Pituitary Region Research Articles

Role of diffusion-weighted imaging in the diagnosis of pituitary region tumors.

This study aimed to assess the role of Diffusion-Weighted Imaging (DWI) in routine pituitary Magnetic Resonance Imaging (MRI) protocols for distinguishing sellar and parasellar tumors, addressing the lack of clear guidelines in contemporary literature. A retrospective analysis of 242 pituitary MRI scans with DWI sequences was conducted in a single-center study using a 1.5T scanner and standard DWI sequence parameters. Measurements of both absolute and relative mean apparent diffusion coefficient (ADC) values, along with minimal ADC values within tumors, were performed. The adopted region of interest (ROI) based method used for these measurements was validated. Invasive pituitary adenomas exhibited significantly lower min ADC and min rADC than meningiomas, with optimal cut-off points of 0.64 (sensitivity 73%, specificity 82%) and 0.78 (sensitivity 73%, specificity 89%), respectively. Post-hemorrhagic pituitary adenomas demonstrated lower ADC values than adamantinomatous craniopharyngiomas, with an AUC of 0.893 for min rADC = 1.07, and Rathke's Cleft Cysts with mucous content, AUC 0.8 for min rADC = 1.01. Specific differentiation with high sensitivity and specificity based on diffusion parameters was observed for these tumor groups. Cystic pituitary non-functional adenomas obtained significantly lower ADC values compared to the adamantinomatous type of craniopharyngiomas and serous Rathke's Cleft Cysts (AUC up to 0.942). The study concludes that integrating DWI into routine pituitary MRI protocols enhances diagnostic accuracy in distinguishing sellar and parasellar tumors. The short scan time of one minute makes DWI a valuable and precise tool, supporting its recommendation as a standard component of pituitary MRI examinations.

Multisystemic recurrent Langerhans cell histiocytosis misdiagnosed with chronic inflammation at the first diagnosis: A case report

BACKGROUND Langerhans cell histiocytosis (LCH) is characterized by diabetes insipidus and is an uncommon occurrence. Pathological biopsies still have a certain degree of diagnostic probability. We present a case in which LCH initially affected the pituitary gland. This resulted in a misdiagnosis of chronic inflammation upon pathological examination. CASE SUMMARY A 25-year-old female exhibited symptoms of diabetes insipidus. Magnetic resonance imaging revealed an enhanced foci in the pituitary gland. After surgical resection of the pituitary lesion, the pathological diagnosis was chronic inflammation. However, the patient later experienced bone destruction in the skull and lower limb bones. After the lower limb bone lesion was compared with the initial pituitary lesion, the final diagnosis was modified to LCH. The patient was treated with multiple chemotherapy courses. However, the patient’s condition gradually worsened, and she eventually passed away at home. CONCLUSION LCH should be considered when patients exhibit diabetes insipidus and absence of high signal intensity in the pituitary gland on sagittal T1-weighted image and abnormal enhancement in the pituitary region.

Double Localization of a Cerebral Germinoma: Case Report

Germinal cell tumors are embryonic tumors located mainly in the gonads, and their cerebral localization is rare, representing less than 1% of intracranial neoplasia. Clinical expression can be polymorphous, while imaging provides a highly suggestive radiological semiology. We report the case of a 31-year-old patient presenting with diabetes insipidus evolving for one year and associated with decreased visual acuity. Cerebro-medullary MRI revealed a tumoral process in the pineal region associated with thickening of the pituitary region. Tumor markers (β HCG and AFP) in plasma and CSF were negative, confirming the diagnosis of pure germinoma without the need for biopsy. Treatment is mostly based on radiotherapy, and sometimes on a combination of radiotherapy and chemotherapy. The latter was our therapeutic choice, with a good clinical and biological evolution.

Thin-slice reverse encoding distortion correction DWI facilitates visualization of non-functioning pituitary neuroendocrine tumor (PitNET)/pituitary adenoma and surrounding normal structures

BackgroundTo evaluate the clinical usefulness of thin-slice echo-planar imaging (EPI)-based diffusion-weighted imaging (DWI) with an on-console distortion correction technique, termed reverse encoding distortion correction DWI (RDC-DWI), in patients with non-functioning pituitary neuroendocrine tumor (PitNET)/pituitary adenoma.MethodsPatients with non-functioning PitNET/pituitary adenoma who underwent 3-T RDC-DWI between December 2021 and September 2022 were retrospectively enrolled. Image quality was compared among RDC-DWI, DWI with correction for distortion induced by B0 inhomogeneity alone (B0-corrected-DWI), and original EPI-based DWI with anterior-posterior phase-encoding direction (AP-DWI). Susceptibility artifact, anatomical visualization of cranial nerves, overall tumor visualization, and visualization of cavernous sinus invasion were assessed qualitatively. Quantitative assessment of geometric distortion was performed by evaluation of anterior and posterior displacement between each DWI and the corresponding three-dimensional T2-weighted imaging. Signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and apparent diffusion coefficient values were measured.ResultsSixty-four patients (age 70.8 ± 9.9 years [mean ± standard deviation]; 33 females) with non-functioning PitNET/pituitary adenoma were evaluated. In terms of susceptibility artifacts in the frontal and temporal lobes, visualization of left trigeminal nerve, overall tumor visualization, and anterior displacement, RDC-DWI performed the best and B0-corrected-DWI performed better than AP-DWI. The right oculomotor and right trigeminal nerves were better visualized by RDC-DWI than by B0-corrected-DWI and AP-DWI. Visualization of cavernous sinus invasion and posterior displacement were better by RDC-DWI and B0-corrected-DWI than by AP-DWI. SNR and CNR were the highest for RDC-DWI.ConclusionsRDC-DWI achieved excellent image quality regarding susceptibility artifact, geometric distortion, and tumor visualization in patients with non-functioning PitNET/pituitary adenoma.Relevance statementRDC-DWI facilitates excellent visualization of the pituitary region and surrounding normal structures, and its on-console distortion correction technique is convenient. RDC-DWI can clearly depict cavernous sinus invasion of PitNET/pituitary adenoma even without contrast medium.Key points• RDC-DWI is an EPI-based DWI technique with a novel on-console distortion correction technique.• RDC-DWI corrects distortion due to B0 field inhomogeneity and eddy current.• We evaluated the usefulness of thin-slice RDC-DWI in non-functioning PitNET/pituitary adenoma.• RDC-DWI exhibited excellent visualization in the pituitary region and surrounding structures.• In addition, the on-console distortion correction of RDC-DWI is clinically convenient.Graphical

Association between PET–CT accumulation in the hypothalamic/pituitary regions and neuron-specific enolase/primary tumor in limited-stage small cell lung cancer: a case-controlled retrospective study

BackgroundResearch on the relationship between neuron-specific enolase (NSE) levels and normal organs, particularly the central nervous system, in small cell lung cancer is limited. Therefore, this study aimed to investigate the relationship between positron emission tomography–computed tomography (PET–CT) accumulation at hypothalamic/pituitary regions, tumor activity, and NSE level in limited-stage small cell lung cancer. We retrospectively analyzed patients who were diagnosed with limited-stage small cell lung cancer at Tokyo Medical University Hospital between July 1, 2019, and May 31, 2023, and were treated with chemoradiotherapy or radiotherapy. Leukocytes, erythrocytes, hemoglobin, platelets, total protein, albumin, NSE, and carcinoembryonic antigen were measured in blood samples obtained before treatment initiation. The maximum standardized uptake value (SUVmax), volume, and total lesion glycolysis (TLG) of each hypothalamic /pituitary region, primary tumor, and lymph node metastases were extracted from PET–CT images. The total tumor volume (primary tumor volume plus lymph node metastases volume) and total TLG (primary tumor TLG plus lymph node metastases TLG) were calculated.ResultsThis study included 19 patients (mean age, 70.1 ± 8.8 years; 13 men and 6 women); the pathology in all patients was small cell lung cancer. Patients were classified into two groups according to the NSE reference value (16.3 ng/mL): six patients having NSE level below the reference value and 13 having NSE level above the reference value. The SUVmax in the hypothalamic/pituitary region was 2.95 in the NSE < 16.3 ng/mL group and 4.10 in the NSE > 16.3 ng/mL group, with a statistically significant difference (p = 0.03). The total tumor volume was 17.8 mL in the NSE < 16.3 ng/mL group and 98.9 mL in the NSE > 16.3 ng/mL group, with a statistically significant difference (p < 0.01). A correlation coefficient of r = 0.458 (p = 0.0486) was observed between SUVmax in the hypothalamus/pituitary and NSE level. A correlation coefficient of r = 0.647 (p < 0.01) was also observed between total tumor volume and NSE level. Finally, a correlation coefficient of r = 0.53 (p = 0.01) was observed between hypothalamic/pituitary TLG and primary tumor TLG.ConclusionsThe findings demonstrated a correlation between hypothalamic/pituitary activity and tumor activity, suggesting the prognostic significance of NSE.

Are prolactin levels efficient in predicting a pituitary lesion in patients with hyperprolactinemia?

Data regarding the presence of a prolactin (PRL) threshold above which a pituitary magnetic resonance imaging (MRI) is mandatory in patients with hyperprolactinemia (hyperPRL) are controversial and derived primarily from studies focused on female populations. Aim of our study was to evaluate in a cohort of patients of both sexes with confirmed hyperPRL, the possible correlation between PRL values and the presence of pituitary abnormalities. We retrospectively analyzed data from patients who underwent serial PRL sampling at our Division between January 2015 and December 2022. Patients diagnosed with monomeric hyperPRL at serial sampling and with subsequent contrast-enhanced MRI results available for the pituitary region were included in the study. Exclusion criteria were prior pituitary disease, severe renal insufficiency, liver cirrhosis, uncompensated primary hypothyroidism and ongoing therapy with hyperprolactinemic drugs. Physiological causes of hyperPRL were also ruled out. Out of the 1253 patients who underwent serial PRL sampling, 139 patients (101 women and 38 men) met the inclusion criteria: 106 (76.3%) patients had some form of pituitary disease, with microlesions observed in 69.8%, macrolesions in 25.5% and other findings in 4.7% of subjects. PRL values showed a modest accuracy in predicting the presence of a pituitary abnormality and the best cut-offs identified were >25 µg/L (AUC 0.767, p = 0.003) and >44.2 µg/L (AUC 0.697, p < 0.001) in men and women, respectively; however, if only patients with PRL values > 500 µg/L were excluded from the analysis, as they were already supposed to harbor a macroprolactinoma, PRL levels were not able to predict the presence of a macrolesion neither in men nor women. Given the high prevalence of pituitary abnormalities in patients of both sexes with hyperPRL at serial sampling, performing a pituitary imaging in all cases of hyperPRL, even if mild, appears to be a cautious choice.

Corticotropin-Releasing Hormone: A Novel Stimulator of Somatolactin in Teleost Pituitary Cells.

Corticotropin-releasing hormone (CRH) is known for its crucial role in the stress response system, which could induce pituitary adrenocorticotropic hormone (ACTH) secretion to promote glucocorticoid release in the adrenal gland. However, little is known about other pituitary actions of CRH in teleosts. Somatolactin is a fish-specific hormone released from the neurointermediate lobe (NIL) of the posterior pituitary. A previous study has reported that ACTH was also located in the pituitary NIL region. Interestingly, our present study found that CRH could significantly induce two somatolactin isoforms' (SLα and SLβ) secretion and synthesis in primary cultured grass carp pituitary cells. Pharmacological analysis further demonstrated that CRH-induced pituitary somatolactin expression was mediated by the AC/cAMP/PKA, PLC/IP3/PKC, and Ca2+/CaM/CaMK-II pathways. Finally, transcriptomic analysis showed that both SLα and SLβ should play an important role in the regulation of lipid metabolism in primary cultured hepatocytes. These results indicate that CRH is a novel stimulator of somatolactins in teleost pituitary cells, and somatolactins may participate in the stress response by regulating energy metabolism.

Identifying patient-related predictors of permanent growth hormone deficiency.

Isolated childhood growth hormone deficiency (GHD) can persist into adulthood, and re-testing at the transition period is needed to determine whether continued growth hormone therapy is indicated. Here, our objective was to identify predictors of permanent GHD. Retrospective single-centre study of patients with childhood-onset GHD who were re-tested after adult height attainment. Auxological, clinical, laboratory, and MRI data throughout follow-up were collected. We included 101 patients. At GH treatment initiation, age was 8.1 ± 0.4 years, height -2.25 ± 0.8, and BMI -0.27 ± 0.1 SDS. The 29 (28.7%) patients with persistent GHD had lower height SDS (-2.57 ± 0.1 vs. -2.11 ± 0.1, p<0.001) and mean GH peaks (8.4 ± 1.0 vs.13.2 ± 0.5 mIU/L, p<0.001) at GHD diagnosis; at adult height, they had lower IGF1 (232 ± 19.9 vs. 331 ± 9.1 ng/mL, p<0.001) and higher BMI SDS (-0.15 ± 0.27 vs. -0.73 ± 0.13, p<0.005). By multivariate analysis, the best predictive model included height and BMI SDS, both GH peaks, and MRI findings at diagnosis. Patients with height at diagnosis <-3 SDS had a 7.7 (95% IC 1.4-43.1, p=0.02) fold higher risk of persistent GHD after adjustment on BMI SDS. An abnormal pituitary region by MRI was the strongest single predictor (7.2 times, 95% CI 2.7-19.8) and after multivariate analysis adjustment for GH peaks and height SDS at diagnosis, the risk increased to 10.6 (1.8 - 61.3) times. Height <-3 SDS at GHD diagnosis and pituitary MRI abnormalities should lead to a high index of suspicion for persistent GHD.

Reliability of intraoperative visual evoked potentials (iVEPs) in monitoring visual function during endoscopic transsphenoidal surgery

ObjectiveTo refine a reliable and reproducible intraoperative visual evoked potentials (iVEPs) monitoring protocol during endoscopic transsphenoidal surgery. To assess the reliability of baseline iVEPs in predicting preoperative visual status and perioperative iVEP variation in predicting postoperative visual outcome.MethodsSixty-four patients harboring tumors of the pituitary region were included. All patients underwent endoscopic endonasal approach (EEA) with iVEPs monitoring, using a totally intravenous anesthetic protocol. Ophthalmological evaluation included visual acuity and visual field studies.ResultsPreoperatively, visual acuity was reduced in 86% and visual field in 76.5% of cases. Baseline iVEPs amplitude was significantly correlated with preoperative visual acuity and visual field (p = 0.001 and p = 0.0004, respectively), confirming the reliability of the neurophysiological/anesthetic protocol implemented.Importantly, perioperatively the variation in iVEPs amplitude was significantly correlated with the changes in visual acuity (p < 0.0001) and visual field (p = 0.0013). ROC analysis confirmed that iVEPs are an accurate predictor of perioperiative visual acuity improvement, with a 100% positive predictive value in patients with preoperative vision loss.ConclusionsiVEPs during EEA is highly reliable in describing preoperative visual function and can accurately predict postoperative vision improvement.SignificanceiVEPs represent a promising resource for carrying out a more effective and safe endoscopic transsphenoidal surgery.

Pituitary Tuberculosis - A Rare Case Report

Tuberculosis is an infectious disease primarily affecting lungs and also involves any body organ. Intracranial tuberculosis constitutes about 0.15-5% of all intracranial space-occupying lesions. Pituitary gland as primary site for tuberculosis is very rare. Delayed diagnosis and treatment can result in permanent endocrine dysfunction. Here we present a case in a 44-year-old female presented to the hospital with complaints of progressive worsening headache, left eye pain, and blurred vision for 6 months. Patient was evaluated and radiological workup was done. MRI brain with contrast showed enhancing mass lesion of 1.7x1.4 cm arising from Sella with mass effect on optic chiasma and it was radiologically diagnosed as pituitary adenoma. A biopsy from the pituitary region was obtained and histopathological examination was done. Microscopic examination revealed pituitary gland parenchyma showing well-formed epithelioid granulomas consisting of epithelioid cells, Langhans type of giant cells, and chronic inflammatory cells along with foci of caseous necrosis. The case was finally diagnosed as pituitary tuberculoma. Keywords: Tuberculosis, granuloma, space-occupying lesion, pituitary gland.

Lymphocytic hypophysitis in dogs infected with Leishmania spp.

Morphological involvement of endocrine glands, such as the pituitary gland, remain uninvestigated in dogs with canine visceral leishmaniasis. Therefore, this study investigated the presence of amastigotes of Leishmania spp. and characterized inflammatory changes, highlighting the involvement of TCD3+ lymphocytes in different regions of the pituitary gland of dogs. Samples were collected from 21 naturally infected dogs and 5 control, uninfected dogs. The different pituitary regions were analyzed in histological sections stained with hematoxylin and eosin (HE) under light microscopy. Inflammation was classified by intensity in a score from 0 to 3, absent (0), mild (1), moderate (2), and marked (3). The immunohistochemical (IHC) evaluation was performed in five high-power fields (hot spot) in a 40x objective of each region with manual counting (Image J1.52ª) of the TCD3+ lymphocytes and for amastigotes analyzed in 40x and 100x objectives. The Shapiro-Wilk test was used to assess the normality of the data. Differences between groups were determined by the Mann Whitney test. The correlation between variables was assessed by Sperman's correlation test. p < 0.05 were considered statistically significant. Amastigotes from the pituitary glands of two infected dogs were identified using IHC. The histopathological evaluation stained with hematoxylin and eosin showed greater intensity of inflammation in the pars distalis and pars intermedia regions of infected dogs. IHC for TCD3+ lymphocytes showed a higher median number of immunolabeled cells in pars nervosa in the infected group than in the control group (p < 0.05); and expecting a variation in the distribution and number of these cells in naturally infected dogs, the median of the control group was considered a cut-off point, an increase in T lymphocytes (p < 0.05) was also observed in the pars intermedia and pars distalis of an infected subgroup (n = 10). A moderate significant correlation between the intensity of inflammation and the number of immunolabeled TCD3+ lymphocytes was established in the analyzed pituitary regions, characterizing the occurrence of hypophysitis. These findings presuppose that inflammation and/or the parasite in the pituitary region can result in gland dysfunction, worsening the clinical condition of the patient and compromising the efficiency of treatment and prognosis.

P11.69.B INTRAVENOUS THROMBOLYSIS WITH OR WITHOUT ENDOVASCULAR TREATMENT FOR SUSPECTED ISCHEMIC STROKE IN PATIENTS WITH INTRACRANIAL TUMORS

Abstract BACKGROUND: AND OBJECTIVES Intravenous thrombolysis (IVT) for suspected ischemic stroke in patients with intracranial neoplasms is off-label. However, data on risks of intracranial hemorrhage (ICH) is scarce. METHODS In a multicenter registry-based analysis within the European Thrombolysis in Ischemic Stroke Patients (TRISP) collaboration, we assessed frequencies of symptomatic and fatal ICH after IVT for suspected ischemic stroke in patients with intracranial tumors by descriptive statistics and analyzed associations with clinical and imaging characteristics by binary logistic regression. Definition of symptomatic ICH was based on the clinical criteria of the European Cooperative Acute Stroke-II trial including hemorrhage at any site in cranial imaging and concurrent clinical deterioration. RESULTS Screening data of 21,289 patients from 14 centers, we identified 105 patients receiving IVT, among them 29 patients (28%) with additional endovascular treatment, with suspected, i.e. imaging-based, or histologically confirmed diagnosis of intracranial tumors. Among 104 patients with CT or MRI after IVT available, symptomatic and fatal ICH were observed in nine and four patients (9% and 4%). Among 82 patients with suspected or confirmed meningioma, symptomatic and fatal ICH occurred in six and three patients (7% and 4%). In 18 patients with intra-axial suspected or confirmed primary or secondary brain tumors, there was one symptomatic nonfatal ICH (6%). Out of four patients with tumors of the pituitary region, two patients (50%) had symptomatic ICH including one fatal ICH (25%). Tumor size was not associated with occurrence of symptomatic ICH (Odds ratio 2.8, 95% CI 0.3-24.8, p=0.34). CONCLUSION In our dataset from routine clinical care, we provide insights on the safety of IVT for suspected ischemic stroke in patients with intracranial tumors, a population that is commonly withheld thrombolysis in clinical practice and prospective trials. Except for a potential high risk of symptomatic ICH after IVT in patients with tumors of the pituitary region, frequencies of symptomatic ICH in patients with intracranial tumors in our cohort appear to be in the upper range of rates observed in previous studies within the TRISP cooperation. These results may guide individual treatment decisions in patients with acute stroke and intracranial tumors with potential benefit of IVT.

Comparison of perimetric 24-2 and 30-2 test patterns in detecting visual field defects in patients with tumours in the pituitary region.

Automated perimetry provides a standardized method of measuring the visual field. The Humphrey Field Analyser (HFA) uses the 24-2 test pattern to cover 24 degrees centrally or the 30-2 test pattern to cover a slightly broader region of 30 degrees. The aim of this study was to determine whether the 24-2 test pattern provides comparable information to the 30-2 test pattern in detecting visual field defects in patients with tumours in the pituitary region. A retrospective cohort study was carried out on patients with tumours in the pituitary region and radiologically confirmed compression of the visual pathway. Included patients (79 of 133) had been examined using the Humphrey 30-2 visual field test, after which the 30-2 test patterns were reduced into corresponding 24-2 test patterns. The location of visual field defects, visual acuity and the perimetric parameters mean deviation (MD) and visual field index (VFI) were also recorded. No patient was classified differently when evaluated with the 24-2 test pattern, compared to the 30-2 test pattern. Interestingly, although the majority of patients had visual field defects located in the temporal visual field of each eye, a significant minority did not. In addition, it was found that a large proportion of patients had normal visual acuity (≥0.8). The use of the HFA 24-2 test pattern reliably detected visual field defects in patients with tumours in the pituitary region. The present study indicates that MD and VFI are not reliable parameters for evaluating visual field defects due to compression.

Endoscopic four-hands technique for pituitary tumours.

Tumours in the pituitary fossa region can be resected by endoscopic transnasal surgery using a four-hands technique. The technique, which is atraumatic, safe and minimally invasive, should be the first-line treatment for pituitary tumours and certain skull-base tumours.

Comparison of neuroimaging features of histiocytic neoplasms with central nervous system involvement: a retrospective study of 121 adult patients

ObjectivesTo compare neuroimaging characteristics of three types of histiocytoses, namely Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), and Rosai-Dorfman disease (RDD), with central nervous system (CNS) involvement.MethodsA total of 121 adult patients with histiocytoses (77 LCH, 37 ECD, and 7 RDD) and CNS involvement were retrospectively included. Histiocytoses were diagnosed based on histopathological findings combined with suggestive clinical and imaging features. Brain and dedicated pituitary MRIs were systematically analyzed for tumorous, vascular, degenerative lesions, sinus, and orbital involvement and for hypothalamic pituitary axis involvement.ResultsEndocrine disorders, including diabetes insipidus and central hypogonadism, were more common in LCH patients than in ECD and RDD patients (p < 0.001). In LCH, tumorous lesions were mostly solitary (85.7%), located in the hypothalamic pituitary region (92.9%), and without peritumoral edema (92.9%), while in ECD and RDD, tumorous lesions were often multiple (ECD: 81.3%, RDD: 85.7%), their distribution was more widespread with meninges mostly involved (ECD: 75%, RDD: 71.4%), and they most likely presented with peritumoral edema (ECD: 50%, RDD: 57.1%; all p ≤ 0.020). Vascular involvement was an exclusive imaging characteristic of ECD (17.2%), which was not observed in LCH or RDD; this was also associated with a higher risk of death (p = 0.013, hazard ratio = 11.09).ConclusionThe typical characteristic of adult CNS-LCH was endocrine disorders with radiological findings limited to the hypothalamic pituitary axis. The pattern of multiple tumorous lesions with predominant involvement of meninges was the main manifestation of CNS-ECD and CNS-RDD, while vascular involvement was pathognomonic for ECD and associated with poor prognosis.Clinical relevance statementInvolvement of the hypothalamic-pituitary axis is the typical imaging characteristic of Langerhans cell histiocytosis. Multiple tumorous lesions, predominantly involving but not limited to meninges, occur in most Erdheim-Chester disease and Rosai-Dorfman disease patients. Vascular involvement occurs only in Erdheim-Chester disease patients.Key Points• The different distribution patterns of brain tumorous lesions can help differentiate among LCH, ECD, and RDD.• Vascular involvement was an exclusive imaging finding of ECD and was associated with high mortality.• Some cases with atypical imaging manifestations were reported to further expand the knowledge on these diseases.

Langerhans Cell Histiocytosis in Children and Adults: Similarities and Differences

Langerhans cell histiocytosis is an uncommon hematological condition affecting children and adults. It is distinguished by neoplastic infiltration of myeloid dendritic cells and an inflammatory response. Any organ can become dysfunctional as a result of tissue destruction and fibrosis. In almost all cases, activating mutations in the MAPK signal transduction pathway have been found. The earlier these mutations appear during myeloid differentiation, the more severe the disease. The skeleton, skin, and pituitary gland are most typically afflicted in children, but the liver, spleen, and hematopoietic system may also be involved, associated with a worse outcome. In adults, manifestations are most commonly observed in the skeleton, lungs, skin, and in the pituitary region. The course of the disease might range from spontaneous remission to persistent relapse. A histological examination is required to get a definitive diagnosis. In youngsters, where therapeutic surgery and radiation are mostly obsolete, medical therapy is the treatment of choice. Chemotherapy is used when special risk lesions are involved or when children have multifocal skeletal involvement and multisystem disease. In adults, however, local treatments are sufficient in unifocal lesions, and supportive therapy with bisphosphonate is suitable for multifocal bone involvement. Systemic therapy is administered only in patients with multisystem disease and treatment-refractory or quickly reoccurring disease activity. Aside from cytostatics, targeted inhibitors of gene mutations are now accessible for this purpose. While most patients survive, chronic recurrent episodes and late sequelae are the main issues.

Neurosarcoidosis Complicated by Systemic Vasculitis (P9-5.005)

<h3>Objective:</h3> To describe a rare case of a patient with neurosarcoidosis complicated by systemic vasculitis of medium-large vessels. <h3>Background:</h3> Sarcoidosis is an inflammatory systemic disease characterized by the presence of noncaseating granulomas in any organ, when it affects the nervous system is called neurosarcoidosis. Systemic vasculitis is an unusual complication of sarcoidosis but it has not been previously reported in neurosarcoidosis. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> A 62-year-old woman with a past medical history of hypertension, diabetes mellitus, coronary artery disease, neurosarcoidosis, s/p craniotomy (right cavernous sinus mass resection) was transferred to the hospital due to worsening headaches, generalized weakness, vomiting, and decreased oral intake for the last two weeks. On admission repeat brain MRI showed interval worsening of the known inflammatory right cavernous noncaseating granuloma, now extending into the right tentorial leaflet, right middle cranial fossa, and pituitary region, repeat vasculitis panel was negative, the patient was started on IV steroids and mycophenolate. However, the hospital stay was complicated by a syncopal episode, CT Head was normal but EKG showed a new T wave inversion with troponin elevation. Later on, the patient had worsening mentation and left-sided hemiparesis, CT head showed acute hypodensity in the right MCA territory and CTA showed bilateral stenosis of the distal right M1 and left M1segments of the MCAs, the patient was out of the window for tPA and was not a candidate for mechanical thrombectomy, she was started on aspirin. TTE and TEE did not show any intracardiac shunting/thrombi. Later on, she developed signs of ischemia in her right 1st and 2nd toes, aortogram showed partial and complete obstructions of both superficial femoral, anterior tibial, and peroneal arteries, and vascular surgery performed RLE SFA stent placement and recommended clopidogrel. IV cyclophosphamide was added with no further thromboembolic events. <h3>Conclusions:</h3> Vasculitis although uncommon can also occur in patients with neurosarcoidosis. <b>Disclosure:</b> Dr. Carvajal- Gonzalez has nothing to disclose. Dr. Arevalo has nothing to disclose. Dr. Chernyshev has nothing to disclose.

Hypopituitarism in Adults: Rational Approaches to Diagnosis and Treatment

AbstractPituitary hormones are responsible for the regulation of growth, development, metabolism, reproduction, and homeostasis. Hypopituitarism is a condition that is defined as partial or complete insufficiency of anterior pituitary hormone secretion, and rarely, posterior pituitary hormone secretion. This condition can result from diseases of the pituitary gland or the hypothalamus. The annual incidence of hypopituitarism has been estimated to be 4.2 per 100,000 yearly, and the prevalence has been estimated at 45.5 per 100,000. The symptoms of hypopituitarism vary. The onset is insidious and depends on the number of hormone deficiencies and their degree of severity. Pituitary hormone deficiency can result in substantial clinical changes that increase the risk of morbidity and mortality. People commonly report persistent symptoms and a decline in their quality of life, both of which can be explained, at least in part, by the inherent shortcomings of hormone replacement strategies in their ability to imitate the normal hormone secretion processes. The diagnosis of hypopituitarism can be straightforward by measuring the lowered basal hormone levels. In cases where the basal hormone levels are uncertain or partial hormone deficiencies have been identified, it may be necessary to perform provocative testing of the hypothalamic–pituitary axis. The hypothalamus and pituitary region can be imaged using magnetic resonance imaging, which provides useful anatomical information. When necessary, genetic studies may be added to the diagnostic approach. The treatment consists of physiological replacement of the individual end-organ hormone deficiencies, and careful monitoring is required throughout the patient's entire life. Individualized hormone replacement therapy that considers potential interactions is recommended. This article provides an overview of the pathophysiology, clinical presentation, general diagnostic guidelines, and treatment options of hypopituitarism.

Unusual circumstance for craniopharyngioma discovery on meningoencephalitis: a pediatric case report

BackgroundCraniopharyngioma is a rare condition in children, but it is the most frequent tumor that occurs in the hypothalamic pituitary region. Chemical meningitis has been described as an uncommon postoperative complication, but no chemical meningitis due to a spontaneous rupture leading to craniopharyngioma diagnosis in children has been reported.Case presentationThis is a case of a 13-year-old boy presenting with fever, vomiting and headache for two days. The CT scan revealed a suprasellar lesion, and lumbar puncture showed aseptic meningitis. The cerebral MRI suggested a craniopharyngioma and the cerebrospinal fluid cholesterol concentration was abnormally high. A thorough medical history indicated some visual disturbance, which improved at the onset of meningitis, and an inflection of the growth curve. The anatomopathological analysis of the tumor confirmed the diagnosis of craniopharyngioma.ConclusionsThis case is the first to report the discovery of a craniopharyngioma with meningoencephalitis caused by the rupture of a craniopharyngioma cyst in a child.Diagnosis was facilitated by determining the cholesterol level in the cerebrospinal fluid, as well as fine anamnesis to identify visual and growth disturbances.

AIRWAY MANAGEMENT IN NEUROANESTHESIA.

Neurosurgical patients have specific airway management. Various conditions and diagnoses make intubation difficult and may also cause neurological damage. Spinal pathology, neurotrauma, cervical spine surgery, and pituitary gland surgery are just some examples. The aim of this review article is to present a broad spectrum of neurosurgical operations and potential complications in maintaining airway patency related to these issues. Quality perioperative preparation is a prerequisite to avoid the potentially irreversible consequences of difficult airways with a poor neurological or even fatal outcome. Patients with tumors of the pituitary region who present with Cushing's disease are prone to difficult ventilation, tracheal obstruction and difficult intubation. Awake craniotomy is also a challenge for the anesthesiologist, given that access to the airway is problematic due to the fixed frame. Unstable cervical spine occurs in cases of rheumatoid arthritis or blunt trauma, requiring precautions to be taken with spinal stabilization during intubation and induction. Pharyngeal edema and hematomas, possible complications of cervical spine surgery can endanger airway patency after extubation; postoperative patient supervision is thus required. Due to the potential threat to the patient's airway during neurosurgical procedures, quality anesthetic preoperative preparation is necessary to avoid irreversible damage and death.