Sellar Region Research Articles

TMIC-02. SINGLE-CELL HIGH-DIMENSIONAL MASS CYTOMETRY (CYTOF) REVEALS IMMUNE LANDSCAPE OF PITUITARY NEUROENDOCRINE TUMORS

Abstract Pituitary neuroendocrine tumors (pitNETs) are a group of neoplasms in the sellar region, ranking as the second most common primary brain tumors, with some exhibiting aggressive behaviors. While immunohistochemical staining has been utilized to characterize the tumor immune microenvironment (TIME) of pitNETs, single-cell analysis remains underexplored. High-dimensional mass cytometry (CyTOF) offers a valuable approach for precise quantification of intratumoral leukocytes and potential therapeutic insights. We prospectively enrolled 56 patients with pitNETs of varying endocrine function, histology, and lineage. CyTOF, employing heavy metal-tagged antibodies, was employed to simultaneously measure 37 cellular parameters at single-cell resolution. Analysis revealed a total of 71,049 CD45+ immune cells. Macrophages (mean, 50.7%) and T cells (mean, 28.7%) were the predominant leukocytes, followed by NK cells, dendritic cells, B cells, and neutrophils. The immune cell composition within the TIME exhibited heterogeneity, with distinct patterns observed between functioning and non-functioning pitNETs. Functioning pitNETs displayed fewer macrophages (28.9% vs. 62.9%) and more T cells (44.9% vs. 19.6%) compared to non-functioning pitNETs. Subgroups within functioning (somatotroph, mammosomatotroph, lactotroph) and those within non-functioning pitNETs (null-cell, gonadotroph, corticotroph) shared similar immune profiles, respectively. PIT-1 lineage pitNETs demonstrated a T cell-dominated pattern, while SF-1, T-PIT, and null-cell lineage pitNETs exhibited macrophage predominance. In functioning pitNETs, macrophage abundance negatively correlated with invasiveness, Ki-67 index, and recurrence, while CD4 T cells showed positive correlations. Further unsupervised clustering identified 27 subpopulations of intratumoral immune cells, with some serving as critical predictors of tumor behavior. Our study provides a comprehensive single-cell immune profile of pitNETs, revealing distinct features across subtypes and targeted therapeutic implications.

EPID-35. COLLISION TUMORS AT THE SKULL BASE: UNVEILING A PETROCLIVAL MENINGIOMA COEXISTING WITH A GROWTH HORMONE-PRODUCING PITUITARY ADENOMA

Abstract Collision tumors are rare occurrences characterized by coexistence of two histologically different tumor types within the same anatomical location. While collision tumors involving skull base meningiomas and pituitary adenomas are uncommon, they pose distinct diagnostic challenges. We report the case of a 57-year-old female initially diagnosed with petroclival meningioma WHO 1, subsequently found to harbor a collision tumor with a growth hormone-producing pituitary adenoma. The patient initially presented with aphasia, right-sided weakness, and gait instability, prompting a diagnosed of left petroclival mass with left temporal horn entrapment. Patient underwent craniotomy and partial resection of the tumor in 2022 and was diagnosed with meningothelial meningioma, WHO1. The patient had history of uncontrolled diabetes and physical features of large hands with hypertrophied terminal phalangeal tufts but patient reported that she has had similar hands since childbirths for decades. Screening pituitary function tests were ordered due to the petroclival meningioma involving suprasellar region, revealing significantly elevated growth hormone, and insulin-like growth factor-1(IGF-1) levels, raising suspicion for pituitary adenoma. Subsequent endoscopic resection of the sellar mass via a transsphenoidal approach in 2024 confirmed the presence of somatotroph pituitary neuroendocrine tumor. Retrospectively growth hormone producing pituitary adenoma likely contributed to patient’s uncontrolled diabetes and coarse, large hands. This case highlights the importance of a high index of suspicion for collision tumors, particularly in patients with skull base tumors involving supra sellar region, especially when presenting with atypical clinical features.

Improving the radiological prediction of surgical resection of nonfunctioning pituitary adenomas.

Nonfunctioning pituitary adenomas (NFPAs) are benign tumors growing in the sellar region. Total surgical excision of the lesion is recommended as the preferred treatment choice with preservation of adjacent structures. The objective is to establish a radiological score to predict the feasibility of NFPA total surgical excision. Patients with treatment-naïve NFPA who underwent a transsphenoidal approach and sellar magnetic resonance imaging (MRI) in the preoperative period and 3 months after surgery were included. Data on age, sex, tumor diameter, extrasellar extension, postoperative cure rates, and hormone and transcription factor expression were collected. A combined score was proposed based on Knosp and SIPAP classifications. We proposed 3 classification groups depending on the tumoral extension to the suprasellar, infrasellar, anterior and posterior directions of the sellar region. A total of 164 patients were included in the study, and 85 (52%) were female. Total excision was obtained in 46% (n = 75) of the patients. The majority of tumors were of gonadotrophic lineage (59%), followed by corticotrophic (17%) and other less common types. Largest tumor diameter was 6.8cm [mean 3.8cm (± 1.1cm)]. From the established groups, 10 patients were classified in Group I, of whom 8 (80%) patients underwent total excision, 115 patients were classified in Group II, of whom 58 (50%) underwent complete excision and 39 patients in Group III, of whom 9 (23%) underwent complete excision (p value < 0.001). The newly proposed score helps to determine the feasibility of total NFPA excision, allowing for better surgical planning and predictions of postoperative outcomes.

Concurrent meningioma and intracranial aneurysm: Insights from an updated systematic review and a case report

Background: The concurrent presentation of meningioma and intracranial aneurysm (IA) poses diagnostic and therapeutic challenges, with no standardized management protocol available. This study aims to address this through an updated systematic review, delineating optimal strategies for managing this dual pathology. Methods: A systematic review was conducted across PubMed, Web of Science, and Embase databases. Articles were screened independently by two reviewers. Treatment strategies and patient outcomes were comprehensively analyzed to formulate a treatment framework based on several characteristics. In addition, one concurrent meningioma and IA case from our institution was presented. Results: A total of 69 articles comprising 115 patients were included in the study. The cohort exhibited a female predominance (80%) with a mean age of 56 (±13) years. Meningiomas were primarily localized to the frontotemporal and sellar regions, while aneurysms favored the anterior circulation – notably, 16.5% of cases presented with ruptured aneurysms. Management strategies varied based on the spatial relationship between lesions and aneurysm rupture status. In unruptured cases, 34% underwent a single craniotomy for simultaneous resection of both pathologies, while endovascular intervention was favored when the IA originated from an artery feeding the meningioma (73%). Remarkably, postoperative aneurysm rupture occurred in 33% of cases managed solely through tumor resection (range 0–30 days postop). Conclusion: This study proposes a comprehensive treatment algorithm to guide neurosurgeons in managing concurrent meningioma and IA cases. By considering individual patient intricacies, the feasibility of simultaneous management, aneurysm rupture risk, and symptomatology, this framework is a valuable tool for clinical decision-making in these complex scenarios.

Exploring the Potential Use of Virtual Reality with a Supraorbital Keyhole Craniotomy for Anterior Skull Base Meningiomas: Two Case Reports

Introduction: A supraorbital keyhole craniotomy (SOKC) is a novel alternative to frontal craniotomies for accessing the anterior fossa for resecting tumors and clipping aneurysms; however, its implementation is limited in patients at a high risk of complications. We present two cases involving the use of augmented reality (AR) and virtual reality (VR) for patient selection and preoperative planning for a supraorbital tumor resection of anterior fossa meningiomas. Methods: This is a prospective, single-center case series at a research institute. We identified patients with an anterior or middle fossa meningioma regardless of age, gender, and tumor characteristics who could undergo an SOKC and MRI. The preoperative planning was performed with the BrainLab Magic Leap AR/VR platform. The meningiomas were resected through the SOKC under neuronavigation. Results: We identified two cases: a 37-year-old male with a meningioma in the sellar region and an 84-year-old male with a right anterior fossa meningioma, both confirmed by MRI. Both patients had a complete tumor resection by a minimally invasive SOKC after preoperative planning with the AR/VR platform. Postoperatively, hyponatremia complicated the first case, while the second case developed an intracranial hemorrhage. They both recovered after the appropriate interventions. Conclusions: The use of an SOKC for anterior skull base meningiomas should be individualized after considering the lesion characteristics, vascular control needs, and the surgeon’s expertise. VR/AR-assisted preoperative evaluation and planning will optimize the patient selection and surgical outcomes. We can utilize VR/AR technologies to identify patients that will benefit from an SOKC and expand the implementation of the approach beyond its current limitations.

Metastatic Intracranial Choriocarcinoma in the Absence of a Primary Lesion: A Case Report

AbstractIntracranial choriocarcinoma is a rare and aggressive neoplasm characterized by the proliferation of trophoblastic tissue. Although choriocarcinoma most commonly arises in the uterus as a component of gestational trophoblastic neoplasia, instances of intracranial choriocarcinoma are exceptionally uncommon. We report a case of intracranial choriocarcinoma without any evidence of a tumor elsewhere. A 25-year-old woman presented with a history of 1 month of evolution with right frontal hemicranial headache, followed by visual disturbances, otalgia, and diplopia. On neurological examination, she was conscious, cooperative, and well-oriented; a grade 1 bilateral papilledema, left homonymous hemianopsia, and sixth cranial nerve paresis, with diplopia, were detected. Neuroimaging showed a right parieto-occipital lesion with features mimicking an atypical meningioma. After surgical resection, a diagnosis of choriocarcinoma was issued. Primary intracranial choriocarcinomas are typically located in the sellar and pineal regions. The occurrence of this tumor within the occipital lobe suggested metastasis; however, a primary tumor in the thoracic or abdominal organs was not observed and a delayed metastasis was considered. This case highlights the diagnostic challenges associated with intracranial choriocarcinoma.

6791 Pituitary Apoplexy as Presentation of Atypical Teratoid/Rhabdoid Tumor

Abstract Disclosure: S.I. Babli: None. H. Alkubedan: None. N.M. Ali: None. R. Alqawahmed: None. M.M. Elhassan: None. Introduction: Pituitary apoplexy occurs in a pituitary adenoma; it migh occur in other less common types of tumors. The atypical teratoid/rhabdoid tumor (ATRT) is a very aggressive malignant tumor of the central nervous system and has a poor prognosis. It is rare to occur in adult and in the sellar region. Clinical Case: 32 years old male presented to ER with 4 days history of fever, headache and vomiting. He was conscious but confused. Vital signs Temperature 39 C, BP 120/80, HR 110 beats/min and Saturation of 95 %, Possibility of meningoencephalitis was raised, patient Brain CT showed Sellar and suprasellar mass with compression over the optic chiasma and invasion to the left cavernous sinus. Patient suddenly developed Generalize tonic-clonic convulsion and hypotension. He received IV midazolam, intubated and was started on noradrenaline and hydrocortisone, he was admitted to ICU as a case of meningoencephalitis and started on Ceftriaxone, Vancomycin and Acyclovir. Cerebrospinal fluid analysis showed RBC 175/cumm, WBC 20/cumm with 20% polymorph and 80% mononuclear cells, Protein 1.38 g/l (0.24-0.49) glucose 7.9 mmol/l with plasma glucose of 10 mmol/l. Repeated Brain CT showed Sellar and suprasellar mass with compression over the optic chiasma and invasion to the left cavernous sinus and with minimal heterogenicity and increased density suggestive of hemorrhagic components. Endocrine history revealed weight gain but no history to suggest acromegaly, Cushing’s syndrome or hypogonadism . Examinations: Patient was intubated and sedated BP 90/60 on Maximum inotropic support. Left oculomotor nerve palsy, Bilateral papilledema, he was having acanthosis nigricans with no signs of acromegaly, CS or hypogonadism. Laboratory finding: WBC17 with neutrophilia, Hgb 14.7 g/dl plt 360 Na 140 mmol/l, creatnin 180 mmol/, FT4 6.2 pmol/l, TSH 0.015, PROLACTINE 22, IGF1 29 ng/ml (41-246) Free testosterone index 2.13 (33-106), LH 0.5, FSH 0.3 .Patient had TTS and histopathology showed: SMARCB1 deficient malignant neoplasm, consistent with sellar atypical teratoid rhabdoid tumor. Post OP, patient had Ventilator associated pneumonia and recurrent attacks of seizures despite multiple antiepileptic medications. Patient was evaluated by medical and radiation oncologists but due to his general condition the treatment was postpone. One month after TSS, he developed intractable seizure new Brain CT showed interventricular haemorrhage with hydrocephalus and marked enlargement of hyperdense pituitary lesion which suggests haemorrhage. External ventricular drain was inserted but patient arrested and died. Conclusions: ATRT of the CNS usually affects young children. In the literature review performed in adults, only nine cases have occurred in the sellar or suprasellar region. Survival is variable in the different cases reported in the literature, with a mean of 26 months. Presentation: 6/2/2024

8203 A Solitary Fibrous Tumor Mimicking a Non-functional Pituitary Macroadenoma

Abstract Disclosure: L. Bernacet Rivera: None. S. Omay: None. A. Huttner: None. S.E. Inzucchi: None. Background: A solitary fibrous tumor (SFT), also known as hemangiopericytoma (HPC), is a rare subset of soft tissue tumors of mesenchymal origin. This type of tumor tends to have aggressive behavior and a high risk of recurrence. Approximately less than 1% of central nervous system (CNS) tumors have been identified as part of this subtype. Case Presentation: A 73-year-old female with medical history of primary hypothyroidism, myelodysplastic syndrome (MDS), and IgM kappa MGUS initially presented to her primary care physician for evaluation of progressive visual loss over several weeks, accompanied by worsening headaches. Ophthalmologic exam revealed bitemporal hemianopsia which prompted urgent imaging. Pituitary MRI with IV contrast revealed a solid sellar mass with suprasellar extension measuring 1.8 x 1.3 x 1.9 cm. The lesion was noted to superiorly displace the optic chiasm as well as to cause mild impingement of the anterior cerebral arteries. Patient was urgently referred to neurosurgery and endocrinology for further evaluation. Initial neuroendocrine laboratory assessment, performed at 8 am, revealed a cortisol 16.5 ug/dL, free T4 1.2 ng/dL, TSH 0.77 mIU/L, prolactin 9.9 ng/mL, IGF-1 17 ng/mL, the latter value consistent with adult growth hormone deficiency. Her LH and FSH were 4.3 mIU/mL and 12.6 mIU/mL, respectively, lower than expected for a post-menopausal woman. She underwent a transnasal transsphenoidal resection of the sellar lesion. Surgery was difficult due to the fibrous nature of the tumor, for which complete resection was not achieved. Post-operative course was complicated by thrombocytopenia due to MDS. Hormonal workup following surgery was consistent with central adrenal insufficiency, hypogonadotropic hypogonadism, and central hypothyroidism. Pathology showed densely cellular spindle cell neoplasm with a collagenous background consistent with hemangiopericytoma. She underwent fractionated radiotherapy and tumor has since remained stable in size. Her visual symptoms resolved quickly following surgery. Discussion: SFT is an aggressive mesenchymal tumor which rarely appears in the sellar region. There are no identified brain MRI findings that would suggest the nature of this tumor. As a result, endocrinologists should be aware of this rare condition while evaluating pituitary neoplasms. Presentation: 6/3/2024

PDOC-01 THERAPEUTIC DIFFICULTY IN THE MANAGEMENT OF A RECURRENT CRANIOPHARYNGIOMA IN A CHILD: REVIEW OF THE LITERATURE AND CASE REPORT

Abstract BACKGROUND Craniopharyngiomas are rare, benign epithelial tumors of embryonic origin in children that develop along the craniopharyngeal duct from Rathke’s pouch. The optimal treatment algorithm remains challenging. We report a child case of recurrent craniopharyngioma with therapeutic challenges. CASE PRESENTATION 12-year-old patient, followed for a multi-operated type 2 craniopharyngioma (December 2017, November 2018, and April 2022), stroke on left sylvian stenosis with right hemiplegia in 2020 during proton therapy (52 Gray), thrombolysed with good recovery after rehabilitation, sequellar panhypopituitarism. He was seen in consultation for visual field deterioration. Clinical examination showed a conscious, coherent, obese patient with a deteriorating visual field. Ophthalmological examination showed visual acuity 10/10 with slight temporal visual field amputation on the left and monocular blindness on the right due to probable optic atrophy sequelae of the craniopharyngioma. Magnetic resonance imaging revealed progression of the residual multicystic lesion in the sellar and left temporal regions, entering the sylvian valley and severely compressing the optic pathways. The tumor was removed under general anesthetic in a supine position, with release of the optic nerve, the carotid artery, and its division, and the left 3rd cranial nerve. The postoperative course is straightforward. CONCLUSION Morbidity and the assessment of sequelae are now at the forefront of the management of craniopharyngiomas. The current therapeutic approach should be reconsidered, in terms of the indications for surgery and the surgical protocol. This patient’s case is a typical example of the therapeutic difficulties involved in the management of craniopharyngiomas.

Relationship between optic nerve length and interoptic angle in the prediction of optic chiasm location

Introduction and objectivesThe sellar region is an area in the base of the skull that is among the most common sites for tumors of the central nervous system. Surgical interventions are currently performed via different routes. While the optic chiasm occupies its expected position in 70% of the population, it can deviate from this position. In such cases, surgery involving this region becomes more difficult as the known surgical routes are narrowed. Advance awareness of these variations can help surgeons to identify the optimal route for safe surgical intervention in the sellar region. By performing simple measurements of both the lengths of the optic nerves and the angle between them, a surgeon can predict the location of the chiasm. Materials and methodsTwenty specimens collected from autopsies performed at Bursa Forensic Medicine İnstitute were examined to determine the optic chiasm types and the relationships between the surrounding subchiasmal structures. ResultsAmong the 20 specimens, we found two prefixed (10%), 10 normo-fixed (50%), and eight postfixed chiasms (40%). The mean interoptic angle was 81.03 (±17.41)⁰. Prefixed chiasms had angles in the range 115.36°–124.76 ° (mean 120.06 [±6.65]⁰), normo-fixed chiasm angles were between 83.11° and 97.53 ° (mean 86.07 [±6.73]⁰), and postfixed chiasms ranged between 53.01 ° and 78.71 ° (mean 69.20 [±9.13]⁰). The length of the right optic nerve ranged between 6.95 and 13.83 mm (mean 10.25 [±1.81] mm), and the length of the left between 7.25 and 12.51 mm (mean 10.40 [±1.47] mm). Obtuse angles indicated that the chiasm was prefixed, and acute angles were indicative of a postfixed chiasm. There was a strong negative correlation between optic nerve lengths and the interoptic angle; thus, as the length of the nerves increases, the interoptic angle becomes more acute. ConclusionsWe have proposed a simple measurement of the optic nerve lengths and the angle between them to predict the relative location of the OC, which can be done easily on MRI.

Obesity in patients with craniopharyngioma in the South Asian region – A distinct phenotype

BackgroundCraniopharyngiomas are rare benign tumors located in the sellar and suprasellar region, with an incidence of 0.5–2 cases per million as reported in Western studies. Post-treatment, including surgery and/or radiotherapy, many patients develop significant obesity, primarily due to hypothalamic damage and associated complications such as hypopituitarism. In the South Asian population, genetic predisposition to obesity at lower BMIs, coupled with a carbohydrate-rich diet, may exacerbate obesity in craniopharyngioma patients, presenting a unique challenge. MethodsThis submission is a commentary based on a comprehensive literature review. The authors conducted the review using PubMed to focus on English-language articles covering hypothalamic obesity, craniopharyngioma and obesity in the South Asian population from 1939 to the present. ResultsThe literature review revealed that 50–60 % of patients treated for craniopharyngioma develop obesity, predominantly linked to hypothalamic damage, although these data are mainly derived from Western studies. Hypopituitarism was frequently observed, further contributing to the obesity. Despite a caloric intake appropriate for the age and gender, these patients exhibited reduced physical activity as measured by wrist accelerometers. Patients with hypothalamic obesity due to craniopharyngioma are at risk for metabolic syndrome and cardiovascular morbidity. Additionally, visual impairment was common, leading to a decreased quality of life. The South Asian population, genetically predisposed to visceral obesity and a carbohydrate-rich diet, may display a distinct phenotype. Although multiple treatment modalities have been tried, there is no definite treatment modality available to counteract this condition at present. ConclusionSouth Asian phenotype of craniopharyngioma-related obesity is characterized by significant metabolic and hormonal dysregulation, influenced by both dietary and genetic factors. Nevertheless, there may be a lot to be still understood about this devastating, rapid, relentless hypothalamic obesity syndrome. Also, a higher morbidity rate within this population, underscores the need for further research to develop targeted interventions.

Shaping the curve from the microscopic transsphenoidal to the endoscopic endonasal approach for the sellar region

Shaping the curve from the microscopic transsphenoidal to the endoscopic endonasal approach for the sellar region

Cranio-Orbital Approach and Decompression of the Optic Nerves: A 2-Stage, 4-by-4-Step Approach to Improve Vision in Sellar and Parasellar Lesions.

Despite advances in cranial base techniques, surgery of the sellar and parasellar regions remains challenging because of complex neurovascular relationships. Lesions within this region frequently present with progressive visual deterioration caused by distortion and compression of the optic chiasm and nerves. In addition to the direct mass effect from mechanical forces acting on the optic apparatus, these lesions alter blood supply and reduce vascular perfusion, prompting surgical treatment to remove the lesion, alleviate compression, and improve blood flow to the optic nerve. We sought to describe a 2-stage, 4-by-4-step approach, broken down and described as a "four-by-four" technique for optic apparatus decompression and a wide approach to different sellar and parasellar lesions. We describe the operative nuances and key anatomic points in the microsurgical removal of sellar and parasellar lesions. The technique is illustrated with examples of different cases with pre- and follow-up MRI imaging and a brief overview of visual outcomes. The described technique has been demonstrated in various lesions in 5 patients. Patients presented with bilateral visual loss in 4 (80.0%) cases and with unilateral visual loss in 1 (20.0%) case. Improvement in visual function was noted in all cases, confirmed with visual acuity and visual field testing. The transcranial approach ("from above") remains an important surgical option for patients with excellent exposure and visualization of the sellar and parasellar regions. It permits early access to the optic canal for careful microsurgical decompression and relaxation of the optic nerve to preserve and improve its microvascularization and ultimately vision. The authors augmented the 2-stage, 4-by-4-step technique of decompression with elaborate illustrations of diverse sellar and parasellar lesions to demonstrate the versatility of this approach.

Endoscopic endonasal approach for resection of sellar leiomyosarcoma metastasis: illustrative case.

Leiomyosarcoma (LMS) is a rare neoplasm that arises from tissues of embryonic mesodermal origin. Primary tissues of origin can include smooth muscle cells of the abdominopelvic viscera, blood vessels, or arrector pili muscles. LMS is known to metastasize to the lungs, with few reported cases of spread to the central nervous system. A 66-year-old male with cutaneous LMS of the left forearm with metastases to the lungs and kidney that had been treated with chemoradiation presented with worsening headaches. Magnetic resonance imaging revealed a sellar lesion. An endocrine workup was unremarkable. Imaging over 6 months revealed rapid interval growth. Positron emission tomography demonstrated moderate uptake. Given the rapid growth, the patient was offered an endoscopic endonasal approach for resection. Pathology confirmed LMS. To the authors' knowledge, this is the first documented case of LMS metastasis to the sella. Pituitary carcinoma or metastases to the sellar region should be in the differential among patients with sellar region tumors with a rapid growth rate, bony erosion, or findings of lesions in the upper cervical lymph nodes or soft tissue. Tumors that show significant interval growth should raise suspicion for nonadenomatous lesions, and surgical intervention should be considered even in the absence of endocrinological dysfunction or cranial neuropathies. https://thejns.org/doi/10.3171/CASE2435.

Transsphenoidal Approach for Sellar Region Tumor : A Case Series

Introduction: Transsphenoidal approaches to the sellar region, with endoscopic or microscopic techniques, are regularly used to treat a diverse collection of pathologies. Case Description: Three patients with presenting symptoms visual disturbance and headache were evaluated with MRI that showes sellar region masses. All patients were undergo transphenoid tumor removal. Post operation evaluation show improvement in visual disturbance and headache with no adverse event after procedure. Discussion : Transsphenoidal endoscopic surgery (TSS) for functional pituitary adenomas yields better endocrinologic results for noninvasive macroadenomas. However, the rate of postoperative CSF leakage was greater with the endoscopic method. In patients with pituitary adenomas, younger age, dense visual field defect, and the preoperative absence of central or bilateral visual field abnormalities were predictive factors of visual field recovery following transsphenoidal approach-tumor excision. Overall survival rates are generally high, ranging from 91% to 98%. Conclusion: Transphenoid approach was a good surgical method for sellar region masses and show good outcomes after operation. Keyword : Sellar region masses; Surgical; Transsphenoid approach

Rathke's cleft cysts: from pathophysiology to management.

Rathke's cleft cysts (RCCs) are benign, non-neoplastic lesions located in the sellar and suprasellar regions of the brain, originating from remnants of Rathke's pouch, an embryonic precursor to the anterior pituitary gland. Although RCCs are frequently asymptomatic and discovered incidentally during imaging studies, they can present with a variety of symptoms, including headaches, visual disturbances, and endocrine dysfunction due to the compression of adjacent neural structures. The management of RCCs is particularly challenging, as the decision to pursue conservative monitoring or surgical intervention depends heavily on the cyst's size, growth potential, and the severity of symptoms. Transsphenoidal surgery is the primary treatment for symptomatic RCCs, offering effective relief from symptoms through decompression of the cyst. However, recurrence remains a significant issue, with rates reported up to 33%, prompting debates about the extent of cyst wall removal during surgery. Recent advancements in minimally invasive endoscopic techniques have improved surgical outcomes, yet the risk of postoperative complications such as hypopituitarism and cerebrospinal fluid leaks persists. Additionally, stereotactic radiosurgery has emerged as a potential alternative for patients with recurrent RCCs or those who are not suitable candidates for repeat surgery. Despite its promise, the long-term safety and efficacy of radiotherapy in RCC management require further investigation. This narrative review aims to provide a comprehensive overview of RCCs, integrating the latest research and clinical guidelines to discuss pathophysiology, clinical presentation, and management strategies, emphasizing the need for a personalized approach to treating this complex condition.

Cavernous hemangioma within the cavernous sinus: illustrative case.

Cerebral cavernous hemangiomas (CHs) are the most common vascular malformations and can be found in many locations in the brain. The most common extra-axial locations are the intrasellar and parasellar regions. Both locations are rare for CH. A 41-year-old female who had experienced headaches for about a year presented to the authors' institution because of a mass located in the left cavernous sinus (CS). It was confirmed that it was not causing any loss in the visual field and that there was no pituitary irregularity. Surgery was planned with a preliminary diagnosis of CH. An endoscopic endonasal approach (EEA) was undertaken with the otorhinolaryngology team. The dura mater was opened following excision of the back wall of the sphenoid sinus. A blue-purple vascular lesion was observed, filling the left CS. Gross-total resection (GTR) was achieved. In the literature, there were 10 cases in which GTR had been performed using the EEA. Patients with CHs located in the sellar region present with various complaints. The preference for endoscopic surgery over cranial surgery for such lesions requires more comprehensive studies, but it is thought that this approach can reduce surgical complications and the time to discharge for the patient. https://thejns.org/doi/abs/10.3171/CASE24227.

The 2022 WHO classification of tumors of the pituitary gland: An update on aggressive and metastatic pituitary neuroendocrine tumors.

The vast majority of pituitary neuroendocrine tumors (PitNETs) are benign and slow growing with a low relapse rate over many years after surgical resection. However, about 40% are locally invasive and may not be surgically cured, and about one percentage demonstrate an aggressive clinical behavior. Exceptionally, these aggressive tumors may metastasize outside the sellar region to the central nervous system and/or systemically. The 2017 (4th Edition) WHO Classification of Pituitary Tumors abandoned the terminology "atypical adenoma" for tumors previously considered to have potential for a more aggressive behavior since its prognostic value was not established. The 2022 (5th Edition) WHO Classification of the Pituitary Tumors emphasizes the concept that morphological features distinguish indolent tumors from locally aggressive ones, however, the proposed histological subtypes are not consistent with the real life clinical characteristics of patients with aggressive tumors/carcinomas. So far, no single clinical, radiological or histological parameter can determine the risk of growth or malignant progression. Novel promising molecular prognostic markers, such as mutations in ATRX, TP53, SF3B1, and epigenetic DNA modifications, will need to be verified in larger tumor cohorts. In this review, we provide a critical analysis of the WHO guidelines for prognostic stratification and diagnosis of aggressive and metastatic PitNETs. In addition, we discuss the new WHO recommendations for changing ICD-O and ICD-11 codes for PitNET tumor behavior from a neoplasm either "benign" or "unspecified, borderline, or uncertain behavior" to "malignant" neoplasm regardless of the clinical presentation, histopathological subtype, and tumor location. We encourage multidisciplinary initiatives for integrated clinical, histological and molecular classification, which would enable early recognition of these challenging tumors and initiation of more appropriate and aggressive treatments, ultimately improving the outcome.

"Evaluating surgical approaches for Rathke's cleft cysts and sellar meningiomas: factors influencing treatment of choice".

This study reviews recent progress in the surgical treatment of Rathke's cleft cysts (RCCs) and Sellar region meningiomas, based on findings from three key studies. RCCs are benign, fluid-filled remnants from pituitary gland development that are usually asymptomatic and found by chance. However, surgical intervention is needed when they become symptomatic or increase in size. Research by Stefan Linsler et al. and others examines various surgical methods, including transcranial keyhole and transsphenoidal techniques for RCCs, and endoscopic endonasal and supraorbital keyhole approaches for Sellar meningiomas. The results show that both transcranial keyhole and transsphenoidal surgeries for RCCs have high success rates with no recurrences over 5.7 years, although the keyhole approach has fewer complications. For Sellar meningiomas, the choice between endoscopic endonasal and supraorbital keyhole techniques should be based on tumor characteristics, highlighting the importance of surgeon proficiency in both methods. These studies emphasize the need for personalized treatment strategies tailored to patient and tumor characteristics and highlight the importance of ongoing surgical skill development and further research to refine minimally invasive techniques. This study highlights the crucial role of personalized surgical approaches in improving outcomes for patients with RCCs and Sellar region meningiomas.

Risk factors associated with postoperative cerebrospinal fluid leak after endoscopic endonasal skull base surgery: Two-center retrospective cohort study.

Over the past two decades, endoscopic endonasal skull base surgery (EESBS) has revolutionized the treatment of skull base tumors by enabling minimal access to resect significant pathologies such as meningiomas, pituitary adenomas, and chordomas. Despite its efficacy, complications such as cerebrospinal fluid (CSF) leak pose significant challenges, with an average incidence of 8.9% following EESBS. Therefore, our study aims to investigate the risk factors associated with postoperative CSF leak after employing an endoscopic endonasal approach for skull base surgery, focusing on patients treated at King Abdul-Aziz Medical City in Jeddah and King Abdullah Medical City in Makkah, Saudi Arabia. A retrospective review of patients who underwent an endoscopic endonasal approach for the resection of intradural skull base pathology between January 2016 and December 2022 was performed with a total of 51 patients. Basic demographic data were collected, along with patient comorbidities, presenting symptoms, tumor pathology, tumor site, the extent of resection, and outcomes. 51 participants were analyzed, with a mean age of 43.41. Male participants comprised (58.8%), while headaches were the most common symptom (74.5%), followed by visual disturbances (51.0%). Nonsecretory tumors predominated, primarily located in the sellar and suprasellar regions (58.8%). Gross total resection was performed in the majority (56.9%), with a 9.8% incidence of postoperative CSF leak. Notably, all four patients with CSF leak experienced preoperative headaches. Hypertension was prevalent in Cases 1 and 4, with Case 4 having a history of neurological disease and radiation therapy. Anterior skull base tumors were most frequent in Cases 3 and 4. The incidence of CSF leakage after surgery in this study was similar to that previously reported. It is worth noting that repeated surgeries may increase the risk of postoperative CSF leakage. Therefore, it is important to carefully evaluate the surgical approach for tumor removal and skull base reconstruction, considering the tumor characteristics and the patient's overall condition.