism, and stress responses, and activity-dependent structural reorganization has been reported. Current knowledge on dynamic structural reorganization in the NH and ME, in which the axonal terminals of neurosecretory neurons directly contact the basement membrane (BM) of a fenestrated vasculature, is discussed herein. Glial cells, pituicytes in the NH and tanycytes in the ME, engulf axonal terminals and interpose their cellular processes between axonal terminals and the BM when hormonal demands are low. Increasing demands for neurosecretion result in the retraction of the cellular processes of glial cells from axonal terminals and the BM, permitting increased neurovascular contact. The shape conversion of pituicytes and tanycytes is mediated by neurotransmitters and sex steroid hormones, respectively. The NH and ME have a rough vascular BM profile of wide perivascular spaces and specialized extension structures called "perivascular protrusions." Perivascular protrusions, the insides of which are occupied by the cellular processes of vascular mural cells pericytes, contribute to increasing neurovascular contact and, thus, the efficient diffusion of hypothalamic neuropeptides. A chronic physiological stimulation has been shown to increase perivascular protrusions via the shape conversion of pericytes and the profile of the vascular surface. Continuous angiogenesis occurs in the NH and ME of healthy normal adult rodents depending on the signaling of vascular endothelial growth factor (VEGF). The inhibition of VEGF signaling suppresses the proliferation of endothelial cells (ECs) and promotes their apoptosis, which results in decreases in the population of ECs and axonal terminals. Pituicytes and tanycytes are continuously replaced by the proliferation and differentiation of stem/progenitor cells, which may be regulated by matching those of ECs and axonal terminals. In conclusion, structural reorganization in the NH and ME is caused by the activity-dependent shape conversion of glial cells and vascular mural cells as well as the proliferation of endothelial and glial cells by angiogenesis and gliogenesis, respectively. [ABSTRACT FROM AUTHOR]

Oxytocin is known to be implicated in a variety of functions, such as learning, stress, anxiety, feeding, and pain perception. Oxytocin is also important for social memory and attachment, human bonding, sexual and maternal behaviour, and aggression. Human disorders characterized by aberrant social interactions, such as autism and schizophrenia, may also involve abnormal oxytocin levels. GSK712043, GSK711320, and GSK664004, three antagonists exhibiting subnanomolar affinity for the human oxytocin receptor (hOTR) and high selectivity over vasopressin receptors were successfully labelled with carbon-11 with suitable yields (0.5-1GBq @EOS), high molar activity (275-700 GBq/μmol), and radiochemical purities. The in vivo regional uptake of these radiotracers was determined in porcine brain. [11C]GSK711320 baseline scan showed no significant brain uptake, and limited initial uptake was observed following administration of [11C]GSK712043 or [11C]GSK664004. The [11C]GSK712043 and [11C]GSK664004 kinetics were slow and peaked at around 2%ID/L at 90 minutes post-injection. For both tracers, the distribution of activity was homogeneous throughout the brain. All the tracers showed high uptake in the pituitary gland, especially [11C]GSK711320; however, its uptake could not be blocked by pretreatment with the known OTR antagonist, L368,899. In vivo evaluation of these candidates demonstrated that they are not suitable as central OTR PET imaging agents. [ABSTRACT FROM AUTHOR]

Key points A growing body of evidence suggests that epithelial Na+ channels (ENaCs) in the brain play a significant role in the regulation of blood pressure; however, the brain structures that mediate the effect are not well understood., Because vasopressin (VP) neurons play a pivotal role in coordinating neuroendocrine and autonomic responses to maintain cardiovascular homeostasis, a basic understanding of the regulation and activity of ENaC in VP neurons is of great interest., We show that high dietary salt intake caused an increase in the expression and activity of ENaC which resulted in the steady state depolarization of VP neurons., The results help us understand one of the mechanisms underlying how dietary salt intake affects the activity of VP neurons via ENaC activity., Abstract All three epithelial Na+ channel (ENaC) subunits (α, β and γ) are located in vasopressin (VP) magnocellular neurons in the hypothalamic supraoptic (SON) and paraventricular nuclei. Our previous study demonstrated that ENaC mediates a Na+ leak current that affects the steady state membrane potential in VP neurons. In the present study, we evaluated the effect of dietary salt intake on ENaC regulation and activity in VP neurons. High dietary salt intake for 7 days caused an increase in expression of β- and γENaC subunits in the SON and the translocation of αENaC immunoreactivity towards the plasma membrane. Patch clamp experiments on hypothalamic slices showed that the mean amplitude of the putative ENaC currents was significantly greater in VP neurons from animals that were fed a high salt diet compared with controls. The enhanced ENaC current contributed to the more depolarized basal membrane potential observed in VP neurons in the high salt diet group. These findings indicate that high dietary NaCl intake enhances the expression and activity of ENaCs, which augments synaptic drive by depolarizing the basal membrane potential close to the action potential threshold during hormonal demand. However, ENaCs appear to have only a minor role in the regulation of the firing activity of VP neurons in the absence of synaptic inputs as neither the mean intraburst frequency, burst duration, nor interspike interval variability of phasic bursting activity was affected. Moreover, ENaC activity did not affect the initiation, sustention, or termination of the phasic bursting generated in an intrinsic manner without synaptic inputs. [ABSTRACT FROM AUTHOR]

Background: Congenital central nervous system abnormalities in children with Fanconi anemia are poorly characterized, especially with regard to specific genetic complementation groups.Objective: To characterize the impact of genetic complementation groups on central nervous system anatomy.Materials and Methods: Through chart review we identified 36 patients with Fanconi anemia with available brain MRIs at the University of Minnesota (average age, 11.3 years; range, 1-43 years; M:F=19:17), which we reviewed and compared to 19 age- and sex-matched controls (average age, 7.9 years; range, 2-18 years; M:F=9:10). Genotypic information was available for 27 patients (15 FA-A, 2 FA-C, 3 FA-G, and 7 FA-D1 [biallelic mutations in BRCA2 gene]).Results: Of the 36 patients, 61% had at least one congenital central nervous system or skull base abnormality. These included hypoplastic clivus (n=12), hypoplastic adenohypophysis (n=11), platybasia (n=8), pontocerebellar hypoplasia (n=7), isolated pontine hypoplasia (n=4), isolated vermis hypoplasia (n=3), and ectopic neurohypophysis (n=6). Average pituitary volume was significantly less in patients with Fanconi anemia (P<0.0001) than in controls. Basal angle was significantly greater in Fanconi anemia patients (P=0.006), but the basal angle of those with FA-D1 was not significantly different from controls (P=0.239). Clivus length was less in the Fanconi anemia group (P=0.002), but significance was only observed in the FA-D1 subgroup (P<0.0001). Of the seven patients meeting criteria for pontocerebellar hypoplasia, six belonged to the FA-D1 group.Conclusion: Patients with Fanconi anemia have higher incidences of ectopic neurohypophysis, adenohypophysis hypoplasia, platybasia and other midline central nervous system skull base posterior fossa abnormalities than age- and sex-matched controls. Patients with posterior fossa abnormalities, including pontocerebellar hypoplasia, are more likely to have biallelic BRCA2 mutations. [ABSTRACT FROM AUTHOR]

Background: In anterior pituitary deficiency, patients with non visible pituitary stalk have more often multiple deficiencies and persistent deficiency than patients with visible pituitary stalk.Objective: To compare the diagnostic value of a high-resolution heavily T2-weighted sequence to 1.5-mm-thick unenhanced and contrast-enhanced sagittal T1-weighted sequences to assess the presence of the pituitary stalk in children with ectopic posterior pituitary gland.Materials and Methods: We retrospectively evaluated the MRI data of 14 children diagnosed with ectopic posterior pituitary gland between 2010 and 2014. We evaluated the presence of a pituitary stalk using a sagittal high-resolution heavily T2-weighted sequence and a 1.5-mm sagittal T1-weighted turbo spin-echo sequence before and after contrast medium administration.Results: A pituitary stalk was present on at least one of the sequences in 10 of the 14 children (71%). T2-weighted sequence depicted the pituitary stalk in all 10 children, whereas the 1.5-mm-thick T1-weighted sequence depicted 2/10 (20%) before contrast injection and 8/10 (80%) after contrast injection (P=0.007).Conclusion: Compared with 1.5-mm-thick contrast-enhanced T1-weighted sequences, high-resolution heavily T2-weighted sequence demonstrates better sensitivity in detecting the pituitary stalk in children with ectopic posterior pituitary gland, suggesting that contrast injection is unnecessary to assess the presence of a pituitary stalk in this setting. [ABSTRACT FROM AUTHOR]

Purpose: To analyse the antigen expression profiles of 27 cases of pituicytoma, spindle cell oncocytoma, and granular cell tumour of the sellar region concerning a common pituicytic origin of neoplastic cells. Methods: Material from 12 female and 15 male patients (13 granular cell tumours of the sellar region, 10 pituicytomas, four spindle cell oncocytomas) collected in the German Registry of Pituitary Tumours between 1993 and 2015 was re-evaluated according to the current WHO classification of tumours of the central nervous system and supplementary immunohistochemistry including S100-protein, CD56, CD68, thyroid transcription factor-1 (TTF-1), and Ki-67 was performed. Results: S100-protein was detected in all 27 tumours and TTF-1 in all 16 tumours that were assessed. Vimentin was expressed in all 13 cases investigated whereas broad spectrum cytokeratin was not detected in any of 14 evaluated cases. GFAP was observed in nine out of 21 cases. 15 out of 17 investigated lesions showed some CD68 expression and five out of 14 cases were labelled with CD56 antibodies. Proliferative activity did not differ significantly between the three tumour subgroups although one primary and one recurrent pituicytoma showed exceptionally high Ki-67-proliferation indices of 15.3 and 12.7 %, respectively (means: granular cell tumour of the sellar region 2.0 %, pituicytoma 2.8 %, spindle cell oncocytoma 2.7 %). Conclusions: The study confirms and expands earlier data and is in line with the notion that the three tumour types are variants of pituicytoma. [ABSTRACT FROM AUTHOR]

Context: Ectopic neurohypophysis (EN) refers to an interrupted, nonvisualized, and thinned out pituitary stalk with ectopic location of the posterior pituitary gland. Concurrent extra-pituitary cerebral and extra-cranial anomalies have been rarely reported in patients of EN. Aim: The aim of this study was to evaluate the magnetic resonance imaging (MRI) findings of extra-pituitary cerebral anomalies in pediatric patients of EN. Settings and Design: A hospital-based cross-sectional study was conducted in a tertiary care center. Subjects and Methods: The study group comprised eight pediatric patients of EN associated with extra-pituitary cerebral or vascular anomalies. Clinical and biochemical assessment was done in all patients. Results: Out of the total eight patients with EN, MRI showed interrupted pituitary stalk in five patients (62.5%) and nonvisible pituitary stalk in three patients (37.5%). Ectopic posterior pituitary bright spot was demonstrated in median eminence in six patients (75%), faintly visualized in one patient (12.5%) and nonvisualized in another one patient. Statistical significant association was noted between pituitary gland height and patient's body height with the pituitary gland volume (P < 0.001). Varied extra-pituitary cerebral anomalies encountered in our patients ranged from isolated anomalies such as optic nerve hypoplasia in three patients (37.5%), corpus callosum dysplasia in four patients (50%), agyria-pachygyria complex in two patients (25%), and intracranial vascular anomalies in two patients to syndromic association of tuberous sclerosis in one patient. Conclusion: Identifying and reporting of associated extra-pituitary cerebral anomalies in patients with EN are crucial in assessing the overall neurological outcome of such patients. [ABSTRACT FROM AUTHOR]

The arginine-vasopressin (AVP)-containing hypothalamic magnocellular neurosecretory neurons (VPMNNs) are known for their role in hydro-electrolytic balance control via their projections to the neurohypophysis. Recently, projections from these same neurons to hippocampus, habenula and other brain regions in which vasopressin infusion modulates contingent social and emotionally-affected behaviors, have been reported. Here, we present evidence that VPMNN collaterals also project to the amygdaloid complex, and establish synaptic connections with neurons in central amygdala (CeA). The density of AVP innervation in amygdala was substantially increased in adult rats that had experienced neonatal maternal separation (MS), consistent with our previous observations that MS enhances VPMNN number in the paraventricular (PVN) and supraoptic (SON) nuclei of the hypothalamus. In the CeA, V1a AVP receptor mRNA was only observed in GABAergic neurons, demonstrated by complete co-localization of V1a transcripts in neurons expressing Gad1 and Gad2 transcripts in CeA using the RNAscope method. V1b and V2 receptor mRNAs were not detected, using the same method. Water-deprivation (WD) for 24 h, which increased the metabolic activity of VPMNNs, also increased anxiety-like behavior measured using the elevated plus maze (EPM) test, and this effect was mimicked by bilateral microinfusion of AVP into the CeA. Anxious behavior induced by either WD or AVP infusion was reversed by CeA infusion of V1a antagonist. VPMNNs are thus a newly discovered source of CeA inhibitory circuit modulation, through which both early-life and adult stress coping signals are conveyed from the hypothalamus to the amygdala. [ABSTRACT FROM AUTHOR]

Pituicytoma is a rare tumor of the sellar and suprasellar regions, arising from the pituicytes, which are specialized glial cells in the neurohypophysis and infundibulum. Due to its rarity, ambiguity persists over the diagnosis, management and prognosis of pituicytoma. The current study presents a case series of three patients, each with a histopathological diagnosis of pituicytoma. A summary of the clinical manifestations, radiological characteristics, histopathological features, treatment strategies and prognoses are presented. In addition, 78 cases of pituicytoma, identified in a search of the published literature in Pubmed, are profiled. Pituicytoma typically presents with dysfunction of the optic nerve and pituitary. The radiological characteristics are nonspecific; diagnosis is typically made on the basis of histopathological results. The tumor is slow growing and benign and is amenable to surgical treatment by gross total resection; subsequent tumor recurrence is rare. A definitive assessment of prognosis requires an extended follow-up in a larger cohort. [ABSTRACT FROM AUTHOR]

We present the first reported case of an infant with 18p deletion syndrome with anterior pituitary aplasia secondary to a ring chromosome. Endocrine workup soon after birth was reassuring; however, repeat testing months later confirmed central hypopituitarism. While MRI reading initially indicated no midline defects, subsequent review of the images confirmed anterior pituitary aplasia with ectopic posterior pituitary. This case demonstrates how deletion of genetic material, even if resulting in a chromosomal ring, still results in a severe syndromic phenotype. Furthermore, it demonstrates the necessity of close follow-up in the first year of life for children with 18p deletion syndrome and emphasizes the need to verify radiology impressions if there is any doubt as to the radiologic findings. [ABSTRACT FROM AUTHOR]

Background: We report the case of a 6 year old boy suffering from adenohypophysis aplasia as well as ectopic neurohypophysis and delayed diagnosis of familial Mediterranean fever (FMF). Case presentation: The boy was diagnosed with panhypopituitarism during the neonatal period and suffered from recurrent episodes during the following years suggesting infections. He also showed signs of adrenal insufficiency. Finally, at the age of 6 years, an additional diagnosis of familial Mediterranean fever (FMF) was clinically suspected and later confirmed by molecular analysis. Conclusion: The clinical pictures of panhypopituitarism and FMF can be overlapping. It is imperative to take a detailed and accurate history in order to find the right diagnosis, particularly a precise family history. In conditions like FMF an early diagnosis is crucial, as initiation of treatment with colchicine is important to prevent long-term complications due to amyloid fibril deposition. [ABSTRACT FROM AUTHOR]

Purpose: Granular cell tumors of the neurohypophysis are rare, solitary lesions, mostly presenting in the adult age. They rarely grow to a sufficient size to cause mass effect related symptoms and they may be found in most cases incidentally at autopsy. Because of their rarity as of now they have been described only as case reports or included in small clinical series. Methods: We report a series of 11 patients, who underwent surgery for granular cell tumors of the neurohypophysis between 1996 and 2013 in a single center. Results: Mean follow-up time after treatment was 92.2 months (range 9-231 months). Mean age at surgery was 40.7 years (range 12-66 years). There were 7 males (63.6 %) and 4 females (36.4 %). Main symptoms at presentation were: hyperprolactinemia (72.7 %), visual impairment (45.5 %) and headache (36 %). Except for 2 patients, all the others underwent surgery as primary treatment at our Institution, through a transsphenoidal (54.5 %) or a transcranial approach (45.5 %). Overall- and progression-free survival times for the entire series (calculated from the time of diagnosis) were 112.9 and 100.5 months respectively. There was one case of perioperative death in a patient who had undergone repeat transcranial surgery for residual tumor. Conclusions: Although extremely rare, granular cell tumors of the neurohypophysis have to be considered in the differential diagnosis of suprasellar masses, to avoid misleading interpretation and consequent wrong therapeutic management. Early diagnosis, extensive tumor removal, opportune indication of adjuvant radiotherapy are the keys to manage these cases. [ABSTRACT FROM AUTHOR]

Introduction: Beta-human chorionic gonadotropin (HCG-β) is considered to be a useful tumor marker for germ cell tumors (GCTs); however, various tumors other than GCTs, including cystic pituitary adenomas, Rathke's cleft cysts, and craniopharyngiomas, were reported to express HCG-β. Case report: We herein present the case of a 5-year-old boy who presented with polyuria and had a solitary lesion in the neurohypophysis with a positive HCG-β titer in the cerebrospinal fluid. Under a preoperative diagnosis of germinoma, a biopsy was performed from the posterior pituitary lobe via the transsphenoidal endoscopic approach and the histological diagnosis was revealed to be Langerhans cell histiocytosis (LCH). Conclusions: The finding of a slightly positive HCG-β titer in the cerebrospinal fluid (CSF) cannot exclude the possibility of LCH, and we strongly recommend a histological diagnosis for the diagnosis of a solitary neurohypophysial lesion. [ABSTRACT FROM AUTHOR]

Sickness behaviour, a syndrome characterized by a general reduction in animal activity, is part of the active-phase response to fight infection. Lipopolysaccharide (LPS), an effective endotoxin to model sickness behaviour, reduces thirst and sodium excretion, and increases neurohypophysial secretion. Here we review the effects of LPS on thirst and sodium appetite. Altered renal function and hydromineral fluid intake in response to LPS occur in the context of behavioural reorganization, which manifests itself as part of the syndrome. Recent data show that, in addition to its classical effect on thirst, non-septic doses of LPS injected intraperitoneally produce a preferential inhibition of intracellular thirst versus extracellular thirst. Moreover, LPS also reduced hypertonic NaCl intake in sodium-depleted rats that entered a sodium appetite test. Antagonism of α2-adrenoceptors abolished the effect of LPS on sodium appetite. LPS and cytokine transduction potentially recruit brain noradrenaline and α2-adrenoceptors to control sodium appetite and sickness behaviour. [ABSTRACT FROM AUTHOR]