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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.

Background Pituitary apoplexy is a rare clinical syndrome. Only a few studies have examined factors associated with recovery of neuro-endocrine functions following transsphenoidal surgery. This study aimed to identify factors associated with neuro-endocrine recovery following surgery for pituitary apoplexy. Methods The records of pituitary apoplexy patients who underwent transsphenoidal surgery at Songklanagarind Hospital between January 2005 and December 2020 were retrospectively reviewed. The primary outcomes were the recoveries of preoperative visual acuity (VA), visual field (VF), cranial nerve function, and pituitary hormone deficits. Using logistic regression analysis, various factors were analyzed for their associations with recovery of neuro-endocrine functions. Results The study included 98 patients. Multivariate analysis showed that older age and greater suprasellar extension were associated with no recovery of VA (P = 0.042 and P = 0.018, respectively). Only 33% of patients aged > 55 years and suprasellar extension of lesion ≥ 25 mm gained recovery of VA, while 100% of patients with neither of these factors had recovery. Underlying hypertension was associated with no recovery of preoperative VF defect (P = 0.027). Wilson-Hardy classification-invasion grades 3-4 and lower preoperative serum prolactin level were associated with no recovery from preoperative hypoadrenalism and hypothyroidism (P = 0.016 and P = 0.007, respectively). Conclusions Older age, higher suprasellar extension, and hypertension were poor prognostic factors for visual recovery. Wilson-Hardy inferior invasion grade 3-4 and lower preoperative serum prolactin level were poor prognostic factors for recovery from hypopituitarism. Stratifying patients according to these prognostic factors may assist in selecting patients for surgery.

Pituitary abscesses are very uncommon. They are divided into primary, arising within a healthy gland, and secondary, observed with an underlying pre-existing lesion. Here we present the eighth case reported of a secondary abscess within a craniopharyngioma. A 59-year-old-woman presented with a 3-week history of headache, and fever. Physical examination was unremarkable. An Magnetic Resonance Imaging (MRI) showed a pituitary lesion suggestive of a chronic inflammatory process. She was diagnosed with lymphocytic meningitis with hypophysitis and she was treated with corticosteroids. Two months later she presented with headache and fever again. Control MRI showed enlargement of the pituitary lesion. Therefore, a transsphenoidal biopsy was performed. During the procedure, purulent material was released. Histological study demonstrated a craniopharyngioma and meningeal inflammation. Empiric antibiotics were started. Three months post-operatively, a follow-up MRI showed a suspect minimal residual mass. Secondary pituitary abscesses are rare. The key to successful management is a high index of suspicion. Transsphenoidal surgical evacuation plus antibiotics is the mainstay of treatment. Although most symptoms resolve, endocrinopathies improve only rarely.

Background Cushing disease (CD) during pregnancy is a rare but serious disease that adversely impacts maternal and fetal outcomes. As the sole use of metyrapone in the management of CD has been rarely reported, we describe our experience of using it to treat a pregnant patient with CD. Case Report A 34-year-old woman with hypertension was diagnosed with adrenocorticotropic hormone–dependent CD on the basis of a urinary free cortisol (UFC) level of 290 μg/24 h (reference range, 6-42 μg/dL) and an abnormal dexamethasone suppression test (cortisol level, 12.4 μg/dL) before becoming pregnant. She conceived naturally 12 weeks after transsphenoidal surgery and was subsequently found to have persistent disease with a UFC level of 768 μg/dL. Surgery was deemed high-risk given the proximity of the tumor to the right carotid artery and the high likelihood of residual disease. Instead, she was managed with metyrapone throughout her pregnancy and titrated to a goal UFC level of Discussion This case highlights the successful use of metyrapone throughout pregnancy to manage CD in patients in whom surgery is considered high-risk or in those with a low likelihood of cure. Although metyrapone is effective, close surveillance is required for worsening hypertension, hypokalemia, and potential adrenal insufficiency. Although no fetal adverse events have been reported, this medication crosses the placenta, and the long-term effects are unknown. Conclusion We describe a case of CD during pregnancy that was successfully treated with metyrapone.

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.

Pituitary tumors and subsequent treatment with endoscopic transsphenoidal surgery (ETSS) may cause injury to suprasellar structures, causing long-term fatigue and neurocognitive impairment. A method to quantify brain injury after ETSS is not available. In this prospective, exploratory study of patients undergoing ETSS for pituitary tumors, a novel approach to detect possible neuronal damage is presented. Plasma concentrations of brain injury biomarkers (glial fibrillary acidic protein [GFAP], tau, and neurofilament light [NFL]) were measured the day before surgery, immediately after surgery, at day 1 and 5, and at 6 and 12 months after surgery, using enzyme-linked immunosorbent assays. The association between the increase of biomarkers with preoperative tumor extension and postoperative patient-perceived fatigue was evaluated. Suprasellar tumor extension was assessed from MRI scans, and self-perceived fatigue was assessed using the Multidimensional Fatigue Inventory before and 6 months after surgery. Thirty-five patients were included in the analysis. Compared to baseline, GFAP showed a maximal increase at day 1 after surgery (p = 0.0005), tau peaked postoperatively on the day of surgery (p = 0.019), and NFL reached its maximum at day 5 after surgery (p < 0.0001). The increase in GFAP correlated with preoperative chiasmal compression (p = 0.020). The increase in tau was correlated with preoperative chiasmal (p = 0.011) and hypothalamus compression (p = 0.016), and fatigue score 6 months after surgery (p = 0.016). In conclusion, the concentrations of brain injury biomarkers in blood increased after ETSS for pituitary tumors. The results indicate that postoperative plasma GFAP and tau might reflect astroglial and neuronal damage after ETSS.

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.

Introduction Pituitary surgery has shifted in recent years from microscopic surgery(MS) to endoscopic endonasal surgery (EES). However, the comparative long-term outcome of these surgical approaches, including the need for subsequent re-operation has never been reported. We present our experience in a high-volume referral center experienced in both endoscopic and microscopic approaches to compare the need for re-operation after initial resection of non-functioning pituitary macroadenomas using these surgical approaches. Methods 684 patients (398 with NF adenomas) underwent trans-sphenoidal pituitary surgery in our institution between 2006 and 2017. Complete follow-up (mean 72 months, minimum two years) was available in 87 newly diagnosed patients with non-functioning pituitary macroadenomas (NFPMA; 48-microscopic and 39-endoscopic). The EES approach has been used almost exclusively since 2012. The need for repeat operation for tumor resection during the follow-up period was assessed as the primary end-point of the study. Extracted data included various demographic and clinical parameters, radiographic findings as well as the extent of resection (EOR). Results The EOR was similar for both groups, with a trend towards better EOR in the EES group. The rate of surgical complications was also similar for both groups. There was a strong trend towards lower need for re-operation in the EES group compared to the MS group (12.8% vs. 29.2%, p = 0.056). In a multivariate analysis, only EOR and Knosp grade were independently associated with the need for re-operation surgery. Conclusion Our data indicate that EES in NFPMA tends to be associated with a lower need for re-operation compared to the MS approach, with a similar rate of EOR and complications.

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.

Objective To discuss optimal treatment strategy for spindle cell oncocytoma (SCO) of the pituitary gland. Methods Institutional cases were retrospectively reviewed. A systematic literature search and subsequent quantitative synthesis were performed for further analysis. The detailed features were summarized and the tumor control rate (TCR) was calculated. Results Eighty-five patients (6 institutional and 79 literature) were included. The annual incidence was approximately 0.01–0.03/100,000. The mean age was 56 years. Vision loss was present in 60%. Seventy-three percent showed hormonal abnormalities. On magnetic resonance imaging, tumor was avidly enhancing, and the normal gland was commonly displaced anterosuperiorly. Evidence of hypervascularity was seen in 77%. Gross total resection (GTR) was achieved in only 24% because of its hypervascular, fibrous, and adhesive nature. The mean postoperative follow-up was 3.3 years for institutional cases and 2.3 years for the integrated cohort. The TCR was significantly better after GTR (5-year TCR, 75%; P = 0.012) and marginally better after non-GTR + upfront radiotherapy (5-year TCR, 76%; P = 0.103) than after non-GTR alone (5-year TCR, 24%). The TCRs for those with low Ki-67 index (≤5%) were marginally better than those with higher Ki-67 index (5-year rate, 57% vs. 23%; P = 0.110). Conclusions Frequent endocrine-related symptoms, hypervascular signs, and anterosuperior displacement of the gland support preoperative diagnosis of SCO. GTR seems to have better long-term tumor control, whereas the fibrous, hypervascular, and adhesive nature of SCO makes it difficult to achieve GTR. In patients with non-GTR, radiotherapy may help decrease tumor progression.

Objective To examine demographic, clinical, and biochemical differences in patients with adrenocorticotropin (ACTH)-dependent Cushing syndrome (CS) based on etiology, sex, and tumor size. Methods This was a single-center study of 211 patients with ACTH-dependent CS followed for 35 years. Patients were stratified into 3 groups based on etiology: Cushing disease (CD)/transsphenoidal surgery, Cushing disease/total bilateral adrenalectomy (CD/TBA), and ectopic ACTH secretion (EAS). Patients were also stratified based on sex and tumor size (nonvisualized, microadenoma, and macroadenoma). Results CD was the commonest cause of ACTH-dependent CS (190; 90%). Most patients presented in the third decade (median age, 29 years). Clinical features, cortisol, and ACTH were significantly greater in the EAS group. The CD/TBA group had more nonvisualized tumors (22% vs 8%; P = .000) and smaller tumor size (4 vs 6 mm; P = .001) compared with the CD/transsphenoidal surgery group. There was female predominance in CD (2.06:1) and male predominance in EAS (2:1). Men had shorter duration of symptoms (2 years; P = .014), were younger (23 years; P = .001), had lower body mass index (25.1 kg/m2; P = .000), and had more severe disease (low bone mineral density, hypokalemia). Macroadenomas were frequent (46; 24.2%), and ACTH correlated with tumor size in CD (r = 0.226; P = .005). Conclusion Our cohort presented at an earlier age than the Western population with a distinct, but slightly lower, female predilection. Patients with CD undergoing TBA had frequent negative imaging. Men had a clinical profile suggesting aggressive disease. Microadenoma and macroadenoma were difficult to distinguish on a clinicobiochemical basis.

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.

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.

Background Although most patients with Rathke's cleft cysts (RCCs) remain asymptomatic throughout their lives, visual impairment in RCCs usually indicates surgical interventions, including endoscopic drainage of the cyst. We report a case of RCC with unique findings in the optic nerve root (ONR) by magnetic resonance imaging (MRI). Case Description A 58-year-old man admitted to our hospital complained of progressive left visual impairment. Preoperative computed tomography MRI revealed a suprasellar cystic lesion that extended anteriorly, and the bilateral ONRs were lateralized with the cyst and were partially enhanced with gadolinium administration. The cyst content was drained, and the cyst wall was partially removed by endoscopic transsphenoidal surgery. Postoperatively, the patient was administered corticosteroid intravenously for 3 days, and his visual acuity improved dramatically. Postoperative MRI revealed that the volume of the cyst decreased, and the position of the bilateral ONRs normalized. Additionally, enhancement of the ONRs with gadolinium administration was not observed. Histological examination of the surgical specimen revealed a ciliated single-layer columnar epithelium with inflammatory cell infiltration. Conclusions To our knowledge, we report the first case of a patient with RCC with ONR enhancement with gadolinium administration on MRI. This unique finding might prove that inflammation is one of the causes of visual impairment in RCC as in optic neuritis.

Objective: Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a heterogeneous condition caused by neuroendocrine neoplasms (NENs) located in the lungs, thymus, or pancreas. Our purpose was to evaluate the long-term outcome of these patients. Methods: Retrospective study at a referral center. The charts of 164 patients with Cushing syndrome, followed at our center from 1993 to 2019, were analyzed. Results: EAS was found in 16 patients (9.75%, 9 women, mean age 36.01 years) who had been followed for a median of 72 months. The source of EAS was a NEN in 10 patients (8 bronchial and 2 thymic carcinoid tumors) and a mixed corticomedullary tumor, consisting of a pheochromocytoma and an adrenocortical carcinoma in 1 patient. In 2 of the 6 patients initially considered to have occult EAS, the source of the ACTH excess became apparent after adrenalectomy, whereas in the remaining 4 (25%) patients, it has remained occult. Of the 11 patients in whom resection of the NEN was attempted, 10 patients achieved an early remission (91%), but 4 (25%) of these patients had a recurrence during follow-up (biochemically and clinically silent in 2 patients). Three patients died (18.75%): the young woman with the mixed corticomedullary tumor, a man with a thymic NEN that evolved into a neuroendocrine (NE) carcinoma after 11 years of follow-up, and a woman with a bronchial NEN. Conclusion:The course of EAS varies according to tumor type and grade. Some patients have a protracted course, whereas others may evolve into neuroendocrine carcinomas. Abbreviations: ACTH = adrenocorticotropic hormone; CS = Cushing syndrome; CT = computed tomography; CV = coefficient of variation; EAS = ectopic ACTH syndrome; IQR = interquartile range; NEN = neuroendocrine neoplasm; SCCL = small cell carcinoma of the lung; TSS = transsphenoidal surgery; UFC = urinary free cortisol

Summary The aim of the study was to document an anatomical variation around the sella turcica which was unique and lacked any reference in the literature. A low lying osseous bar connecting the right middle clinoid process to the midpoint of the right lateral border of the dorsum sella was observed in a human skull specimen. The osseous bar was forming a foramen close to the floor of the sella, and the structure in association with this part of sella is the inferior intercavernous sinus. Measurements of the sella turcica bridge (STB), carotico–clinoid foramen (CCF), interclinoid foramen (ICF) on both sides and that of the ossified bar and foramen formed by itself were taken with the help of ImageJ software. The values observed for the measurements of different parameters of STB and its components, for CCF and ICF were bilaterally different. While the values for the osseous bar and foramen formed by it cannot be compared because of its unilateral occurrence. The presence of foramen around the inferior intercavernous sinus may lead to difficulty in performing procedures like transsphenoidal surgery (TSS) or extended TSS (eTSS) while extracting intradural lesions, thus increasing the chances of haemorrhage.

Background The endoscopic endonasal approach (EEA) has been accepted as an alternative option for diseases at the craniovertebral junction. However, the inferior destination through the endoscopic endonasal approach is anatomically higher than that of the transoral approach. Therefore, preoperative assessment of accessibility is mandatory for appropriate selection of indication. Using a navigation system, we examined the inferior limit through the endonasal route and evaluated the relationships between surrounding anatomicl structures and the lowest point. Methods This study included patients who underwent endoscopic transsphenoidal surgery for intrasellar lesions at our hospital (N = 23). At the start of surgery, the lowest point (target point [TP]) was marked with a straight probe under guidance of the navigation system. We measured 4 parameters on preoperative computed tomography: nasal length, hard palate length, anterior-posterior diameter of the nasopharynx, and nasopalatine angle. Patients were classified into groups depending on whether the TP was at or above (group A) or below (group B) the hard palatine line. Results TPs were above the hard palatine line in 15 patients (group A) and below the hard palatine line in 8 patients (group B). No TPs reached the nasopalatine line. Nasal length (P = 0.03) and nasopalatine angle (P = 0.01) were larger in group B than in group A. There were no significant differences in anterior-posterior diameter of the nasopharynx or hard palate length. Conclusions The hard palatine line is a reliable parameter for assessing the inferior limit of the endoscopic endonasal approach. Nostril size affects accessibility with surgical instruments.

Transsphenoidal surgery (TSS) is a first-line treatment for Cushing disease (CD). However, a subset of patients with CD have no visible adenoma on magnetic resonance imaging (MRI), and whether MRI results affect surgical outcomes is controversial. The aim of this study was to compare the surgical outcomes of CD patients with negative MRI findings to those of patients with positive MRI findings.The clinical features and outcomes of CD patients who underwent TSS between January 2000 and July 2019 at Peking Union Medical College Hospital were collected from medical records. The clinical, endocrinologic, histopathologic, surgical outcomes, and a minimum 12-month follow-up of 125 consecutive CD patients with negative MRI findings were compared with those of 1,031 consecutive CD patients with MRI-visible adenomas.The total remission rate was 73.3% after TSS, and 11.8% of patients experienced recurrence. Of 1,031 patients with MRI-visible adenomas, postoperative remission was achieved in 762 patients (73.9%), and the recurrence of CD was observed in 94 (12.3%) patients. Of the 125 patients with negative MRI findings, postoperative remission was achieved in 85 (68%) patients, and recurrence was observed in 6 (7.1%) patients. The remission rate and recurrence rate were not significantly different between patients with negative MRI findings and those with positive MRI findings (all P.05). The remission rate was not significantly different between patients who did or did not undergo bilateral inferior petrosal sinus sampling (BIPSS) in patients with negative MRI findings (P.05). In the patients with negative MRI findings who underwent BIPSS, the remission rate of patients with positive BIPSS results was not different from that in patients with negative BIPSS results (P.05). The lack of prior TSS, the detection of a tumor during operation, and pathologic confirmation of adenoma were associated with a higher surgical remission rate in patients with negative MRI findings (all P.05). Similar results were observed in the patients with positive MRI findings (all P.05). In addition, the major perioperative complications, including intraoperative cerebrospinal fluid leakage, hypopituitarism, and transient diabetes insipidus, were not related to the MRI results (all P.05).The remission rate and recurrence rate were not different between patients with negative MRI findings and those with positive MRI findings. If CD is clearly diagnosed according to biochemical tests, radiologic examinations, and BIPSS, we recommend TSS as the first-line treatment for patients, even if the MRI results are negative.

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 Posterior reversible encephalopathy syndrome is manifested by a reversible neurologic deficit such as vision loss, encephalopathy, and a posterior location, typically the occipital lobes. It is commonly thought to be related to acute, severe hypertension. Case Description A 51-year-old woman presented with visual loss for several months, and a suprasellar mass was diagnosed. She underwent transsphenoidal surgery, which was complicated by cerebrospinal fluid leak, and she developed posterior reversible encephalopathy syndrome while undergoing postoperative cerebrospinal fluid drainage via lumbar catheter. Her visual acuity progressed to blindness, but blindness was reversed by discontinuation of lumbar drainage, tight blood pressure control, and high-dose steroid drip. Conclusions To our knowledge, this is only the second case of posterior reversible encephalopathy syndrome following transsphenoidal surgery to be reported in the neurosurgical or ophthalmic English language literature.

Background Steroid-related spinal epidural lipomatosis (SEL) is a well-known condition most commonly encountered in patients taking chronic steroid medications. Endogenous Cushing syndrome is a rare cause of SEL. The authors report an unusual presentation of acute thoracic myelopathy due to SEL with osteoporotic vertebral compression fractures in a patient with Cushing disease. Case Description A 33-year-old man presented with thoracic myelopathy due to SEL and multiple vertebral compression fractures. He had Cushingoid clinical features, and investigations revealed a pituitary microadenoma as the cause of hypercortisolism. The diagnosis was missed on multiple occasions before the onset of paraparesis. The patient was treated with urgent thoracic laminoplasty followed by transsphenoidal surgery on the same day. Although normalization of the serum cortisol levels was delayed by 6 months, he had a complete neurologic recovery and remains symptom free at 2 years follow-up. Conclusions This is a rare presentation of Cushing disease and has been reported only twice before in the 1990s. This case highlights the need to keep a high degree of suspicion for development of thoracic myelopathy in a patient with Cushing syndrome who develops thoracic compression fractures associated with SEL. Early recognition of this clinical association and addressing the primary endocrine problem urgently could have prevented spinal cord involvement and the subsequent spine surgery. This scenario is also documented in previous published case reports of this association where the diagnosis of Cushing syndrome has been revealed retrospectively after the development of neurologic symptoms.

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 Pituitary melanoma metastases (PMMs) are extremely rare and only a few cases are reported in the literature. PMMs can grow rapidly and present local invasiveness, leading to acute onset of neurological symptoms such as headache, visual and oculomotion disorders or endocrinological signs such as diabetes insipidus and hypopituitarism, and can be life-threatening. For this reason, PMMs must be recognized and treated promptly. Case Description The authors present 2 cases of PMMs managed at their institution, performing a review of the dedicated literature and analyzing current therapeutic strategies.

Background Recent molecular investigations for craniopharyngiomas have investigated possible predictive biologic markers. Growth hormone receptor (GHR) is thought to be involved in tumor aggressiveness, and high expression of GHR is associated with shorter duration of postoperative stable disease. Case Description A 27-year-old man with a large suprasellar tumor underwent an inexplicable clinical course. Transsphenoidal surgery achieved gross total removal of the tumor. Histologic diagnosis was adamantinomatous craniopharyngioma, and immunohistochemistry revealed very low GHR expression. He was discharged with multiple hormonal supplements except for growth hormone. Recurrence was detected 18 months later, and removal of the second tumor was performed with coagulation of the superior surface of the remaining pituitary gland. Growth hormone supplementation was started in the postoperative period, and he returned to work. However, a further recurrence was detected. Head magnetic resonance imaging showed almost the same pattern of tumor recurrence as preoperative imaging of the second surgery, and simultaneous removal of the tumor and the normal pituitary gland was performed. Reevaluation of histology revealed no morphologic differences between the first and the third surgical specimens, but immunohistochemical staining for GHR showed diffuse high expression in the third specimen. The difference was thought to reflect the heterogeneity of GHR, and appearance of histologic hot spots greatly affected the postoperative prognosis. Conclusions Extensive removal of the possible tumor bed may be necessary for patients requiring growth hormone supplementation even after gross total removal of craniopharyngioma.

Background Tension pneumoventricle is an extremely rare, but treatable, neurosurgical emergency. The prompt and accurate diagnosis of tension pneumoventricle requires vigilance for the detection of clinical signs, which should also be corroborated by the imaging findings. We have reported on the pathophysiology of tension pneumoventricle and its management. Case Description A 66-year-old woman had presented with a Rathke cleft cyst. The patient underwent transsphenoidal surgery (TSS), with no clinical cerebrospinal fluid leakage observed peri- or postoperatively. However, she developed an altered mental status 8 hours after surgery, and a computed tomography scan showed evidence of a tension pneumoventricle. The patient underwent emergent external ventricular drainage insertion and exploratory endoscopic TSS. A 1-way valve was observed during TSS, and the sella floor was packed with a fat graft for 1-way valve obliteration. The patient recovered well without neurologic deficits. No radiologic regrowth was noted at the 48-month follow-up examination. Conclusions Tension pneumoventricle is an extremely rare, but life-threatening, complication of TSS. The development of tension pneumoventricle should be kept in mind even when the surgery has proceeded very smoothly. Sellar reconstruction will, theoretically, prevent this extremely rare complication but might increase the recurrence rate of Rathke cleft cysts. The related symptoms and signs should be recognized. Prompt recognition and treatment of this condition can be life-saving, and the long-term outcomes have generally been favorable if the condition has been recognized early.

To elucidate the sleep quality characteristics and factors related to either good or poor sleep quality in acromegaly patients before surgery and to explore sleep quality changes after transsphenoidal surgery and the factors related to these changes.We prospectively enrolled 39 acromegaly patients and 78 patients with nonfunctioning pituitary adenomas. Scales for anxiety, depression, disease stigma and nasal condition were evaluated. The Pittsburgh Sleep Quality Index (PSQI) questionnaire was administered before surgery as well as one month and three months after surgery.A higher percentage of acromegaly patients had poor sleep quality compared to controls (35.9% vs. 5.1%, p 0.001). In addition, acromegaly patients experienced worse subjective sleep quality, extended sleep latency, increased sleep disturbance and decreased daytime functioning. Higher scores for anxiety, disease stigma and sinonasal outcomes were correlated with worse sleep quality in acromegaly patients. At one month after transsphenoidal surgery, we found worse subjective sleep quality, extended sleep latency, shortened sleep duration, impaired sleep efficiency and increased sleep disturbance in acromegaly patients. At three months postoperatively, most impaired PSQI domains in acromegaly patients recovered to preoperative levels. The use of soluble gauze was related to decreased sleep quality at one month after surgery and severe anxiety and depression were related to improved sleep quality at three months after surgery.Sleep quality was reduced in acromegaly patients. Moreover, sleep quality initially worsened after surgery but later recovered. Emotional problems and the use of soluble gauze were related factors.None.

We sought to assess whether controlled, intraoperative lumbar drainage (LD) of cerebrospinal fluid (CSF) could facilitate resection of pituitary macroadenomas and reduce the rate of CSF leak.A retrospective cohort study from a prospective database was conducted on 189 patients with pituitary macroadenoma who received endoscopic transsphenoidal surgery between 2013 and 2017. Patients were classified into 2 groups: 119 patients received an intraoperative LD (LD group) and 70 patients underwent routine endoscopic surgery without LD (control group). In the LD group, lumbar catheters were placed preoperatively and CSF was drained intermittently during tumor resection. The rates of gross total resection (GTR) and CSF leaks were assessed both intraoperatively and postoperatively.Intraoperative LD was associated with a higher rate of GTR (92.4% in the LD group vs. 78.6% in the control group, P = 0.006), especially in macroadenomas with suprasellar extension (90.3% vs. 75.0%, P = 0.012). Both intraoperative and postoperative CSF leak rates were significantly decreased in the LD group (intraoperative: 10.1% vs. 31.4%, P0.001; postoperative: 3.4% vs. 11.4%, P = 0.035). In functioning adenomas, a better remission rate of excess-hormone secretion was observed in the LD group compared with the controls (89.1% vs. 60.6%, P = 0.001). Patients in the LD group also had an enhanced recovery with a shorter postoperative length of stay (7 days vs. 5 days, P = 0.020).Intraoperative LD may assist surgeons during endoscopic transsphenoidal resection of pituitary macroadenomas by achieving a higher rate of GTR and a lower rate of perioperative CSF leaks. Validation in prospective randomized controlled studies is needed.

Cushing's disease (CD) is a spectrum of clinical manifestations due to adrenocorticotropic hormone-secreting pituitary adenoma. Transsphenoidal adenomectomy remains the standard treatment. There has been a paradigm shift from microscopic to endoscopic transsphenoidal surgery in recent years. However, the efficacy of endoscopic transsphenoidal surgery has not been established. Therefore, it is of interest to determine the superiority of endoscopic transsphenoidal surgery, if any, over microscopic surgery.To assess the efficacy of endoscopic endonasal transsphenoidal surgery for the treatment of CD and to determine the factors affecting remission.Patients undergoing surgery for CD from 2009 to 2017 were analyzed retrospectively. Transsphenoidal resection was the preferred treatment, with recent trends in favor of the endonasal endoscopic skull base approach. Postoperative cortisol level of2 μg/dL was taken as remission and value between 2 and 5 μg/dL as possible remission.In total, 104 patients operated primarily for CD were included for analysis; 47 patients underwent microscopic surgery, 55 endoscopic surgery, and 2 were operated transcranially. Remission was achieved in 76.47% of patients. In univariate analysis, factors significantly associated with remission were 1) type of surgery (P = 0.01); remission in endoscopy surgery (88.23%) is better than microscopy (56.6%); 2) postoperative day 1 morning cortisol (P = 0.004); and 3) postoperative day 1 morning ACTH (P = 0.015). In multivariate analysis, only postoperative day 1 cortisol was found to be significant predictor of remission (P = 0.02).Postoperative plasma cortisol level is a strong independent predictor of remission. Remission provided by endoscopy is significantly better than the microscopic approach.

Objective To evaluate and understand the clinical behavior and radiologic correlates of tumors originating from the posterior pituitary gland. To review the management strategy for these rare tumors and add to the limited existing literature. Methods Retrospective review of 8 cases (5 pituicytomas, 2 spindle cell oncocytomas, and 1 granular cell tumor) managed at our institution between 2004 and 2019. The patients' clinical course, histologic features, and radiologic findings were reviewed. Their management and long-term follow-up is presented and compared with the literature. Results Long-term follow-up ranged from 1 to 9 years. There was 1 recurrence in a patient with spindle cell oncocytoma, and this was treated with radiotherapy. The endoscopically managed cases resulted in complete tumor excision with no recurrence. Conclusions Epidemiologic data on primary tumors of the neurohypophysis is limited because of the rarity of these tumors. This study adds to the literature that these tumors behave as World Health Organization grade I tumors, although close follow-up is recommended as a few cases have shown recurrence. The endoscopic approach resulted in better gross total tumor resection rate in this series.

Background Carotid-cavernous fistula (CCF) is a rare complication after transsphenoidal surgery. Methods The aim of this article is to report a case of CCF after the endoscopic resection of a growth hormone secreting pituitary microadenoma, and to discuss and review all the cases of CCF secondary to transsphenoidal procedures described in literature. Results A patient aged 74 years was operated for a growth hormone pituitary microadenoma through an endoscopic transsphenoidal surgery. During the procedure, a copious bleeding from the left cavernous sinus was managed with hemostatic material. A direct CCF was diagnosed and managed with transvenous and transarterial coiling. A complete exclusion of the fistula was possible, and the patency of the internal carotid artery was maintained. A total of 9 other cases have been reported. A transsphenoidal approach was performed for sellar tumors in 6 cases, and for chronic sinusitis in 2 cases. In 7 cases, intraoperative hemorrhage was reported, which could be controlled in 5 cases. The postoperative diagnosis of CCF was immediate in 5 cases. Patients presented with persistent bleeding after nasal unpacking or later with chemosis and proptosis. Cerebral angiography was the gold standard for the diagnosis. Eight cases were successfully treated through endovascular techniques with no recurrence observed at follow-up (mean of 15 months). No major neurologic complications were observed. Conclusions CCF should be suspected with every abnormal bleeding after transsphenoidal surgeries, even when the symptoms are mild. Diagnostic arteriography and endovascular treatment represent the mainstay of the management, and an early diagnosis strongly improves the prognosis.

Introduction Persistent trigeminal artery (PTA) is the most common remnant of primitive circulation communicating the developing carotid and vertebrobasilar junction. Although discovered incidentally, an altered hemodynamic may lead to an increased association of aneurysms, vascular malformations, and stroke. Neurosurgeons should be aware of the presence and significance of PTA when interpreting imaging and planning interventions. Methods We retrospectively reviewed all magnetic resonance angiography and cerebral digital subtraction angiography performed between 2012 and 2017 for the presence of PTA. The radiologic and anatomic details were noted and analyzed along with the clinical profiles. We categorized the radiologic findings with respect to the available classification systems. A review of the available literature was done comparing our results. Results We found 33 cases of incidentally detected PTA. The average age of the patients was 45.42 years. The lateral surface of the proximal cavernous internal carotid artery was the most common origin (n = 23). Only 3 cases had a medial/transsellar course. Most cases were Saltzman/Weon type I (19/33). Intracranial aneurysms were associated with 6 patients (18.18%). Trigeminal neuralgia (TN) was a presenting feature in 5 patients. None had a direct neurovascular conflict at the root entry zone. Conclusions Our study is one of the largest to describe the incidence of PTA. We emphasized the importance of PTA to the neurosurgeons; increased association of aneurysms, as a route for intervention in occlusive disease of the posterior fossa; risk of injury and bleeding during transsphenoidal surgery; and the association with TN. However, we found that only PTA variants are likely to be associated with TN because of their cisternal course causing NV conflict.

Silent thyrotropin pituitary adenomas (TSHomas) are defined by absence of hyperthyroidism despite TSH immunopositivity. Data pertaining to clinical and surgical characteristics of silent TSHomas remains limited. We aim to describe the clinical presentation, pathological characteristics, and outcomes in silent TSHoma patients treated at a tertiary pituitary center. We retrospectively identified patients with histologically-proven silent TSHoma who underwent transsphenoidal resection at our center between 2000 and 2016 (n = 1244 total patients). Patients with preoperative hyperthyroidism or thyroidectomy were excluded. Twenty patients with silent TSHomas were included (1.6% of surgically treated PAs), of which 35% were reoperations. Presenting symptoms included vision loss (45%) and headache (40%). Preoperative pituitary dysfunction included hypothyroidism (40%), hypogonadotropic hypogonadism (30%), and panhypopituitarism (15%). Nineteen patients (95%) had macroadenomas (mean diameter 29.9 mm). Extrasellar growth was identified in 17 patients (85%) and 65% had cavernous sinus invasion. Immunostaining for alpha-subunit was positive in 19 patients (95%), and 75% of tumors expressed immunopositivity for hormones other than TSH. Gross total tumor resection was achieved in 9 patients (45%) on follow-up MRI. Major postoperative complications included hydrocephalus (1 patient) and cerebrospinal fluid leak with meningitis (1 patient). Tumor progression and recurrence occurred in 1 patient each (10% total) over the follow-up period (median 18.5 months). Silent TSHomas tend to be large, invasive tumors. In addition to TSH, a majority express immunopositivity for alpha-subunit and gonadotropins, thereby potentially supporting a primitive adenoma lineage and subtype. Despite reoperation in several patients, good overall outcomes with low complication rates were achieved.

Background Little is known on the impact of the pattern and extent of pneumatization of the sphenoid sinus (SS) on the dimensions of the surgical windows used in extended endoscopic endonasal transsphenoidal approaches. We therefore investigated whether the distances between the 2 optic canals and between the paired paraclival carotid arteries are influenced by the pattern and extent of pneumatization of the SS. Methods One hundred high-resolution computed tomography scans from 47 adult female and 53 adult male patients were analyzed. The pattern of SS pneumatization was classified into conchal, presellar, and sellar types. Sellar-type sinuses were then classified according to a newer detailed classification system. Maximal anteroposterior (AP), transverse (TR), and craniocaudal diameters of the SS, interoptic distance at the limbus sphenoidale (IODL) and at the entrance of the optic canal (IODE) and the intercarotid distance between the paraclival carotids (ICD) were measured. A 2-tailed Mann-Whitney U test and Pearson correlation coefficient (R) were used for statistical analysis. A P value Results Positive correlation was found between IODL and both AP and TR diameters of the SS; between IODE and both AP and TR diameters of the SS; and between ICD and all diameters of the SS. The highest correlation for each of the IODL, IODE, and ICD was noted with the TR diameter of the SS. Conclusions During the development of the SS, pneumatization progress likely exerts quantitative and direction-specific forces, which gradually increase the interoptic and intercarotid distances.

This study aimed to determine the anatomical and histological features of diaphragma sellae that affect the suprasellar extension of intrasellar tumours. Twenty-four fresh adult cadavers were dissected for the study. Diaphragma sellae and pituitary capsules with sellar structures were resected. The diaphragma sellae was anatomically reviewed in detail. Immunohistochemical staining was performed for collagen types I, II, III, and IV. We examined the suprasellar growth of 13 sellar tumours extending superiorly through the diaphragma sellae by performing a series of 2704 endoscopic transnasal operations to analyse the anatomic and histologic results of the study. The diameter of the foramen of diaphragma sellae varied between specimens. Of 24 specimens, the diaphragma sellae in five (21%) had a tight-type foramen and those in 19 (79%) were more spacious. An increased expression of collagen types I and IV was observed in the pituitary capsule and the diaphragma sellae. In this clinical series, we observed that all types of sellar tumours could expand through the foramen. We observed radiologically and intraoperatively that the diaphragma sellae was displaced laterally and formed a dome in two cases with an adenoma extending to the suprasellar area. Two types of suprasellar extension through the diaphragma sellae are possible: 1) The collagen structure of diaphragma sellae can be destroyed by invasive tumours; 2) The morphology of the foramen of the diaphragma sellae facilitates suprasellar tumoural extension. All sellar tumours, including non-invasive cystic tumours, may invade the suprasellar area by expanding through the foramen of the diaphragma sellae.

Carotid artery pseudoaneurysm is a rare complication of transsphenoidal surgery, usually diagnosed within 90 days post procedure. Sequelae of pseudoaneurysm rupture, such as severe epistaxis or carotid cavernous fistula (CCF), have significant morbidity and mortality. A case of epistaxis from pseudoaneurysm rupture over a decade after transsphenoidal surgery is presented, with staged treatment using coiling, endonasal mucosal flap repair and interval flow-diverting stent insertion. This case illustrates that pseudoaneurysm rupture occurs regardless of time course after transsphenoidal surgery, and treatment strategies using combined endovascular and endonasal techniques are reviewed.

The endoscopic endonasal transsphenoidal (EET) approach for skull base tumors has become increasingly popular. We know that bone defects in the skull base can cause cerebrospinal fluid rhinorrhea, but for patients who need to be intubated through the nose, the tube can enter the brain through a skull base bone defect. Nasogastric tube feeding into the brain is a rare occurrence, and this situation can occur only in the case of a skull base defect. We treated a patient with an unusual complication after the EET approach for pituitary adenoma resection. This particular case suggests that bone defects after EET surgery can not only cause cerebrospinal fluid rhinorrhea but also allow the entry of a nasogastric tube into the brain. For patients with a history of EET surgery, endoscopy-assisted gastric tube implantation can be performed if necessary.

Sodium perturbations are a common complication after pituitary surgery, with hyponatremia being the most frequent. Postoperative assessments should be tailored to the early and late periods, and monitoring sodium perturbations is recommended. Cerebral salt wasting is rare after pituitary surgery, and diagnosis and management can be challenging. Providing patient counseling and close postoperative follow-up is important to effectively manage diabetes insipidus and reduce hospital readmissions due to sodium perturbations.

Objective Plurihormonal pituitary adenomas (PPAs) have recently been divided into 2 subtypes: pituitary specific transcription factor 1–positive plurihormonal pituitary adenomas (PIT-1+ PPAs) and plurihormonal adenomas with unusual immunohistochemical combinations (PAwUIC). The purpose of this study was to review patients with PPAs, elucidate their clinicopathologic characteristics, and present the surgical results. Methods Records were analyzed for 665 patients who underwent endoscopic endonasal transsphenoidal surgery for pituitary adenomas between 2007 and 2018. Data were reviewed for 27 patients who met the definition of PPAs and delineated regarding clinical, radiologic, pathologic features, and surgical outcomes. Results Of the 27 patients, 18 had PAwUIC, and 9 patients were diagnosed with PIT-1+ PPAs. Twenty-four patients (88.8%) had macroadenomas, including 6 giant adenomas (≥4 cm) (22.2%). Cavernous sinus invasion was found in 12 patients (44.4%). Pathologic examinations showed high aggressivity in nearly half of the patients. Most patients with PAwUIC (77.8%) had features of nonfunctioning pituitary adenomas, and only 4 had features of hormone-secreting pituitary adenomas. Gross total resection rates were 57.1% for PAwUIC and 77.8% for PIT-1+ PPAs. The remission rate for hormone-secreting pituitary adenomas was 100% in this case series. Mean follow-up was 74.4 ± 33 months (range, 6–121 months) for overall groups. Conclusions To our knowledge, this is the first study to describe the clinicopathologic features of newly classified PPAs, which are characterized by aggressive behavior with higher values of percentage of cavernous sinus invasion. Relevant pathologic diagnosis of PPAs is crucial for appropriate management and follow-up.

Prolactinomas are the most common functional pituitary adenoma. Many prolactinomas can be treated with medication, but all patients should be evaluated at a neuroendocrine center including experienced neurosurgeons trained in transsphenoidal surgery. Surgery for prolactinomas is feasible and can be performed with low morbidity. Patients never previously treated with dopamine agonists should be considered for surgery if they have neurologic deficits, pituitary apoplexy, an uncertain diagnosis, or a significantly cystic prolactinoma. Patients previously treated with dopamine agonists should be considered for surgery in cases of intolerance or resistance. Recurrent and aggressive prolactinomas often require multimodal therapy.

Endoscopic endonasal transsphenoidal approaches to craniopharyngioma has become increasingly popular over the last 15 years. We present the results of our retrospective series of craniopharyngiomata resected by an endoscopic, endonasal approach at a low-volume service in Australasia. Between the years of 2009 and 2017, 11 patients underwent pure endoscopic endonasal transsphenoidal resection for a craniopharyngioma at our institutions. The medical records, histopathology, intraoperative findings and patient imaging were retrospectively assessed. 11 patients were included with 5 male and 6 female patients. The mean age was 32.0 years (range 14–68 years). Of this patient series a gross total resection of the tumour was achieved in 8 of 11 patients (73%). In the immediate postoperative phase, 10 of the 11 patients developed diabetes insipidus (91%). The pituitary stalk was formally not seen in 4 patients and all were treated with vasopressin. Of the 7 patients where the pituitary stalk was identified it was formally divided in 6 and preserved in 1 patient where the tumour was separate to the stalk. The endoscopic endonasal transsphenoidal approach for excision of craniopharyngioma, utilising and progressing the surgical mentoring model, can achieve adequate decompression of critical structures. Furthermore, our aggressive approach to divide and remove the involved pituitary stalk results in high rates of gross macroscopic resection with excellent long-term disease control with a greater risk of postoperative diabetes insipidus and panhypopituitarism.

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

Background Clinical examination, including pre- and postoperative assessment of olfaction function, should be included in evaluating surgical outcomes in patients with pituitary adenomas. Most of the studies are lacking assessment of olfactory function. Methods A prospective study of 143 patients who underwent surgical resection of pituitary adenomas from January 2014 to December 2017 was performed. Data on clinical presentation, pre- and postoperative neurologic, endocrinologic, and ophthalmologic examinations, complications, and follow-up outpatient examinations were recorded. Olfactory function was assessed using the Sniffin' Sticks odor identification test preoperatively, postoperatively (3 months), and 1 year after surgery. Results Normosmia was present preoperatively in 93.7% of patients, postoperatively in 95.8% of patients, and in 95.1% 1 year postsurgery. Hyposmia was present preoperatively in 4.2% of patients, postoperatively in 2.1% of patients, and in 1.4% 1 year after surgery. Anosmia was present preoperatively in 2.1% of patients, postoperatively in 2.1% of patients, and in 3.5% 1 year after surgery. In patients with preoperative normosmia, postoperative hyposmia and anosmia were present in 1.5% of patients. There were no differences according to age, sex, size, or type of pituitary adenoma. Conclusions Assessment of olfactory function should be included in the analysis of pituitary adenoma surgery results. This prospective study showed low risk of olfaction deterioration if an endoscopic endonasal approach is used without any mucosal flaps for skull base reconstruction. Further studies that include objective olfaction assessment are warranted.

Objective Little is known about the effectiveness of lumbar drainage (LD) in the treatment of delayed or recurrent cerebrospinal fluid (CSF) leaks. We report our institutional experience and the effectiveness of LD in the management of delayed or recurrent CSF leaks. Methods Between January 2014 and December 2018, a total 21 patients with delayed or recurrent CSF leaks were enrolled in the research. All patients were treated conservatively for 48 hours, and LD was prescribed if CSF leaks still existed after 48 hours. If LD failed, endoscopic endonasal surgery (EES) was performed as soon as possible. Medical records were collected to analyze the effectiveness of LD. Results Among 21 patients, 4 patients experienced resolution with conservative treatment, and 17 patients were treated by CSF diversion by LD. The total cure rate of LD was 9/17 (52.9%). The cure rate was not statistically significantly different (relative risk = 3.33; Fisher exact test P = 0.131) between the traumatic group (8/12, 66.7%) and the transsphenoidal surgery group (1/5, 20.0%). During the follow-up time, no recurrence of CSF leaks was observed. Conclusions The cure rate of LD in delayed or recurrent CSF leaks was lower than that of initial treatment with LD. The cure rate in the traumatic group tended to be higher than that in the transsphenoidal surgery group. EES can be used as a remedial treatment for patients in whom LD has failed.

The aim of this study was to investigate the occurrence, correlated factors and prognosis of posttraumatic stress symptoms (PTSS) in patients with Cushing’s disease (CD). A total of 49 patients who were newly diagnosed with CD and underwent transsphenoidal surgery in our hospital from April 2015 to August 2017 were asked to participate in this study. Another group of 49 age and sex matched healthy control participants were also included for comparison. PTSS (measured with Impact of Event Scale-Revised, IES-R), depression/anxiety (measured with Hospital Anxiety and Depression scale, HADS) and quality of life (QoL; measured with 36-item short-form, SF-36) were evaluated at pre-surgery, 6 months post-surgery and 12 months post-surgery. The results showed that at preoperative stage, 15 (30.6%) CD patients developed PTSS, and they had higher 24 h UFC, and presented worse levels of depression, anxiety and QoL compared with patients without PTSS. Although most of them recovered postoperatively, there were still 5/15 (33.3%) patients persisted with PTSS for over a year. Additionally, one patient with recurred CD developed PTSS between 6 and 12 months postoperatively. Among the whole group of CD patients, the PTSS severity showed consistent improvement after surgery, which was in accordance with the progressing trends of depression, anxiety and psychological aspects of SF-36. However, compared with healthy individuals, CD patients in remission still performed worse in physical/mental health. In conclusion, patients with CD can develop PTSS, and they may persist for over a year even after successful surgery. Combined psychological intervention is advised for these patients.

Background Cerebral vasospasm causing delayed cerebral ischemia after transsphenoidal surgery is a rare but life-threatening complication. Reports in the literature after extended endoscopic endonasal approach (EEEA) are scarce. Considering the progressive use of this technique during recent years, a better understanding of the potential adverse effects after this procedure is needed. The aim of this report was to systematically analyze the current literature and discuss management and causes for cerebral vasospasm after EEEA surgery. Methods We performed a systematic search of the literature according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement and aimed to discuss relevant risk factors for cerebral vasospasm after extended transsphenoidal surgery. Results Thirty-four cases of delayed cerebral vasospasm after transsphenoidal surgery were identified. Among these, 4 were operated with an EEEA. We provide an extensive literature review and discuss causes and management of this delayed complication. We further present the case of a young woman who underwent resection for a large suprasellar recurrent pituitary adenoma. Surgery and initial postoperative course were uneventful. On the eighth postoperative day, the patient developed aphasia and brachiofacial paresis. Digital subtraction angiography (DSA) demonstrated cerebral vasospasm, and repeated intra-arterial nimodipine infusion was administrated. The patient recovered completely and was discharged without neurologic deficits. Conclusions The threshold for DSA after unexplained neurologic deterioration after extended transsphenoidal surgery should be low, and cerebral vasospasm should be treated early and aggressively. Surgeons performing EEEA need to consider the possibility of this delayed complication.

Objective Lymphocytic hypophysitis (LYH) is a rare autoimmune inflammatory disease of the pituitary gland. In this study, we aim to characterize LYH at presentation and focus on the management and prognosis of LYH. Methods A retrospective study of patients with LYH was conducted between 2011 and 2018 at a single institute. The patients were included by pathologic conformation and strict exclusion criteria. Clinical profile, imaging, and management data were collected. Results Twenty patients with LYH (16 women and 4 men) were included. Ten patients were diagnosed histologically and the remaining 10 patients were confirmed clinically of exclusion criteria. The median age at diagnosis was 37 years (range, 16–58 years). Presenting symptoms were followed by polyuria/polydipsia (11, 55%), vision changes (10, 50%), headache (8, 40%), menstrual irregularities and amenorrhea (4, 20%), diplopia (1, 5%), or sexual dysfunction (1, 5%). Eight patients had partial anterior pituitary hormone dysfunction. The thyroid-stimulating hormone axis was most involved. Ten patients received transsphenoidal surgery, 5 patients experienced steroid pulse therapy, and observation was performed on 5 patients. Only 5 patients (25%) showed improvement of anterior pituitary dysfunction after initial management. Recovery of diabetes insipidus occurred in 2 patients (18%). The overall recurrence rate was 22.2%. Conclusions Nonoperative treatment is a better option for most patients with LYH because it is effective and noninvasive. Surgery is recommended for definitive diagnosis, severe or rapid progression of neurologic impairment, and glucocorticoid insensitivity. Periodic follow-up is mandatory in a patient's long-term management.

The effect of intraoperative magnetic resonance imaging (iMRI) on tumor control during endoscopic transsphenoidal surgery (TSS) for nonfunctioning pituitary adenomas (NFPAs) has not yet been completely characterized. The present study assessed the effect of the iMRI findings on gross total resection (GTR) and progression-free survival at a mean follow-up of 62 ± 9.4 months.Retrospective analyses were performed on the data from 133 consecutive patients with NFPA who had undergone endoscopic TSS with iMRI using a 1.5T scanner.Compared with the first and final iMRI scans, the GTR rate increased from 42.9% to 63.9%. Multivariate logistic regression analysis revealed that a lower GTR rate was associated with a higher Knosp score (odds ratio [OR], 3.612; 95% confidence interval [CI], 1.568-8.321; P = 0.003), an increased tumor volume (OR, 0.926; 95% CI, 0.866-0.991; P = 0.025), and a history of surgery (OR, 0.376; 95% CI, 0.155-0.917; P = 0.031). During follow-up, 8 tumors (9.4%) recurred, and 29 remnants (60.4%) regenerated. The recurrence and regeneration rates were 0.019 and 0.121 patient/year, respectively. Kaplan-Meier analysis revealed that the tumor recurrence rates were lower for patients who had undergone GTR (P0.01) compared with those who had not. Univariate Cox regression analysis suggested that the presence of tumor remnants (hazard ratio, 1.546; 95% CI, 1.071-2.232; P = 0.02) was the influencing factor associated with tumor progression.High-field iMRI can increase the GTR rate in endoscopic TSS for NPFAs. The increased GTR rate was associated with longer progression-free survival. Cavernous sinus invasion, tumor size, and surgical history were important predictors of GTR in patients with NFPA.

Background Resection and remission rates are low in pituitary adenomas that invade the cavernous sinus (CS) because of the complexity of the region. In this study, the authors describe the invasion patterns and surgical approaches for these corridors. Methods Endoscopic transsphenoidal surgery was performed in 1849 patients between August 1997 and 2017; 381 of these patients were included in the study because of the presence of CS invasion. CS invasion pathways and CS compartments were defined, and these patients were divided into 3 groups according to their invasion corridors. The cases were also categorized according to the Knosp classification. Results A total of 381 patients were included, 199 of whom were men. The mean follow-up duration was 34 months. In preoperative magnetic resonance imaging measurements, 2 of 381 patients had microadenoma, 350 of 381 had macroadenoma, and 29 of 381 had giant adenoma. When the early postoperative magnetic resonance images were compared with the preoperative images, 91 of 381 (23.9%) patients had subtotal tumor resection, 83 of 381 (21.8%) had near-total tumor resection, and 207 of 381 (54.3%) had gross total tumor resection. Considering the invasion classification with endoscopic confirmation, there were 168 of 381 (44.1%) cases of isolated medial corridor involvement, 74 of 381 (19.4%) cases of isolated lateral corridor involvement, and 139 of 381 (36.5%) cases of total involvement cases. Conclusions Medial wall defect has no importance in the invasion. Similarly, seeing the existence of pitholes is not an invasion criterion. Pituitary adenomas invade 4 compartments: the superior, anteroinferior, posterior, and lateral compartments of the CS via 2 corridors. Increased experience, novel approaches, and endoscopic developments have contributed immensely to this field.