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OBJECTIVE One of the more serious risks in the treatment of third ventricle craniopharyngiomas is represented by hypothalamic damage. Recently, many papers have reported the expansion of the indications for the endoscopic endonasal approach (EEA) to be used for these tumors as well. The aim of this study was to assess the outcome of sleep-wake cycle and body core temperature (BCT), both depending on hypothalamic control, in patients affected by craniopharyngiomas involving the third ventricle that were surgically treated via an EEA. METHODS All consecutive adult patients with craniopharyngiomas that were treated at one center via an EEA between 2014 and 2016 were prospectively included. Each patient underwent neuroradiological, endocrinological, and ophthalmological evaluation; 24-hour monitoring of the BCT rhythm; and the sleep-wake cycle before surgery and at follow-up of at least 6 months. RESULTS Ten patients were included in the study (male/female ratio 4:6, mean age 48.6 years, SD 15.9 years). Gross-total resection was achieved in 8 cases. Preoperative BCT rhythm was pathological in 6 patients. After surgery, these disturbances resolved in 2 cases, improved in another 3, and remained the same in 1 patient; also, 1 case of de novo onset was observed. Before surgery the sleep-wake cycle was pathological in 8 cases, and it was restored in 4 patients at follow-up. After surgery the number of patients reporting diurnal naps increased from 7 to 9. CONCLUSIONS The outcome of the sleep-wake cycle and BCT analyzed after EEA in this study is promising. Despite the short duration of the authors' experience, they consider these results encouraging; additional series are needed to confirm the preliminary findings.

Recently, an alternative endoscopic endonasal approach to Meckel’s cave (MC) tumors has been proposed. To date, few studies have evaluated the results of this route. The aim of our study was to evaluate long-term surgical and clinical outcome associated with this technique in a cohort of patients with intrinsic MC tumors. All patients with MC tumors treated at out institution by endoscopic endonasal approach (EEA) between 2002 and 2016 were included. Patients underwent brain MRI, CT angiography, and neurological evaluation before surgery. Complications were considered based on the surgical records. All examinations were repeated after 3 and 12 months, then annually. The median follow-up was of 44.1 months (range 16–210). The series included 8 patients (4 F): 5 neuromas, 1 meningioma, 1 chondrosarcoma, and 1 epidermoid cyst. The median age at treatment was 54.5 years (range 21–70). Three tumors presented with a posterior fossa extension. Radical removal of the MC portion of the tumor was achieved in 7 out of 8 cases. Two patients developed a permanent and transitory deficit of the sixth cranial nerve, respectively. No tumor recurrence was observed at follow-up. In this preliminary series, the EEA appeared an effective and safe approach to MC tumors. The technique could be advantageous to treat tumors located in the antero-medial aspects of MC displacing the trigeminal structures posteriorly and laterally. A favorable index of an adequate working space for this approach is represented by the ICA medialization, while tumor extension to the posterior fossa represents the main limitation to radical removal of this route.

OBJECTIVEIn the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach.METHODSAll consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter. Postoperative adjuvant therapies were considered.RESULTSSixty-five patients (male/female ratio 1:0.9) were included in this study. The median age was 48 years (range 9–80 years). Gross-total resection (GTR) was achieved in 47 cases (58.7%). On univariate analysis, primary procedures (p = 0.001), location in the superior or middle third of the clivus (p = 0.043), extradural location (p = 0.035), and histology of conventional chordomas (p = 0.013) were associated with a higher rate of GTR. The complication rate was 15.1%, and there were no perioperative deaths. Most complications did not result in permanent sequelae and included 2 CSF leaks (2.5%), 5 transient cranial nerve VI palsies (6.2%), and 2 internal carotid artery injuries (2.5%), which were treated with coil occlusion of the internal carotid artery without neurological deficits. Three patients (3.8%) presented with complications resulting in permanent neurological deficits due to a postoperative hematoma (1.2%) causing a hemiparesis, and 2 permanent ophthalmoplegias (2.5%). Seventeen patients (26.2%) have died of tumor progression over the course of follow-up (median 52 months, range 7–159 months). Based on Kaplan-Meier analysis, the survival rate was 77% at 5 years and 57% at 10 years. On multivariate analysis, the extent of tumor removal (p = 0.001) and the absence of previous treatments (p = 0.001) proved to be correlated with a longer survival rate.CONCLUSIONSThe EEA was associated with a high rate of tumor removal and symptom control, with low morbidity and preservation of a good quality of life. These results allow for a satisfactory overall survival rate, particularly after GTR and for primary surgery. Considering these results, the authors believe that an EEA can be a helpful tool in chordoma surgery, achieving a good balance between as much tumor removal as possible and the preservation of an acceptable patient quality of life.

Introduction The endoscopic endonasal approach (EEA) might seem an “unnatural” route for intradural lesions such as meningiomas. The aim of this study is to critically revise our management of anterior skull base meningiomas to consider, in what cases it may be advantageous. Material and Methods Each consecutive case of anterior skull base meningioma operated on through an EEA or combined endoscopic–transcranial approach at our institution, between 2003 and 2017, have been included. Tumors were classified on the basis of their location and intra or extracranial extension. Follow-up consisted of an MRI (magnetic resonance imaging) and a clinical examination 3 months after the surgery and then repeated annually. Results Fifty-seven patients were included. The most common location was the tuberculum sellae (62%), followed by olfactory groove (14%), planum sphenoidale (12%), and frontal sinus (12%). Among these, 65% were intracranial, 7% were extracranial, and 28% both intra and extracranial. Radical removal was achieved in 44 cases (77%). Complications consisted in 10 CSF (cerebrospinal fluid) leaks (17.6%), 1 overpacking (1.7%), and 1 asymptomatic brain ischemia (1.7%). Visual acuity and campimetric deficits improved respectively in 67 and 76% of patients. Recurrence rate was of 14%. Conclusions EEA presents many advantages in selected cases of anterior skull base meningioma. However, it is hampered by the relevant risk of CSF leak. We consider that it could be advantageous for planum/tuberculum sellae tumors. Conversely, for olfactory groove or frontal sinus meningiomas, it can be indicated for tumors with extracranial extension, while its role is still debatable for purely intracranial forms as considering our surgical results, it could be advantageous for midline planum/tuberculum sellae tumors. Conversely, it can be of first choice for olfactory groove or frontal sinus meningiomas with extracranial extension, while its role for purely intracranial forms is still debatable.

OBJECT Adrenocorticotropic hormone (ACTH) adenomas have been recognized as a more aggressive and invasive subtype of pituitary adenomas. An additional and clinically relevant peculiarity of these tumors is their ability to modify their clinical expression from a silent form to Cushing disease or vice versa. The aim of this study was to review a series of patients with pituitary adenomas and analyze the clinical implications of the transformation of clinical expression in 5 cases that showed this phenomenon. METHODS The authors retrospectively reviewed a series of patients with pituitary adenoma and collected clinical, biohumoral, and neuroradiological data of those who presented with a transformation from silent ACTH adenomas to functioning tumors or vice versa. In all the cases, preoperative assessment consisted of brain MRI, ophthalmological examination, and complete baseline endocrinological investigation. In patients with clinical and/or biochemical findings suspicious for Cushing syndrome, a low-dose dexamethasone suppression test was performed to rule in or out this diagnosis. Endocrinological evaluations were repeated 1 month after surgery, 3 months after surgery, and every 6 months or annually thereafter. Ophthalmological evaluations and brain MRIs were repeated after 3 months and then every 6 or 12 months thereafter. RESULTS Five patients (2 men and 3 women) included in this series had corticotropic tumors that showed transformation from an endocrinologically silent form to manifest Cushing disease and vice versa. The mean age at presentation was 40 years (range 18–51 years). In 3 of these patients, a transformation from silent to functioning ACTH adenoma with manifest Cushing disease occurred. In 1 patient, the authors observed the transition from a functioning to a silent adenoma with spontaneous resolution of hypercortisolism. Another patient's silent adenoma “shifted” to a functioning adenoma and then regressed back to a silent form with spontaneous resolution of Cushing disease. This patient again developed hypercortisolism, which finally resolved spontaneously. In this series, the transformation occurred after a mean of 3.5 years (range 6 months to 7 years). The shift from an ACTH-silent to a functioning adenoma was observed in 9% of the ACTH-silent adenomas in this series (4 of 44 cases), and the spontaneous remission of Cushing disease to a silent corticotroph cell adenoma occurred in 1.5% of cases of this series (2 of 132 functioning ACTH adenomas). At follow-up (mean 107 months; range 60–177 months), cortisol levels were within normal limits in all 5 cases. However, 1 patient required Gamma Knife radiosurgery and eventually adrenalectomy for disease control to be achieved. CONCLUSIONS The ability of silent ACTH adenomas to transform their secretion pattern poses a challenge for neurosurgeons and endocrinologists. Because the transformation is often unexpected, the clinical and biochemical data can be underestimated. Furthermore, this bizarre and unpredictable postoperative tumor behavior can lead to misinterpretation of clinical and endocrinological outcomes. Even if these cases are very rare, they are not anecdotal in large series. Thus, ACTH adenomas require careful biohumoral and neuroradiological follow-up to detect possible transformations.

Object In the last decade the indications for the endoscopic endonasal approach have been progressively expanded to include lesions that have not been traditionally considered amenable to resection through the transsphenoidal route. In this study, the authors analyze their experience with hypothalamic gliomas treated via the endoscopic endonasal approach. Methods Consecutive cases of hypothalamic gliomas treated since 2007 via an endoscopic endonasal approach were reviewed. Preoperative and postoperative neuroimaging as well as endocrinological, neurological, and visual symptoms were analyzed to assess the surgical outcome. Signs and symptoms of hypothalamic dysfunction including body mass index (BMI), memory, sleep-wake rhythm, and polyphagia were prospectively collected pre- and postoperatively to assess hypothalamic function. Quality of life was evaluated using the Katz scale. Results In the initial phase the endoscopic endonasal approach was adopted in 3 cases with a palliative intent, to obtain a biopsy sample or for debulking of the mass followed by radio- or chemotherapy. In 2 later cases it was successfully adopted to achieve gross-total tumor resection. Complications consisted of 2 postoperative CSF leaks, which required an endoscopic endonasal reintervention. Visual deficit improved in 3 cases and normalized in the other 2. Four patients developed diabetes insipidus, and 3 an anterior panhypopituitarism. All patients had a moderate increase in BMI. No patients presented with any other signs of hypothalamic damage, and their quality of life at follow-up is normal. Conclusions Despite the limitations of a short follow-up and small sample, the authors’ early experience with the endoscopic endonasal approach has revealed it to be a direct, straightforward, and safe approach to third ventricle astrocytomas. It allowed the authors to perform tumor resection with the same microsurgical technique: dissecting the tumor with 2 hands, performing a central debulking, and controlling the bleeding with bipolar coagulation. The main limitations were represented by some anatomical conditions, such as the position of the chiasm and the anterior communicating artery complex and, finally, by the challenge of watertight plastic repair. To definitively evaluate the role of this approach in hypothalamic gliomas, a comparison with transcranial series would be necessary, but due to the rarity of these cases such a study is still lacking. The authors observed that more aggressive surgery is associated with a worse endocrinological outcome; thus they consider it to be an open question (in particular in prepubertal patients) whether radical removal is an advisable goal for hypothalamic gliomas.

To the Editor: We read with interest the article by McLaughlin and colleagues1 about Pituitary Centers of Excellence (PCOEs), along with the outstanding comments published in the same issue of the journal. We agree with the authors that PCOE may be defined as a cohesive team of specialists (neurosurgeons, endocrinologists, otorynolaryngologists, neuro-ophthalmologists, neuroradiologists, neuropathologists, radiation oncologists) who promote collaboration and apply best practices to improve results and overall outcomes in patients with pituitary tumors and related disorders. Again, in accordance with McLaughlin and colleagues, the 3 primary missions to be fulfilled by the centers to be recognized as a PCOE are the following: providing multidisciplinary, optimal clinical care to patients with pituitary tumors and related disorders; providing residency, fellowship training, and/or continuing medical education and patient support; and contributing to research in the field of pituitary disorders.

Object Transnasal endoscopic (TNE) approaches have been proposed for the resection of anterior cranial base meningiomas. The purpose of this article was to evaluate the results of endoscopic resection of anterior cranial fossa meningiomas by reviewing available published data in addition to the authors' experience with 13 cases. Methods The literature was searched via OVID to identify all available disaggregate data pertaining to anterior fossa meningiomas treated primarily by TNE. In addition, the authors reviewed the records of a personal series of 13 patients who underwent TNE removal of parasellar meningiomas through a pure TNE approach. Results Meta-analysis from studies included a total of 69 patients with adequate disaggregated data to summarize. Mean age (± SD) at surgery was 53.3 ± 13.0 years (range 27–80 years); 78% of the patients were women. Tumor size was skewed toward smaller lesions, with a mean volume of 24.4 ± 2.9 cm3. Intraoperative impressions were of gross-total resection in 76% of cases. The most common complication reported was CSF leakage, occurring in 32% of the cases (22 of 69). The rate of CSF leakage was not affected by size (p = 0.52), location of tumor (p = 0.9), or age (p = 0.56). There was 1 death overall. The mean duration of follow-up was 21 ± 18 months. Conclusions Transnasal endoscopic resection of anterior cranial base meningiomas is feasible in selected cases. Reported resection rates are adequate, although the follow-up in the reported series is too short (mean 21 months) to make definitive conclusions regarding the long-term effectiveness. Cerebrospinal fluid leakage is a common complication, although it appears not to be associated with additional morbidity except for the need for reoperation. Long-term results are necessary before considering TNE resection as a valid alternative, in selected cases, to the more established transcranial techniques.