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Background and aims Intraoperative MRI (ioMRI) consists of performing a MRI during brain or spinal surgery. Although it is a safe and useful technique, it is available in a few hospitals. This means some aspects are not perfectly defined or standardized, forcing each center to develop its own solutions. Our goal is to describe the technique, evaluate the changes made to optimize its use and thus be able to facilitate the intraoperative resonance implementation in other neurosurgery departments. Methods A prospective analysis of patients consecutively operated using high-field ioMRI guidance was carried out, describing the type of tumor, clinical data, time and sequences of ioMR, use of intraoperative neurophysiology, preoperative tumor volume, after ioMR, and postoperative, as well as complications. Results ioMR was performed in 38 patients selected from among 425 brain tumors (9%) operated on in this interval. The tumor types were: 11 glioblastomas , 8 anaplastic astrocytomas , 5 diffuse astrocytomas, 4 meningiomas, 3 oligodendrogliomas , 2 metastases , 2 epidermoid cysts , 1 astroblastoma , 1 arachnoid cyst and 1 pituitary adenoma . The mean age was 45 years. The mean preoperative tumor volume was 45.22cc, after the ioMR 5.08cc and postoperative 1.28cc. Resection was extended after ioMR in 76%. Gross total resection was achieved in 15 patients and residual tumor of less than 1cc was observed in 8. An intentional tumor tissue was left in an eloquent brain region (mean volume 7cc) in 13 patients. Bleeding and ischemia complications were detected early on ioMR in 5%. MRI length was 47 min on average. Conclusions Intraoperative MRI was a useful and safe technique, and no associated complications were registered.

Objective The hypothalamic-pituitary-adrenal axis stimulation during pregnancy complicates the investigation of Cushing's syndrome. Our objective is to present a pregnant patient with Cushing syndrome caused by pituitary tumor in which the desmopressin stimulation test helped in the diagnosis and led to appropriate management. Case report A 27-year-old woman with 9-week gestation presented with proximal myopathy for 2 months. She had high blood pressure, wide abdominal purplish striae, and proximal myopathy. Her past medical history revealed hypertension and dysglycemia for 1 year. The 8 AM cortisol was 32.4 μ g/dL (5-18), late-night salivary cortisol at 11 PM was 0.7 μ g/dL ( μ g/day (21.0-143.0). The mean ACTH concentrations at 8 AM were 44.0 pg/mL (0-46.0). Non-gadolinium enhanced pituitary magnetic resonance imaging (MRI) reported no obvious lesion. The desmopressin stimulation test showed a 70% increase in ACTH levels from baseline after desmopressin administration. The pituitary MRI with gadolinium showed an 8x8x7-mm pituitary adenoma. Transsphenoidal surgery with tumor removal was done, which showed ACTH-positive tumor cells. After the surgery, the patient carried on the pregnancy uneventfully. Discussion During pregnancy, the ACTH level may not be an accurate marker to help in the differential diagnosis of Cushing's syndrome. Moreover, non-gadolinium pituitary imaging may not detect small pituitary lesions. Conclusion In the present Case, the desmopressin stimulation test suggested the diagnosis of Cushing's disease, which subsequently led to successful treatment. This suggested that the desmopressin test may serve as a useful test to diagnose Cushing's disease in pregnant individuals.

Growth hormone has a strong role in stimulation of the thymus. We report a case of thymic hyperplasia due to excess endogenous growth hormone in the setting of acromegaly. Acromegaly often presents with systemic manifestations that may be confused with a systemic hematologic malignancy or infection, especially if an anterior mediastinal mass is present but unrecognized as a benign thymic hyperplasia. It is important for radiologists to be aware of this association between growth hormone and thymic stimulation because it may increase confidence diagnosing thymic hyperplasia in this setting, and avoid unnecessary mediastinal biopsy or surgery.

PURPOSE: Prior studies on compressive optic neuropathy (CON) have come from large tertiary centers, which may contain referral bias toward more severe or atypical disease. To our knowledge, there are no studies to determine the population-based etiologies and clinical outcomes of compressive optic neuropathy (CON). This study aims to bridge that gap using the Rochester Epidemiology Project database. DESIGN: Retrospective, population-based cohort METHODS: Medical records of all residents living in Olmsted County, Minnesota from January 1, 2000 through December 31, 2018 were screened for CON. Demographic and clinical information were collected before and after treatment. RESULTS: 23 patients had a confirmed diagnosis of CON during our study period, which provided an overall incidence of 1.14 per 100,000 per year. Average age at onset of CON was 51 years (SD 24), and 39% were male. The most common etiologies were pituitary adenoma and meningioma. There was significant improvement in visual fields (p < 0.003) but not in visual acuity (p = 0.08) after patients underwent treatment for CON. There was also a significant relationship between the time until treatment and the degree of visual field improvement at follow-up (Pearson correlation rho = −0.58, p < 0.047). CONCLUSION: To our knowledge, this study provides the first population-based incidence of CON. The finding that earlier treatment leads to better visual outcomes stresses the importance of having CON on the differential diagnosis of patients with optic neuropathy.

Background / objective CHEK2 is a cell-cycle check point kinase and is part of the ATM-CHEK2-p53 cascade, which is protective against carcinogenesis. To describe a germline CHEK2 mutation in a patient with acromegaly and other tumors. Case report We present a woman with a germline CHEK2* 110delC mutation previously diagnosed with fibroadenoma of the breast and papillary thyroid carcinoma. She presented with acromegaly at age 48 [IGF-I: 556 mcg/L (reference range, 90-360) and lack of GH suppression on glucose tolerance testing], and underwent transsphenoidal resection of a somatotroph microadenoma. Four years after surgery, she developed recurrent GH excess. She was treated with cabergoline, which was discontinued due to intolerance, and transitioned to lanreotide depot, which was switched to pegvisomant because of prediabetes. Her IGF-I levels have remained normal on pegvisomant. Follow-up MRI examinations showed no evidence of tumor progression. Shortly after the diagnosis of acromegaly, the patient was diagnosed with endometrial carcinoma, bilateral ovarian cystadenomas and uterine leiomyomas. She was additionally found to have a non-functioning adrenal nodule, and hyperplastic and adenomatous colon polyps. There are multiple family members with malignancies, including colon, thyroid and lung cancer. Discussion This is a novel report of a patient with a pathogenic germline CHEK2 mutation and multiple malignant and benign tumors, including recurrent acromegaly. Conclusion Our data raise the possibility that CHEK2 mutations might be involved in the development of acromegaly. Additional studies are needed to elucidate the potential role of CHEK2 mutations in the pathogenesis of somatotroph adenomas.

Purpose This study evaluates the temporal evolution of the spatial relationship between the pituitary adenoma transsphenoidal surgical cavity and the adjacent optic chiasm and discusses implications on timing and choice of radiotherapy modality. Methods This retrospective observational review analyzed factors that might influence the postoperative relationship between the surgical cavity and the optic chiasm, including tumor type, craniocaudal tumor and resection cavity dimensions, the preoperative distance between tumor and the optic chiasm, the presence of cavernous sinus invasion, and the choice of intraoperative packing material. Changes observed on magnetic resonance imaging in the preoperative, immediate (within 72 h), and delayed (≥3 months) postoperative periods were compared. Results Sixty-five patient histories were analyzed. Preoperatively, the pituitary adenoma was apposed to the optic chiasm in 43 patients (66%). Postoperatively, 34 patients (52%) in the immediate postoperative period and 54 patients (83%) in the delayed postoperative period had a distance ≥2 mm between the resection cavity and the optic chiasm. This distance provides a greater margin of safety with adjuvant radiosurgery. Preoperative tumor size showed a strong association with postoperative descent of the optic chiasm. Conclusions Preoperative tumor size and degree of mass effect on the optic chiasm predict postoperative changes. In this study, the distance between the resection cavity and the optic chiasm was greater at ≥3 months postoperatively than in the immediate postoperative period, regardless of preoperative mass effect, indicating radiotherapy planning should be deferred to ≥3 months postoperatively when not precluded by aggressive histological characteristics that necessitate more immediate treatment. Precis To investigate the temporal relationship between the postoperative sellar surgical cavity and the adjacent optic apparatus after transsphenoidal resection of pituitary adenomas and the implications for radiotherapy.

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

Pituitary incidentalomas are discovered in approximately 10% to 40% of brain images. A complete patient history, physical examination, and dedicated pituitary function testing are needed, and subsequent results should lead to appropriate patient management. However, most lesions are asymptomatic pituitary adenomas or Rathke cleft cysts with a benign course. Many lesions can be clinically significant, including prolactinomas or other pituitary adenomas that warrant specific pituitary disease treatment. In other cases, mass effect causing visual compromise or refractory headache indicates a need for surgery. Here, various facets of a complex evaluation and treatment algorithm for pituitary incidentalomas are reviewed.

Considering intracranial tumours, only few indications of protontherapy, such as chordoma, chondrosarcoma or uveal melanoma, are uniformly approved in the world. Other indications, excluding paediatric pathologies, are still debated. The aim of this article is to describe the rationale for the use of protonbeam irradiation for meningioma, pituitary adenoma, craniopharyngioma, paraganglioma, glioma, and schwannoma, and to inform the radiation oncologists if prospective studies or randomized studies are opened for inclusions. This article deals only with indications for adults.

Multiple endocrine neoplasia (MEN) describes the occurrence of tumours affecting two or more endocrine glands in one patient. Two main forms are recognized: MEN type 1 (MEN1) and type 2 (MEN2). MEN1 is characterized by the combined occurrence of parathyroid, pituitary and pancreatic neuroendocrine tumours, whereas MEN2 features medullary thyroid cancer in association with phaeochromocytoma and parathyroid tumours. Although both MEN1 and MEN2 are autosomal dominant disorders, they have contrasting molecular aetiologies: MEN1 results from inactivating germline mutations of the MEN1 tumour suppressor gene, whereas MEN2 results from activating mutations in the RET proto-oncogene. The clinical features arising in each condition relate to the location of tumour development and/or hormonal hypersecretion, while treatment approaches aim to minimize morbidity and mortality, and preserve quality of life. Genetic testing is a key component of management, not only to confirm the diagnosis in affected patients, but also to identify family members who are at risk of disease but may be asymptomatic. It is recommended that patients ‘at risk' of developing MEN1 and MEN2 (i.e. mutation carriers) undergo periodic clinical, biochemical and radiological surveillance to enable the early identification and/or treatment of tumours. Here, a brief overview of MEN1 and MEN2 is provided.

Background Pituitary adenoma (PA) accounts for 10–15% of all intracranial neoplasms. Despite their benign nature, PA often shows invasive growth. MicroRNAs (miRNAs) and long non-coding RNAs (lncRNAs) are a class of non-coding RNAs that play important roles in PA initiation and progression. Aim The aim of this study was to find specific profiles of miR-200a and long non-coding RNA (lncRNA) antisense non-coding RNA in the INK4 locus (ANRIL) in PA based on a comparative study using Real-time quantitative reverse transcription polymerase chain reaction (qRT-PCR) analyses of tumor tissue and plasma. Methods Plasma and PA tissue samples were obtained from two groups of included patients (15 invasive and 15 non-invasive PA). In addition, plasma samples from patients with invasive PA have collected pre- and post-operation. Plasma and tissue samples subjected to qRT-PCR analyses for the expression levels of miR-200a and lncRNA ANRIL. Results The expression levels of miR-200a and lncRNA ANRIL were increased in tissue samples patients with invasive PA than in the patients with non-invasive PA. In addition, the expression levels of circulating miR-200a and lncRNA ANRIL were increased in patients with invasive PA than in patients with non-invasive PA in the pre-operation period. However, the expression level of plasma circulating miR-200a and lncRNA ANRIL was decreased in patients with invasive PA in the post-operation period. Our results depicted a miR-200a and lncRNA ANRIL expression in tissue and plasma samples in the patients with invasive PA. In addition, Receiver Operating Characteristic (ROC) curve was used to evaluate the diagnostic value of these circulating miR-200a and lncRNA ANRIL. Conclusion The expression of these tumor-associated ncRNAs has been elevated in the PAs. Therefore, miR-200a and lncRNA ANRIL represents as biomarkers for diagnosis and potential targets for novel invasive PA treatment strategies.

Objective Secondary adrenal insufficiency (sAI) is a severe endocrinologic complication associated with nonfunctioning pituitary adenoma (NFPA). However, its prevalence is not simply related to tumor size. In this study, we aimed to detect the clinical characteristics of NFPAs that cause sAI. Methods We retrospectively investigated the clinical data of 218 patients with newly diagnosed macro NFPA between April 2011 and March 2020. The patients for whom endocrinologists had prescribed hydrocortisone after comprehensive endocrinologic evaluation were defined as having sAI. The 7 clinical factors analyzed for association with sAI were age, sex, presence of neurologic symptoms, hospitalization for emergency management of pituitary apoplexy, degree of optic chiasm compression, and Knosp grades on both sides. Results Seventy-three patients (33%) were classified into the sAI group. Multinomial logistic regression showed the strongest correlation between sAI and Knosp grade on the less extending side (P = 0.0001), followed by sex (male) (P = 0.0013) and pituitary apoplexy (P = 0.098). Tumors that extended bilaterally and had Knosp grades of 1–3 were frequently observed in sAI and were common in males. Conclusions The NFPAs that occupy the sella space and compress the walls on both sides of the cavernous sinus, but do not penetrate them, have a higher risk of developing sAI. This type is more common in males and is seen even in patients without visual field disturbances. This clinical finding will be beneficial in management of patients with NFPA.

Objective Primary resection of adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma has become a front-line standard-of-care treatment for Cushing disease. However, surgical intervention can be challenging because of elevated blood pressure, as well as direct cortisol impacts on endothelial cells, vascular permeability, and tissue friability—potentially resulting in increased intraoperative bleeding. Tranexamic acid (TXA) is a well-studied, widely used intravenous hemostatic; however, the potential benefit during resection of ACTH-secreting pituitary adenoma is unstudied. The purpose of this study was to define an institutional protocol for perioperative administration of TXA in patients undergoing endoscopic endonasal approach for resection of ACTH-secreting pituitary adenoma, and to study the implementation of our novel protocol in a prospective fashion. Methods Criteria for preoperative TXA were defined by age, medical history, and risk factors. Descriptive statistics were reported for all patients receiving perioperative TXA. Results Thirty patients met inclusion criteria and underwent perioperative administration of TXA, using a standardized dosing protocol of a 10 mg/kg bolus in 30 minutes prior to incision, followed by maintenance infusion of 2 mg/kg/hour for the duration of the procedure. No incidence of myocardial infarction or postoperative thromboembolic events were noted. Subjective assessments indicated satisfaction with the patient selection protocol, and meaningful reduction in the extent of intraoperative bleeding. Conclusions Perioperative TXA represents a potentially efficacious approach for control of intraoperative bleeding during endonasal resection of ACTH-secreting tumors. Careful preoperative patient selection is emphasized, given the potential for thromboembolic complications; however, initial experience with our institutional protocol suggests a favorable risk/benefit profile when this treatment is applied judiciously.

Background Giant pituitary adenomas (GPA) with subarachnoid extension can be challenging to achieve a gross total resection (GTR) through a single endonasal or transcranial approach, and any residual tumor is at risk for postoperative apoplexy. Intraoperative venous congestion of the suprasellar tumor can occur following resection of the sellar tumor, limiting tumor descent, and leading to suprasellar residual. Objective We propose a technique for resecting the suprasellar component first, which we call the “second floor” strategy (SFS) for GPA. Methods A retrospective review from 2010-2020 identified 586 endoscopic endonasal approaches (EEA) for pituitary adenoma resection. We report the rate of post-operative apoplexy and describe the SFS technique used in two cases. Results Of 586 cases, two developed symptomatic postoperative apoplexy (0.3%), and a third transferred to our care after undergoing postoperative apoplexy. All three cases had subarachnoid extension of a pituitary adenoma, underwent EEA and had residual suprasellar tumor. All three had permanent morbidity due to the post-operative apoplexy including blindness, stroke, or death, despite undergoing reoperation. The SFS was used for reoperation on one of these patients and as a primary strategy in a fourth patient who presented with a GPA with subarachnoid extension. We describe the SFS technique and demonstrate it with a 2D operative video. Conclusion Post-operative apoplexy of residual adenoma is a rare but serious complication after GPA resection. The proposed SFS allows early access to the suprasellar tumor and may improve the ability to safely achieve a gross total resection without need for additional procedures.

Tumours growing in the sella turcica are mostly pituitary adenomas. We describe a rare case of primary intrasellar schwannoma with intratumoral bleeding. A 38-year-old man presented with headache in association with bilateral supratemporal quadrantopsia. MRI showed an intrasellar mixed signal mass lesion with suprasellar extension. The majority of the pituitary hormones were normal. He was diagnosed as non-functional pituitary adenoma with pituitary apoplexy. Subtotal resection was achieved eventually via an endoscopic transnasal trans-sphenoidal approach. The histopathologic diagnosis was schwannoma. It is the first intrasellar schwannoma with intratumoral hemorrhage in literature to date. It implied that the primary intrasellar schwannoma has potential risk of intratumoral bleeding, which should be considered in the differential diagnoses of sellar lesions.

Background Pituitary apoplexy (PA) is a rare, but life-threatening, condition characterized by pituitary infarction and hemorrhage, most often in the setting of a preexisting adenoma. The risk factors and mechanisms associated with PA are poorly understood. Although neurovascular manifestations of coronavirus disease 2019 (COVID-19) infection have been documented, its association with PA has not yet been determined. Methods From a prospectively collected database of patients treated at a tertiary care center for pituitary adenoma, we conducted a retrospective medical record review of PA cases during the COVID-19 pandemic from March 2020 to December 2020. We also conducted a literature review to identify other reported cases. Results We identified 3 consecutive cases of PA and concomitant COVID-19 infection. The most common symptoms at presentation were headache and vision changes. The included patients were successfully treated with surgical decompression and medical management of the associated endocrinopathy, ultimately experiencing improvement in their visual symptoms at the latest follow-up examination. COVID-19 infection in the perioperative period was corroborated by polymerase chain reaction test results in all the patients. Conclusions With the addition of our series to the literature, 10 cases of PA in the setting of COVID-19 infection have been confirmed. The present series was limited in its ability to draw conclusions about the relationship between these 2 entities. However, COVID-19 infection might represent a risk factor for the development of PA. Further studies are required.

Pure intrasellar meningioma is an extremely rare tumor that is difficult to diagnose as it resembles a pituitary adenoma in its clinical, endocrinological, and radiological presentation; it can only be distinguished through histopathological examination. All previously reported cases of this lesion were considered those of pituitary adenoma at the initial diagnosis. This poses major challenges in clinical diagnosis. We report a case of a pure intrasellar meningioma in a 27-year-old man. A boundary between the tumor and pituitary gland may be evident on magnetic resonance imaging, thus enhancing the diagnosis of pure intrasellar meningioma.

Background Cavernous sinus (CS) invasion is found in 15–20% of pituitary adenomas; it represents a poor prognosis factor and a surgical challenge even in experienced pituitary centers. We present our experience and technical note description for surgical management of pituitary adenomas with CS invasion in acromegaly by the transsellar lateral approach with an endoscopic endonasal transsphenoidal route. Method prospective case series of patients who underwent endoscopic endonasal surgery for Growing Hormone (GH) producing adenomas with CS invasion treated at the Neurosurgery departments of National Institute of Neurology and Neurosurgery in Mexico City, and of Toluca Medical Center of Social Security Institute of the State of Mexico and Provinces between January 2014 and March 2019. Results Thirty-two of 94 patients with diagnosis of pituitary adenoma treated at our institutions (34%) had acromegaly; thirteen of patients with acromegaly diagnosis met the inclusion criteria for CS invasion. Postoperative images reported gross total resection in 10 patients (76.9%). Mean follow-up time was 28.3 months. Remission criteria were achieved in nine patients (69.2%), with one of these patients (11.1%) having recurrence during follow up. All patients with no biochemical remission had improvement in GH and IGF profiles. Three patients without remission underwent radiosurgery (14 Gy), and one patient had remission after the procedure. Conclusions We consider this to be a safe and efficient approach for tumors invading CS, when surgical team have good experience in endoscopy of the skull base and reconstruction techniques, appropriate instruments are available, and tumor has soft consistency.

Objective Acromegaly is a syndrome of metabolic alterations secondary to increased growth hormone levels from a somatotroph pituitary adenoma. A multidisciplinary approach beginning with surgery, followed by adjuvant radiation or medical therapy for residual disease, is considered standard of care. Several factors affect the likelihood of remission after surgery, but the impact of surgical experience on remission rates has not been adequately assessed. Methods Retrospective review of 203 patients, divided into 2 eras (era 1, 102 patients; era 2, 101 patients) of patients who underwent transsphenoidal surgery for acromegaly by a single surgeon over 11 years, was performed, determining the effect of surgical experience on rates of remission and various complications. Remission was defined according to the 2014 Endocrine Society Clinical Practice Guideline. Results The rate of surgical remission was 40.6% (62.9% among noninvasive adenomas). Rates of surgical remission significantly improved in the latter half of this cohort (31.2% in the first half vs. 50% in the second half), despite other factors being comparable. On multivariate analysis, surgeon experience, cavernous sinus invasion, and preoperative growth hormone levels affected the rates of surgical remission. Rates of cerebrospinal fluid leak and hypopituitarism were lower in the second half, whereas resolution of acromegaly-associated comorbidities was increased. Conclusions We report, in this large single-surgeon review of endoscopically operated acromegaly cases, increased rates of surgical remission and reduced complications with increasing surgeon experience. The overall experience of the treating team in dealing with perioperative and intraoperative factors also contributes to improved outcomes.

Background Gonadotroph pituitary adenoma (Gn-PA) may rarely cause ovarian hyperstimulation syndrome, leading to infertility in women, although this remains poorly described. Methods We present a quantitative systematic review including 2 patients from our institutional and 48 from the literature with Gn-PA causing ovarian hyperstimulation syndrome to thoroughly describe the clinical features and therapeutic outcomes from multidisciplinary aspects. Results The patients had a mean age of 31.5 years and a mean follicle-stimulating hormone level of 14.4 IU/L. Estradiol level was high in 82% of patients, at >350 pg/mL. The mean maximal adenoma diameter was 22 mm, with a Knosp grade ≥3 in 10 patients. Abdominal surgery preceded adenoma resection in 24 patients (48%). Among 25 patients for whom extent of resection was recorded, total adenoma resection was achieved in 12. Through a mean follow-up of 25 months, adenoma recurrence was observed in 5 patients, who were treated with re-resection (n = 2), radiation (n = 2), and medical therapy followed by bilateral oophorectomy (n = 1). Medical therapies were partially effective or ineffective, and adenoma shrinkage did not follow; gonadotropin-releasing hormone agonists/antagonists were partially effective in 20% of patients (2/10), dopamine agonists in 44% (8/18), and somatostatin analogues in 50% (1/2). Four experienced swelling of tumor/ovaries after gonadotropin-releasing hormone agonists/antagonists administration. Overall, chemical remission was obtained in 26 of 28 patients, normalization of ovaries in 25 of 27, and successful pregnancy in 12 of 14. Conclusions Adenoma resection is the main treatment, leading to reduction in ovarian size and biochemical remission, with a high likelihood of subsequent spontaneous pregnancy. Increased awareness of this rare condition may help avoid unnecessary abdominal procedures.

Objectives Fluorescence-guided surgery may improve completeness of resection in transsphenoidal surgery for Cushing disease (CD) by enabling visualization of residual tumor tissue at the margins. In this review we discuss somatostatin receptors (SSTRs) as targets for fluorescence-guided surgery and overview existing SSTR-specific imaging agents. We also compare SSTR expression in normal pituitary and corticotrophinoma tissues from human and canine CD patients to assess canines as a translational model for CD. Methods A PubMed literature search was conducted for publications containing the terms canine, somatostatin receptor, Cushing's disease, and corticotroph adenoma. SSTR expression data from each study was documented as the presence or absence of expression or, when possible, the number of tumors expressing a given SSTR subtype within a group of tumors being studied. Studies that used reverse transcription polymerase chain reaction to quantify SSTR expression were selected for additional comparative analysis. Results SSTR5 is strongly expressed in human corticotroph adenomas and weakly expressed in surrounding pituitary parenchyma, a pattern not conclusively observed in canine patients. SSTR2 mRNA expression is similar in human normal pituitary and corticotrophinoma cells but may be significantly higher in canine normal pituitary tissue than in corticotroph tumoral tissue. Limited data were available on SSTR subtypes 1, 3, and 4. Conclusions Further studies must fill the knowledge gaps related to species-specific SSTR expression, so using canine CD as a translational model may be premature. We do conclude that the expression profile of SSTR5 (i.e., high local expression in pituitary adenomas relative to normal surrounding tissues) makes SSTR5 a promising molecular target for FGS.

Internal carotid artery (ICA) stenosis rarely occurs in pituitary apoplexy. Little is known of the causes of this condition. The present study investigated the factors related to ICA stenosis associated with pituitary apoplexy.Forty-five patients with pituitary apoplexy were retrospectively examined and divided into the stenotic and normal ICA groups. The baseline characteristics of patient background, tumor properties, clinical findings, and treatment overview were compared between the groups.Eight patients were assigned to the stenotic ICA group and 37 to the normal ICA group. Patient age in the stenotic ICA group was significantly lower than that in the normal ICA group (P = 0.001). Maximum tumor diameter (P = 0.001), tumor volume (P = 0.044), and Knosp grade (P0.001) were significantly greater in the stenotic ICA group than in the normal ICA group. The stenotic ICA group had a significantly greater incidence of sphenoid sinus mucosal thickening than the normal-ICA group (P = 0.039). Multivariate logistic regression analysis demonstrated that age (odds ratio 0.915, 95% confidence interval 0.846-0.991, P = 0.029) was a significant and independent predictor of ICA stenosis associated with pituitary apoplexy. Receiver operating characteristic curve analysis showed that the optimal cut-off point for age was 35.0 years (specificity 0.946, sensitivity 0.750).Our study revealed that age, tumor size, and sphenoid sinus mucosal thickening were strongly related to the occurrence of ICA stenosis in pituitary apoplexy. Among these factors, age had the potential of being an independent predictor of the condition.

Rathke cleft cysts (RCCs) are benign sellar lesions originating from remnants of primitive ectoderm. They have not been previously linked to other cystic lesions, such as pineal cysts (PCs). Our objective was to perform a multicenter cross-sectional neuroimaging study to examine prevalence rates of coexisting RCC and PC.We retrospectively queried prospectively maintained, institutional review board-approved, databases from the authors' centers. All patients undergoing transsphenoidal surgery for RCC between the years of 2011 and 2020 were included for analysis. Preoperative magnetic resonance imaging was reviewed to identify the coexistence of a PC. Patient demographics and neuroimaging characteristics were recorded. A control cohort comprised of 100 age- and sex-matched patients with nonfunctional pituitary adenoma (NFPA) who also underwent surgical intervention was utilized.Eighty-four patients with RCC were identified for analysis. A coexistent PC was identified in 40.5% (n = 34) of patients with RCC compared with 14.3% (n = 12) in the NFPA cohort (P0.001). There was no significant difference in PC size between patients with RCC and PA (8 vs. 8.8 mm, respectively; P = 0.77). Although the majority (85.7%; n = 72) of the RCC cohort were female patients, there was no sex predominance with respect to coexisting PC in either the RCC or PA cohort.This is the first study to report an increased prevalence of coexisting PC and RCC, possibly because of an embryologic link or other propensity for intracranial cyst formation. Additional studies in more generalizable populations can further explore the relation between RCC and PC, or other cyst formation.

Objective The collision of pituitary adenoma and craniopharyngioma is extremely rare and thus there remains a paucity of data. Methods We described a patient from our institution. We also performed a systematic review and subsequent quantitative synthesis of the literature (n = 21) and our institutional case to yield an integrated cohort, and a descriptive analysis was carried out. Results Twenty-two patients (15 males and 7 females) were included in the integrated cohort. The median age was 47.0 years (range, 8–75 years). The tumor subtypes were 5 somatotropic, 5 lactotropic, 4 nonfunctioning, 3 gonadotropic, 2 corticotropic, 1 plurihormonal, and 1 silent subtype 3 for pituitary adenomas, and 19 adamantinomatous, 2 papillary, and 1 unknown subtype for craniopharyngiomas. Three different radiographic patterns were observed: solid mass with cystic component (n = 5), coexistence of two distinct solid components (n = 3), and a mixed-intensity solid mass (n = 5). The first 2 were consistent with histologically separate collision, whereas the third was consistent with histologically admixed collision. Among 19 patients in whom the postoperative course was recorded, a secondary intervention was required in 14 (73.7%) because of tumor progression or residual. The recurrence rate after gross total resection was 33.3%. Postoperative hormone replacement was required in 33.3%. The 10-year cumulative overall survival was 73.1%. Conclusions Most craniopharyngiomas were adamantinomatous. There are 2 types of collisions: separated and admixed. Tumor control, overall survival, and endocrinologic remission are more challenging to achieve than for solitary tumors, but gross total resection of both tumors is important for satisfactory tumor control.

Background The direct endoscopic endonasal approach (EEA) has become the primary technique used for resection of sellar pathology, meriting investigation into the risk factors for complications and predictors of postoperative outcomes after direct EEA. Methods We retrospectively analyzed the patient and tumor characteristics from 404 patients who had undergone direct EEA for sellar pathology at the USC Pituitary Center from September 2011 to December 2019. Results Of the 404 pathologic entities included, 349 (86%) were pituitary adenomas (PAs), 29 (7%) were Rathke cleft cysts, and 26 (6%) were other sellar lesions. The mean lesion diameter was 2.3 cm, with 34 microadenomas (10%) and 315 macroadenomas (90%). Cavernous sinus invasion was present in 39% of the PAs. No patient died. The surgical complications included internal carotid artery injury without neurological sequelae (0.2%), vision loss (0.7%), meningitis (0.7%), cerebrospinal fluid leak (4%), epistaxis (4%), sinusitis (1%), transient cranial nerve paresis (0.5%), and postoperative abscess (0.25%). New hypopituitarism developed in 3%. Gross total resection was achieved in 208 PA cases (58%). Clinical improvement of headaches and visual deficits were reported for 67% and 76% of cases, respectively. Hormonal remission was achieved in 82% of patients with functional PAs. The median hospital stay was 2 days, with 34 patients (8%) readmitted within 30 days and 10 (3%) undergoing early reoperation. Disease recurrence or progression developed in 10% and was less likely in the case of gross total resection and apoplexy. Conclusion In the present, large, consecutive, mostly single-surgeon series, the patients experienced clinical improvement in most preoperative symptoms and had low rates of perioperative morbidity. We have demonstrated that direct EEA can be efficiently, safely, and successfully performed by a neurosurgical team.

Objective We report a case of pituitary apoplexy (PA) with negative radiographic findings for PA and cerebrospinal fluid (CSF) analysis consistent with neutrophilic meningitis. PA is a rare endocrinopathy requiring prompt diagnosis and treatment. Presentation with acute neutrophilic meningitis is uncommon. Methods The diagnostic modalities included pituitary function tests (adrenocorticotropic hormone, thyroid-stimulating hormone, luteinizing hormone, prolactin), brain computed tomography and magnetic resonance imaging (MRI), and CSF analysis. Results A 67-year-old man presented with worsening headache, nausea, and retching. He was somnolent with an overall normal neurologic examination other than a peripheral vision defect in the left eye. MRI showed a pituitary mass bulging into the suprasellar cistern with optic chiasm elevation, consistent with pituitary macroadenoma. Laboratory evaluation revealed decreased levels of adrenocorticotropic hormone, random cortisol, thyroid-stimulating hormone, thyroxine, luteinizing hormone, and testosterone. He had worsening encephalopathy with left eye ptosis and decreased vision, prompting a repeat computed tomography and MRI, showing no interval change in the pituitary adenoma or evidence of bleeding. CSF analysis revealed a leukocyte count of 1106/mm3 (89% neutrophils), a total protein level of 138 mg/dL, red blood cell count of 2040/mm3 without xanthochromia, and glucose level of 130 mg/dL. The CSF culture result was negative. Transsphenoidal resection revealed a necrotic pituitary adenoma with apoplexy. Conclusions Including PA in the differential diagnosis of acute headache is important, particularly in patients with visual disturbances. PA can present with sterile meningitis, mimicking acute bacterial meningitis. While neuroimaging can help detect PA, the diagnosis of PA remains largely clinical.

Objective Despite the efforts made to determine the achieved resection grade after pituitary adenoma surgery, there is a high level of disagreement among all the available classifications and measurement methods used. Our objective is to identify the factors that preoperatively could predict a gross total resection (GTR) of a clinically nonfunctioning pituitary adenoma through an endoscopic endonasal approach. Methods Across 100 surgeries, we analyzed epidemiologic and clinical data, radiologic relevant data, extent of resection (EOR), and postoperative outcomes. The EOR was measured objectively through an accurate volumetric analysis. Results The median presurgical volume was 8.58 cm3 (range, 0.5–58 cm3), the median maximum diameter was 27.3 mm (range, 7–67 mm), and the Knosp grade was 0 in 1 patient, 1 in 23%, 2 in 31%, 3 in 23% and 4 in 22% of patients. In the multivariate logistic regression analysis, we found 3 factors that significantly predicted the chances of a successful GTR: previous sellar surgery, Knosp grade, and tumor signal in the T2-weighted magnetic resonance imaging scan. Another 10 radiologic variables were analyzed and had no effect on the EOR. Conclusions Knosp grade (P

Objective Nelson syndrome (NS) is a rare clinical disorder that can occur after total bilateral adrenalectomy (TBA), performed as a treatment for Cushing disease. NS is defined as the accelerated growth of an adrenocorticotropic hormone-producing pituitary adenoma. Our objective is to describe a case of NS and discuss it based on existing knowledge of this syndrome. Methods We describe the case of a woman diagnosed with NS at our facility in the Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran and review published cases of NS. Results The patient, a 35-year-old woman with Cushing disease, had been diagnosed in 2006 at the endocrinology department in the Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran. In 2007, a laparoscopic TBA was performed, and 2 years later, she presented with hyperpigmentation and adrenocorticotropic hormone levels of up to 11 846 pg/mL. NS was suspected, and as magnetic resonance imaging showed macroadenoma, transsphenoidal surgery was performed. The patient remained asymptomatic until 2012, when she presented with a right hemicranial headache, photophobia, and phonophobia. A fresh magnetic resonance imaging was performed, which documented tumor growth. She was referred to the Instituto Nacional de Neurologia y Neurocirugia, where she underwent surgery. Conclusion NS develops as a complication of TBA, which is used as a treatment of Cushing disease. The main treatment is surgery and radiotherapy.

Acromegaly is a rare, chronic disorder that mostly results from growth hormone (GH)-secreting pituitary adenoma. Transsphenoidal surgery is the first-line treatment of this adenoma. This study aimed to identify factors associated with remission outcome in patients with GH-secreting pituitary adenomas following transsphenoidal surgery.Patients with GH-secreting pituitary adenomas who underwent transsphenoidal surgery for tumor removal at Songklanagarind Hospital between January 2003 and December 2019 were retrospectively reviewed. The primary outcome was the remission of disease at the last follow-up using 2000 and 2010 consensus criteria. Using logistic regression analysis, various factors were analyzed for association with disease remission outcome.This study included 51 patients. The remission rate of GH-secreting pituitary microadenomas and macroadenomas following transsphenoidal surgery were 100% and 43.75%, respectively. Multivariate analysis showed that preoperative insulin-like growth factor 1 index ≥2.5 and Knosp classification grade 3-4 were significantly associated with nonremission outcome (P0.001 and P = 0.012, respectively). Patients with both of these factors had poor outcomes and never achieved remission after treatment, while patients with neither of these factors had high remission rates (87.5%) following surgery. Four of 6 (66.7%) patients who underwent repeat surgery gained remission.Preoperative insulin-like growth factor 1 index ≥2.5 and Knosp classification grade 3-4 were important prognostic factors that determined remission outcome after treatment. Patients who have both of these poor prognostic factors should be aggressively treated with surgery, medication, and probably radiation to optimally control the disease.

The aim of this study was to compare radiosurgical treatment plan quality of a linear accelerator with Leksell Gamma Knife (LGK) for pituitary adenoma irradiation. Thirty pituitary adenoma patients were evaluated in this study. Treatment plans were prepared on LGK and stereotactic linear accelerator Varian TrueBeam STx. Volumetric Modulated Arc Therapy (VMAT) plans (21 plans with 2 coplanar arcs and 9 plans with 4 non-coplanar arcs) were calculated for linear accelerator. All the plans were evaluated in terms of conformity, selectivity, gradient index and organ at risk (OAR) sparing. VMAT produced dosimetrically comparable treatment plans to LGK regarding conformity and selectivity (New Conformity Index (NCI): 1.76 ± 0.65 for 4 arc VMAT, 2.33 ± 1,16 for 2 arc VMAT and 1.96 ± 0.71 for LGK; Selectivity Index (SI): 0.63 ± 0.16 for 4 arc VMAT, 0.51 ± 0.16 for 2 arc VMAT and 0.58 ± 0.17 for LGK). Gradient index (GI) was superior for LGK plans (GI: 2.74 ± 0.20 for LGK and 5.28 ± 2.29 for 4 arc VMAT). OAR sparing for optics, brainstem, and hypophysis was similar for both modalities while target volume coverage was maintained the same. Finally, treatment time resulted in favor of VMAT plans (in this study VMAT plans were almost 5 times faster than LGK treatment regarding beam on time). According to the results of this study stereotactic linear accelerator with VMAT treatment could be used as a reasonable alternative to LGK for pituitary adenoma radiosurgery but only if the same head fixation method accuracy and target volume delineation are maintained for both modalities.

The pituitary gland is a small endocrine organ located within the sella turcica. Various pathologic conditions affect the pituitary gland and produce endocrinologic and neurologic abnormalities. The most common lesion of the pituitary gland is the adenoma, a benign neoplasm. Dedicated MR imaging of the pituitary is radiologic study of choice for evaluating pituitary gland and central skull region. Computed tomography is complimentary and allows for identification of calcification and adjacent abnormalities of the osseous skull base. This review emphasizes basic anatomy, current imaging techniques, and highlights the spectrum of pathologic conditions that affect the pituitary gland and sellar region.

International audience; Gadolinium-based contrast agents (GBCAs) are used in up to 35% of magnetic resonance imaging (MRI) examinations and are associated with an excellent safety profile. Nevertheless, two main issues have arisen in the last two decades: the risk of nephrogenic systemic fibrosis and the risk of gadolinium deposition and retention. As a first step, this article reviews the different categories of GBCAs available in neuroradiology, their issues, and provides updates regarding the use of these agents in routine daily practice. Recent advances in MRI technology, as well as the development of new MRI sequences, have made GBCA injection avoidable in many indications, especially in patients with chronic diseases when iterative MRIs are required and when essential diagnostic information can be obtained without contrast enhancement. These recent advances also lead to changes in recommended MRI protocols. Thus, in a second step, this review focuses on consensus concerning brain MRI protocols in 10 common situations (acute ischemic stroke, intracerebral hemorrhage, cerebral venous thrombosis, multiple sclerosis, chronic headache, intracranial infection, intra- and extra-axial brain tumors, vestibular schwannoma and pituitary adenoma). The latter allowing the standardization of practices in neuroradiology. Recommendations were also made concerning the use of GBCAs in neuroradiology, based on evidence in the literature and/or by consensus between the different coauthors.

In patients with residual or recurrent nonfunctioning pituitary adenomas (NFPAs) after transsphenoidal resection, both GammaKnife (GKRS) and CyberKnife (CKRS) stereotactic radiosurgery (SRS) are viable treatment options.We report a retrospective single center series comparing assessing the effectiveness and complications from of these 2 commonly used SRS techniques.A total of 53 patients with prior surgical resection and residual or recurrent NFPAs who underwent GKRS or CKRS and minimum 3-month follow-up between January 2002 and February 2017 at a single center were identified.A total of 34 patients underwent GKRS and 19 received CKRS. CKRS patients had a larger maximal tumor diameter (P = 0.005) and tumor volume treated (P = 0.001). Differences between GKRS and CKRS treatment parameters included target volume, target volume treated, prescribed dose, maximum dose, prescription isodose line, and conformity index (P0.05). The mean follow-up time was 53.74 months for GKRS and 41.48 months for CKRS patients. Tumor progression developed in 6% of cases after GKRS versus 5% after CKRS. The mean progression-free survival was 48.44 months after GKRS and 38.57 months after CKRS (P = 0.61). Five-year actuarial tumor control rates were 91% after GKRS versus 89% after CKRS (P0.99). There were no differences in worsened vision or rates of hypopituitarism.In patients undergoing single fraction GKRS versus fractionated CKRS for NFPAs, both modalities had similar rates of tumor control, new hypopituitarism, and visual morbidity despite varying indications. This study validates the versatile use of these 2 SRS modalities for patients meeting their relative criteria, especially based on proximity to the optic apparatus and normal pituitary gland.

International audience; We report a case of malignant melanoma revealed by a metastasis to the pituitary gland. The tumor was misdiagnosed as a pituitary adenoma and aggressive transsphenoidal surgery was complicated by a cerebrospinal fluid fistula. Nine weeks later, the patient presented multiple leptomeningeal and brain metastases spreading from the sellar region. Regarding these observations, we conducted a systematic review of the literature in order to investigate clinicoradiological features that should lead clinicians to suspect pituitary metastasis and how it should impact the surgical management.

Background Anatomic compression of the optic nerve secondary to a dolichoectatic cerebrovascular compression is a rare clinical entity. Because of the limited number of published cases and variable clinical presentation, the natural history remains ambiguous and no consensus exists regarding management. In addition, there is an ongoing debate regarding whether a dolichoectatic cerebral blood vessel can actually cause optic neuropathy, or it merely represents an incidental finding. As a result, it is thought that a diagnosis of compressive optic neuropathy from an adjacent dolichoectatic internal carotid artery (ICA) should be considered only after other possible etiologies are excluded. Although this might seem straightforward, the clinical scenario becomes complex if the patient is also found to have additional incidental pituitary lesions. Such coexistence has not been reported previously in the literature. Case Description A 52-year-old left-handed man presented to us with intermittent headache and painless progressive visual deterioration in the right eye for 1 month. Screening magnetic resonance imaging (MRI) scan revealed a 9-mm eccentrically placed pituitary adenoma with right optic nerve compression because of dolichoectatic ICA. He underwent microvascular decompression of the right optic nerve. On follow-up, significant vision improvement was noticed and MRI scan revealed no change in the size of the pituitary adenoma. Conclusions The compression of the optic nerve by dolichoectatic ICA is commonly thought to be a diagnosis of exclusion. However, the presence of a coexisting pathology should not prompt the exclusion in every case and a case-based approach is highly recommended to correctly manage this rare clinical condition.

Background Spindle cell oncocytomas are extremely rare neoplasms of the sellar, parasellar, and suprasellar regions that can frequently mimic pituitary adenomas. Fewer than 50 cases have been ever reported in the literature, and there is no consensus on best treatments to be provided. Case Description We hereby present a challenging case of sellar and suprasellar spindle cell oncocytoma in a patient of 64 years. The patient, who presented with hydrocephalus, hypopituitarism, and visual deficit, underwent urgent transsphenoidal (TNS) resection of the mass, which was aborted for massive life-threatening bleeding. The patient received ventriculoperitoneal shunt with relief of symptoms. An endovascular embolization of tumor feeders from the distal portion of the right internal maxillary artery, in particular the sphenopalatine artery, was then performed and a second-look TNS surgery was feasible. The patient was discharged in optimal clinical condition, recovered both endocrinologic and visual deficits, and is now in follow-up. Conclusions We found that the oncocytoma was radiologically and clinically comparable with a pituitary adenoma, except for higher representation of vasculature. According to our recent experience and review of the literature, we believe that surgery (transsphenoidal or transcranial approach) is the recommended treatment in those who are symptomatic and preoperative embolization might be a suitable option to reduce intraoperative bleeding and increase radicality.

Sellar metastases account for 0.87% of all intracranial metastases. They are usually asymptomatic and can be the first manifestations of some occult malignancy. The diagnosis is made mainly during the screening of patients with known primary lesions or can present with neurologic or hormonal changes related to compression or invasion of surrounding structures. Differentiating these lesions from other more common lesions such as pituitary adenoma maybe difficult. Management is mainly aimed at the primary lesion and is palliative to improve quality of life or for pathologic confirmation.

The 2017 (fourth edition) World Health Organization Classification of Endocrine Tumors has recommended major changes in classification of tumors of the pituitary gland and region. In addition to the accurate tumor subtyping, assessment of the tumor proliferative potential (mitotic and/or Ki-67 index) and other clinical parameters such as tumor invasion is strongly recommended in individual cases for consideration of clinically aggressive adenomas. It is expected that this new WHO classification will establish more uniform biologically and clinically groups of pituitary tumors and contribute to understanding of clinical outcomes for patients harboring pituitary tumors.

Pituitary tumors are common and require complex and sophisticated procedures for both diagnosis and therapy. To maintain the highest standards of quality, it is proposed to manage patients in pituitary tumors centers of excellence (PTCOEs) with patient-centric organizations, with expert clinical endocrinologists and neurosurgeons forming the core. That core needs to be supported by experts from different disciplines such as neuroradiology, neuropathology, radiation oncology, neuro-ophthalmology, otorhinolaryngology, and trained nursing. To provide high-level medical care to patients with pituitary tumors, PTCOEs further pituitary science through research publication, presentation of results at meetings, and performing clinical trials.

Background Factors associated with visual outcomes after transcranial surgery for large-to-giant pituitary adenomas have not been fully elucidated. Methods We recruited 37 patients with large-to-giant pituitary adenomas between January 2014 and December 2016 and assessed their tumor characteristics and surgical outcomes. Visual acuity and visual field were evaluated by visual impairment score before and 3–6 months after transcranial surgery. Multivariable logistic regression analysis was applied to show the factors associated with visual outcomes after surgery. Results The severity levels of visual impairment before surgery were mild, moderate, severe, and complete in 24.3% (9/37), 24.3% (9/37), 35.1% (13/37), and 16.2% (6/37), respectively. After surgery, the visual function was improved, stabilized, and worsened in 43.2% (16/37), 43.2% (16/37), and 13.5% (5/37) of patients, respectively. Multivariable logistic regression analysis showed that subarachnoid space invasion was the only independent prognostic factor adversely influencing the postoperative visual outcomes. Patients with subarachnoid space invasion had a higher possibility of visual deterioration (36.4% vs. 3.8%; P = 0.021) after transcranial surgery, compared with those without subarachnoid space invasion. Conclusions Visual compromise is still an unignorable complication during transcranial surgery for large-to-giant pituitary adenomas. Subarachnoid space invasion indicated by preoperative magnetic resonance imaging was an independent negative predictor for visual outcomes after surgery.

Background A novel viral strain known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has created a worldwide pandemic known as coronavirus 2019 (COVID-19). Early reports from China have highlighted the risks associated with performing endoscopic endonasal skull base surgery in patients with SARS-CoV-2. We present a rare complication of nasoseptal flap (NSF) necrosis associated with COVID-19, further emphasizing the challenges of performing these procedures in this era. Case Description A 78-year-old man underwent an extended endoscopic endonasal transplanum resection of a pituitary macroadenoma for decompression of the optic chiasm. The resulting skull base defect was repaired using a pedicled NSF. The patient developed meningitis and cerebrospinal fluid (CSF) leak on postoperative day 13, requiring revision repair of the defect. Twelve days later, he developed persistent fever and rhinorrhea. The patient was reexplored endoscopically, and the NSF was noted to be necrotic and devitalized with evident CSF leakage. At that time, the patient tested positive for SARS-CoV-2. Postoperatively, he developed acute respiratory distress syndrome complicated by hypoxic respiratory failure and death. Conclusions To our knowledge, this is the first reported case of NSF necrosis in a patient with COVID-19. We postulate that the thrombotic complications of COVID-19 may have contributed to vascular pedicle thrombosis and NSF necrosis. Although the pathophysiology of SARS-CoV-2 and its effect on the nasal tissues is still being elucidated, this case highlights some challenges of performing endoscopic skull base surgery in the era of COVID-19.

To systematically evaluate the sella morphology and pituitary gland height on brain MRI of Chiari II malformation (C2M) patients to understand the observed high incidence of apparent enlargement of the pituitary gland.Brain MRIs of C2M patients at a single tertiary care adult institution were retrospectively reviewed. We also evaluated two age and gender-matched control groups-patients with normal brain MRI (C1 group) and chronic ventricular shunts (C2 group). The heights of tuberculum sella, dorsum sella, and pituitary gland were measured and compared. The presence or absence of dural thickening was noted.21 patients were included in each group. In C2M group, a pituitary adenoma was suggested on 24% of the MRIs. The dorsum sella was significantly smaller in the C2M group (4.8 mm) compared to both the C1 group (7.4 mm, p 0.001) and the C2 group (7.1 mm, p 0.001). The pituitary gland was also larger in the cranial-caudal dimension in C2M group (8.6 mm) as compared to both the C1 group (6.6 mm, p 0.01) and the C2 group (6.0 mm, p 0.001). One C2M patient with a pituitary gland10 mm was suggested to have a pituitary adenoma on outside MRI, although a normal pituitary gland was seen on pathology.C2M patients have shallow sella which can accentuate the pituitary gland height. The mean pituitary height was larger in C2M patients, but no functional pituitary pathology was present. It is important to consider sella morphology when evaluating the pituitary gland of C2M patients to avoid unnecessary medical and surgical interventions.

Background Pituitary adenomas are the most common lesion of the sellar region. Very few cases in the literature have described their association with craniopharyngiomas in the same anatomic compartment, an entity defined as collision tumors of the sella. Case Description A 35-year-old man presented with headaches and progressive visual disturbances. Radiographic imaging initially highlighted the presence of a pituitary craniopharyngioma. An endoscopic transsphenoidal pituitary approach was performed, during which the tumor was partially resected. The pathology report was positive for 2 entities: a nonfunctioning pituitary adenoma and a papillary craniopharyngioma. This was an unexpected diagnosis based on the surgical and initial radiologic findings. Conclusions To our knowledge, this is the first documented case of a collision tumor of the sella comprising a pituitary adenoma and a craniopharyngioma of the papillary type.

Background Xanthomatous hypophysitis (XH) is the rarest histologic type of primary hypophysitis. It is nonlymphocytic and characterized by an infiltration of the pituitary gland by lipid-laden histiocytes and macrophages. The clinical and radiologic features overlap heavily with pituitary adenomas and are prone to misdiagnosis. We describe a rare case of XH recurrence at 1 year, treated surgically. Moreover, we provide an updated review of the literature to further elucidate useful management and detection strategies. Case Description A 45-year-old woman presented with a history of menstrual irregularity for 9 months, amenorrhea, galactorrhea, and headache for 2 months duration. Preoperative endocrinologic studies showed increased prolactin levels. Magnetic resonance imaging of the sella showed a cystic lesion with suprasellar extension suggestive of a pituitary adenoma. The patient underwent transsphenoidal resection, showing a thick yellowish colloidal material. Histopathology showed necrotic tissue with no definitive diagnosis and no identified microorganisms. At 2 months after surgery, prolactin levels and menstrual cycle normalized. At 1 year after surgery, her menstrual cycle again became irregular. Repeat magnetic resonance imaging showed a recurrent mass and a second transsphenoidal resection was undertaken. Repeat histopathology was consistent with XH. The patient is 9 years postoperative and is doing well without evidence of recurrence. Conclusions XH presents similarly to nonfunctional adenomas and diagnosis remains difficult without surgical pathology, requiring meticulous immunohistochemistry to prevent misdiagnosis. Thus, XH should be considered as a rare cause in the differential of disease of the sellar region. Management can parallel that of pituitary neoplasm, with a focus on radiosurgery, re-resection, and high-dose steroids.

The present prospective cohort study evaluates the effect of three-dimensional (3-D) endoscopy on outcome in transphenoidal endoscopic surgery of pituitary adenomas compared to conventional two-dimensional (2-D) endoscopy. Prospective data was collected from patients undergoing endoscopic surgery for pituitary adenomas before and after the introduction of 3-D endoscopy. Patients, grouped by having 2-D or 3-D endoscopic surgery, were compared in regard to procedure time, intraoperative blood loss, complications, hospital stay, grade of resection and quality of life (QoL). Twenty-six patients having surgery with 2-D endoscopy were compared with 29 patients having surgery with 3-D endoscope. Only primary procedures were included. There were no significant differences in baseline characteristics between the two groups. No statistically significant differences in outcome were noted with 3-D endoscopy. Procedure time, complication rate, hospital stay, rate of gross total resection and post-operative QoL were unaffected by surgical technique though there were non-significant increases in new pituitary insufficiency with 3-D endoscopy and diabetes insipidus with 2-D endoscopy. This prospective cohort study fails to show obvious outcome advantages with 3-D endoscopy in pituitary surgery using basic parameters including post-operative QoL. To our knowledge this is the first prospective study published on the matter, thus corroborating results from previous retrospective studies with similar results on 3-D neuroendoscopy and 3-D endoscopy in general. The main advantage of increased depth perception is more likely found in more complex extended transphenoidal skull base procedure.

Background Crooke cell adenoma is a very rare subtype of pituitary neoplasm that is known to be clinically aggressive. These tumors can secrete adrenocorticotropic hormone or may be endocrinologically silent. We evaluated the effect of Gamma Knife radiosurgery (GKRS) on endocrine remission and tumor control. Materials and Methods This study comprised 5 patients (2 men, 3 women; median age at GKRS, 55 years [range, 21–65 years]) with pathology-confirmed Crooke cell adenoma treated with GKRS at the Gamma Knife Center of the University of Virginia. The median time interval between transsphenoidal resection and GKRS was 5.8 months. The median margin dose was 25 Gy (range, 18–25 Gy). Median treated adenoma volume was 3.12 mL. Median follow-up was 107 months (range, 44–122 months). Results Tumor control was achieved in all patients. Three patients achieved endocrine remission at the last follow-up. The median time interval to cortisol normalization when off of anti–hormone secreting medication was 12 months (range, 6–24 months). Newly developed or worsening endocrinopathy occurred in 3 patients at 6, 15, and 18 months, respectively. Cranial nerve III neuropathy developed in 1 patient. Two patients required bilateral adrenalectomy at 44 months and 50 months, respectively, following GKRS. Conclusions GKRS appears to be a safe and reasonably effective treatment option for Crooke cell adenoma. Multicenter studies with larger numbers of patients are needed to verify these findings.

Background Pituitary apoplexy is an acute clinical syndrome caused by infarction and/or hemorrhage of pituitary adenoma, which typically presents with severe headache, visual deterioration, and endocrine abnormalities. However, temporal lobe seizure (and temporal lobe epilepsy) has not been viewed as a symptom of pituitary apoplexy in the literature. Case Description To elucidate further such a rare complication of temporal lobe seizure, we describe here the rare clinical manifestations of a 55-year-old previously healthy man with pituitary apoplexy harboring headache, combined palsies involving cranial nerves III to VI, endocrinologic disturbances, and temporal lobe seizure. In addition, we discuss the temporal lobe seizure (and temporal lobe epilepsy) associated with pituitary adenoma based on the literature. Conclusions Although further accumulation of clinical data is needed, we would like to emphasize the importance of recognition of temporal lobe seizure caused by pituitary apoplexy, and to suggest that early surgery could be considered as an option in patients displaying such a rare complication.

Objectives To evaluate long-term olfactory outcomes in patients who underwent pituitary surgery through the endoscopic endonasal transsphenoidal approach (EETSA) by T&T olfactometer. Methods We retrospectively reviewed 26 patients who underwent pituitary surgery via EETSA. Olfactory function was assessed by T&T olfactometer before and 6 months after surgery. The mean of recognition thresholds for five different odorants was used. The change in the mean recognition threshold values was evaluated in the entire cohort and the subgroup analysis was performed according to the age, sex, past history of pituitary surgery (primary surgery or revision surgery), histopathology (non-functioning adenoma (NFA) or functioning adenoma (FA)), reconstruction procedure (rescue flap or nasoseptal flap), and superior turbinate management (preserved or resected). Results Of the 26 patients (12 men and 14 women, median age 53 years), 21 patients were newly diagnosed with pituitary gland tumor (16 NFAs, 5 FAs) and the remaining 5 were diagnosed with recurrent pituitary gland tumor (4 NFAs and 1 FA). In the whole cohort, the mean recognition threshold values of T&T olfactometer significantly improved after surgery (P = 0.01). Thirteen out of 26 patients (50%) showed olfactory improvement, whereas only 3 (12%) showed deterioration. In the subgroup analysis, olfactory function outcomes were not significantly different between the subgroups with respect to the age, sex, past history of pituitary surgery, histopathology, reconstruction procedure, or superior turbinate management. The olfactory function tended to worsen in the revision surgery group compared to that in the primary surgery group, but not significantly (P = 0.06). Conclusions The olfactory function was improved or maintained after pituitary surgery via EETSA in 88% of patients, indicating the benefits of low invasiveness of our surgical treatment. On the other hand, three patients (12%) demonstrated deterioration of olfactory function, suggesting that the risk of postoperative olfactory dysfunction should be informed to patients.