Successful treatment of medically and surgically refractory lymphocytic hypophysitis with fractionated stereotactic radiotherapy: a single-center experience and systematic literature review.

PURPOSE: To explore the potential role of focused radiotherapy in managing the lymphocytic hypophysitis (LH) refractory to medical therapy and surgery. METHOD: A systematic literature review was conducted following PRISMA guidelines to identify the studies on radiation treatment for hypophysitis, along with the experience in our institution. RESULTS: The study included eight patients, three from our institution and five from existing literature. The age at presentation ranged from 37 to 75 years old, with a median age of 58. The presenting symptoms involved headache in seven patients and diplopia in two patients. Pre-radiation visual field defects were noticed in four patients. All patients exhibited variable degrees of hypopituitarism before radiation, with oral corticosteroids being the initial medical treatment. Immunosuppressive therapy was attempted in two patients prior to radiation. Seven patients had a history of transsphenoidal surgery with a histologically confirmed LH. Three patients underwent stereotactic radiosurgery (SRS), while the remaining received FSRT, with a mean irradiation volume of 2.2 cm3. A single-session total dose of 12 -15 Gy was administered in the SRS group. In the FSRT group, doses ranged from 24 to 30 Gy with a median dose of 25 Gy, delivered in 2 Gy fractions. Four patients achieved a resolution of visual field defects, while another two patients demonstrated improvement in their associated focal neurologic deficits. No change in pre-existing endocrine status was shown after radiation, except in one patient. Clinical response was achieved in seven patients after a single course of radiation, while one patient required the second course. Six patients remained stable on low-dose glucocorticoid during at least a 12-month follow-up period, and one discontinued it entirely without experiencing relapse. Three patients demonstrated a complete radiologic response, while the remaining showed a partial radiologic response. CONCLUSIONS: Focused radiation, including FSRT, can play a role in symptomatic relief, effective mass shrinkage, and minimizing radiation exposure to critical surrounding structures in patients with refractory LH. However, further research efforts are necessary to better clarify its effects and optimal dose planning.

Tuberculum meningioma with recovery of glaucoma-like visual field defects after chiasmal decompression: a case report.

BACKGROUND: To report a case of tuberculum meningioma with recovery of glaucoma-like visual field defects after chiasmal decompression. CASE PRESENTATION: A 39-year-old woman presenting with headache was found to have bilateral arcuate retinal nerve fiber layer (RNFL) thinning on optical coherence tomography (OCT) with a corresponding arcuate scotomas consistent with glaucomatous change. However a suprasellar tumor compressing the anterior chiasm from below was found on magnetic resonance imaging of the brain. After resection of the mass, which was diagnosed as meningothelial meningioma by the pathological examination, the glaucoma-like visual field defects resolved despite the RNFL thinning on the OCT showing no improvement. CONCLUSIONS: Chiasmal compression may mimic glaucoma and produce arcuate scotoma rather than temporal visual field loss. There is a possibility that the development of chiasmal compression somehow converted preperimetric glaucoma into a more advanced form accompanied by visual field defects and that the glaucoma reverted to the preperimetric state after chiasmal decompression.

Prenatal dispositions and genetic analysis of monozygotic female twins with suprasellar cysts and hydrocephalus: A case report.

INTRODUCTION: We present a unique case of monozygotic female twins with virtually identical clinical and radiological presentations of supratentorial hydrocephalus and cystic formations from the suprasellar cistern. DISCUSSION: Evaluating genetic predispositions and prenatal exposures is crucial for hydrocephalus in twins. Familial cases imply a genetic contribution to the development of these anomalies, including chromosomal abnormalities and specific variants linked to arachnoid cyst formation in various syndromes. Extensive genetic analyses found no pathogenic variants in the twins. Prenatal exposure to anti-epileptic medication was known during pregnancy and may be associated with fetal abnormalities, but not central nervous system (CNS) malformations, and was therefore not considered the cause of the condition in the twins. The twins presenting simultaneously with hydrocephalus caused by suprasellar cysts (SAC) underwent a two-step surgical management: initial ventriculoperitoneal shunt (VPS) placement followed by fenestration. Postoperative imaging showed cyst reduction, but a secondary VPS was necessary in both cases. CONCLUSION: Genetic analysis is less likely to identify a monogenic etiology in non-syndromic cases of SACs, which are assumed to be multifactorial. There is no established evidence linking a teratogenic effect of anti-epileptic drugs to CNS malformations. Moreover, the surgical treatment of this complex condition constitutes a point of discussion.

Classification of the relationship between suprasellar arachnoid cyst and hydrocephalus based on treatment modalities: shunting versus neuroendoscopic approaches.

PURPOSE: Children diagnosed with suprasellar arachnoid cysts often concurrently have hydrocephalus. This study aims to classify the relationship between suprasellar arachnoid cysts and hydrocephalus, discussing surgical strategies-shunting or neuroendoscopic approaches-and their sequence, based on this classification. METHODS: A retrospective analysis was conducted on 14 patients diagnosed with suprasellar arachnoid cysts and hydrocephalus, treated surgically by the first author between January 2016 and December 2020. Clinical features, radiological findings, surgical strategies, and outcomes were reviewed. The classification of the relationship between the suprasellar arachnoid cysts and hydrocephalus was developed and illustrated with specific cases. Recommendations for future surgical management based on this classification are provided. RESULTS: We classified the relationship between suprasellar arachnoid cysts and hydrocephalus into three categories. SACH-R1, the direct type, represents cases where the cysts cause obstructive hydrocephalus. Here, neuroendoscopic ventriculocystocisternostomy (VCC) effectively treats both conditions. SACH-R2, the juxtaposed type, involves concurrent occurrences of cysts and hydrocephalus without a causative link. This is further subdivided into SACH-R2a, where acute progressive communicating hydrocephalus coexists with the cyst, initially managed with a ventriculoperitoneal shunt, followed by VCC upon stabilization of hydrocephalus; and SACH-R2b, where the cyst coexists with chronic stable communicating hydrocephalus, first addressed with VCC, followed by monitoring and potential secondary shunting if needed. Key factors differentiating SACH-R2a from SACH-R2b include the patient's age, imaging signs of fourth ventricle and cisterna magna enlargement, and the rapid progression or chronic stability and severity of hydrocephalus symptoms. SACH-R3, the reverse type, describes scenarios where shunting for hydrocephalus leads to the development or enlargement of the cyst, managed via neuroendoscopic VCC with precautions to prevent infections in existing shunt systems. CONCLUSION: The simultaneous presence of suprasellar arachnoid cysts and hydrocephalus requires a nuanced understanding of their complex relationship for optimal surgical intervention. The analysis and classification of their relationship are crucial for determining appropriate surgical approaches, including the choice and sequence of shunting and neuroendoscopic techniques. Treatment should be tailored to the specific type identified, rather than blindly opting for neuroendoscopy. Particularly for SACH-R2a cases, we recommend initial ventriculoperitoneal shunting.

Safety and efficacy of hydroset cranioplasty as an adjunct to gasket-seal and nasoseptal flap closure of the skull base. A case-controlled study.

BACKGROUND: Cerebrospinal fluid leak after endoscopic skull base surgery remains a significant complication. Several investigators have suggested Hydroset cranioplasty to reduce leak rates. We investigated our early experience with Hydroset and compared the rate of nasal complications and CSF leak rates with case-controlled historic controls. METHODS: We queried a prospective database of patients undergoing first time endoscopic, endonasal resection of suprasellar meningiomas and craniopharyngiomas from 2015 to 2023. We compared cases closed with a gasket seal, Hydroset, and a nasoseptal flap with those closed with only a gasket seal and nasoseptal flap. Demographics, technical considerations and postoperative outcomes (SNOT-22) were compared. RESULTS: Seventy patients met inclusion criteria, twenty patients in the Hydroset group (meningioma n = 12; craniopharyngioma n = 8) and 50 control patients (meningioma n = 25; craniopharyngioma n = 25). CSF diversion was used in fewer Hydroset patients (75%, 15/20) compared with control group (94%, 47/50; p = 0.02). CSF leak was less frequent in the Hydroset than the control group (5% versus 12%, p = 0.38). One Hydroset patient required delayed nasal debridement. SNOT-22 responses demonstrated no significant difference in sinonasal complaints between groups (Hydroset average SNOT-22 score 22.45, control average SNOT-22 score 25.90; p = 0.58). CONCLUSIONS: We demonstrate that hydroxyapatite reconstruction leads to improved CSF leak control above that provided by the gasket-seal and nasoseptal flap, without significant associated morbidity as long as the cement is fully covered with vascularized tissue.

Customized ventral bony and dural opening in the transplanum/transtuberculum and transclival variants of extended endoscopic endonasal approach to suprasellar craniopharyngiomas: an approach-based stepwise cadaveric dissection and clinical applicability.

BACKGROUND: Optimal initial exposure through an extended endoscopic endonasal approach (EEA) for suprasellar craniopharyngiomas ensures safe and unrestricted surgical access while avoiding overexposure, which may prolong the procedure and increase neurovascular adverse events. METHOD: Here, the authors outline the surgical nuances of a customized bony and dural opening through the transplanum/transtuberculum and transclival variants of the extended EEA to suprasellar craniopharyngiomas based on the tumor-pituitary stalk relationship. A stepwise cadaveric dissection and intraoperative photographs relevant to the approaches are also provided. CONCLUSION: Safe maximal resection of suprasellar craniopharyngiomas through extended EEAs can be feasibly and safely achieved by implementing of tailored ventral exposure.

Massive traumatic epidural hematoma in a child with suprasellar arachnoid cyst and hydrocephalus.

We present a case of a child with a suprasellar arachnoid cyst and hydrocephalus who developed a massive traumatic epidural hematoma following a fall. This represents the first reported case of such a condition. The case is characterized by a progressive increase in hemorrhage leading to a massive hematoma, yet with relatively mild clinical symptoms. The hemorrhage originated from extensive blood seepage from the dura mater. Intraoperative hemostasis was challenging, and there was a large residual cavity of the epidural hematoma without repositioning of brain tissue after removal of the hematoma. Surgical measures such as extensive continuous compression hemostasis with Surgicel, the half-suspension technique, and continuous external drainage were employed to address these challenges. A second-stage surgery for the treatment of the suprasellar arachnoid cyst was performed 1.5 months after hematoma evacuation, utilizing neuroendoscopic ventriculocisternostomy (VCC). We recommend that for patients with traumatic brain injury and hydrocephalus, especially those with skull fractures or minimal intracranial hemorrhage, relying solely on clinical symptom observation and monitoring is insufficient. Timely and close monitoring with cranial CT is crucial for the early detection of progressive intracranial hemorrhage.

Anterior-posterior diameter is a key driver of resectability and complications for pituitary adenomas with suprasellar extension in endoscopic transsphenoidal surgery.

BACKGROUND: As endoscopic transsphenoidal approaches are more routinely selected for progressively larger pituitary adenomas with parasellar extension, understanding potential anatomical factors that limit resection and contribute to complications is becoming increasingly important for tailoring a surgical approach. This study aimed to reevaluate existing predictive tools for resectability in pituitary adenomas specifically with suprasellar extension, and furthermore identify any additional measurable features that may be more useful in preoperative planning. METHODS: A single-center retrospective chart review of adult patients who underwent endoscopic transsphenoidal surgery for pituitary adenomas with suprasellar extension from 2015 to 2020 was performed. Preoperative MRIs were systematically assessed to assign a Knosp classification, a Zurich Pituitary Score (ZPS), and for dimensional measurements of the suprasellar aspect of the lesions. Univariate comparisons and multivariate regression models were employed to assess the influence of these factors on extent of resection and postoperative complications. RESULTS: Of the 96 patients with suprasellar pituitary adenomas who underwent endoscopic transsphenoidal surgery, 74 patients (77%) had a gross total resection (GTR). Neither Knosp grade nor ZPS score, even when dichotomized, demonstrated an association with GTR (Knosp 3A-4 versus Knosp 0-2, p = 0.069; ZPS III-IV versus ZPS I-II, p = 0.079). Multivariate regression analysis identified suprasellar anterior-posterior tumor diameter (SSAP) as the only significant predictor of extent of resection in this cohort (OR 0.951, 95% CI 0.905-1.000, p = 0.048*). A higher SSAP also had the strongest association with intraoperative CSF leaks (p = 0.0012*) and an increased overall rate of postoperative complications (p = 0.002*). Further analysis of the regression model for GTR suggested an optimal cut point value for SSAP of 23.7 mm, above which predictability for failing to achieve GTR carried a sensitivity of 89% and a specificity of 41%. CONCLUSIONS: This study is unique in its examination of endoscopic transsphenoidal surgical outcomes for pituitary adenomas with suprasellar extension. Our findings suggest that previously established grading systems based on lateral extension into the cavernous sinus lose their predictive value in lesions with suprasellar extension and, more specifically, with increasing suprasellar anterior-posterior diameter.

Clinical value of classification in the treatment of children with suprasellar arachnoid cysts.

OBJECTIVE: The aim of this study is to investigate the clinical characteristics and prognostic effects of different subtypes of suprasellar arachnoid cysts (SAC) in children. METHODS: Fifty patients with primary SAC who had undergone endoscopic treatment in our department between January 2010 and December 2020 were studied retrospectively. All patients underwent endoscopic treatment after diagnosis, including ventriculocystostomy (VC) in 23 cases and ventriculocystocisternostomy (VCC) in 27 cases. All patients were followed up regularly after the operation, including head computed tomography (CT)/magnetic resonance imaging (MRI), and Evans index (EI) and frontal and occipital horn ratio (FOHR) index were measured to assess changes in cyst volume and hydrocephalus. The prognosis was evaluated comprehensively on the data of the improvement of clinical symptoms, child growth and development correlation score, and reduction of cyst volume 12 months after surgery. According to the new classification of SAC, 50 cases of children were classified into three groups in which we compared the clinical characteristics of different subtypes of the three groups. Logistic regression was used to analyze the influencing factors of prognosis. RESULTS: Completed success was achieved in 50 cases, including 31 cases with cyst volume reduction of more than 50% and 19 cases with cyst volume reduction of less than 50%. The median follow-up time was 55.3 months (22 ~ 113 months). According to the new classification criteria of SAC, there were 21 cases of SAC-1, 16 cases of SAC-2, and 13 cases of SAC-3. There were no statistically significant differences among the three groups in gender, birth weight, prenatal diagnosis, hydrocephalus, endocrine abnormalities, relief of postoperative symptoms, cyst wall texture, and surgical methods (P > 0.05). There was a statistically significant difference among the three groups in the change of the cyst volume and the maximum cyst diameter (P < 0.05), in which SAC-1 had the largest volume reduction, SAC-2 was more likely to cause endocrine symptoms and SAC-3 was inclined to lie in between. Multivariate logistic analysis showed that SAC classification and cyst wall texture were independent risk factors for the prognosis. CONCLUSION: The clinical characteristics of different SAC subtypes are different, and SAC classification is one of the independent risk factors affecting prognosis.

Intracranial arachnoid cysts.

PURPOSE: The purpose of this review article is to outline the natural history, pathogenesis, anatomic considerations and surgical decision-making in caring for patients with intracranial arachnoid cysts. METHODS: A review of the literature for intracranial arachnoid cysts was performed using Embase, PubMed, and Web of Science databases, including review of the bibliographies of eligible articles and the author's own experience. RESULTS: Among those reviewed, 59 relevant original articles were included as well as illustrative cases from the authors own experience. CONCLUSIONS: Arachnoid cysts are congenital lesions characterized by split arachnoid membrane, thick collagen in the cyst wall, absent traversing trabecular processes within the cyst, and hyperplastic arachnoid cells in the cyst wall. The underlying etiology is not entirely known, and they occur in greater proportion in males and in greater incidence with various genetic conditions including Down syndrome, mucopolysaccharidosis, schizencephaly, neurofibromatosis, autosomal dominant polycystic kidney disease (ADPKD), acrocallosal syndrome, and Aicardi syndrome. Most intracranial arachnoid cysts are incidentally found and occur in the middle cranial fossa, with the remaining occurring in the cerebellopontine angle, suprasellar cistern, quadrigeminal cistern, convexity, and posterior fossa/cisterna magna. The current article outlines the natural history, prevalence, demographic factors, and treatment decisions in managing patients with intracranial arachnoid cysts.

Craniopharyngioma in Pediatrics and Adults.

Craniopharyngiomas are rare malignancies of dysembryogenic origin, involving the sellar and parasellar areas. These low-grade, epithelial tumors account for two main histological patterns (adamantinomatous craniopharyngioma and papillary craniopharyngioma), which differ in epidemiology, pathogenesis, and histomorphological appearance. Adamantinomatous craniopharyngiomas typically show a bimodal age distribution (5-15 years and 45-60 years), while papillary craniopharyngiomas are limited to adult patients, especially in the fifth and sixth decades of life. Recently, craniopharyngioma histological subtypes have been demonstrated to harbor distinct biomolecular signatures. Somatic mutations in CTNNB1 gene encoding ß-catenin have been exclusively detected in adamantinomatous craniopharyngiomas, which predominantly manifest as cystic lesions, while papillary craniopharyngiomas are driven by BRAF V600E mutations in up to 95% of cases and are typically solid masses. Despite the benign histological nature (grade I according to the World Health Organization classification), craniopharyngiomas may heavily affect long-term survival and quality of life, due to their growth pattern in a critical region for the presence of eloquent neurovascular structures and possible neurological sequelae following their treatment. Clinical manifestations are mostly related to the involvement of hypothalamic-pituitary axis, optic pathways, ventricular system, and major blood vessels of the circle of Willis. Symptoms and signs referable to intracranial hypertension, visual disturbance, and endocrine deficiencies should promptly raise the clinical suspicion for sellar and suprasellar pathologies, advocating further neuroimaging investigations, especially brain MRI. The optimal therapeutic management of craniopharyngiomas is still a matter of debate. Over the last decades, the surgical strategy for craniopharyngiomas, especially in younger patients, has shifted from the aggressive attempt of radical resection to a more conservative and individualized approach via a planned subtotal resection followed by adjuvant radiotherapy, aimed at preserving functional outcomes and minimizing surgery-related morbidity. Whenever gross total removal is not safely feasible, adjuvant radiotherapy (RT) and stereotactic radiosurgery (SRS) have gained an increasingly important role to manage tumor residual or recurrence. The role of intracavitary therapies, including antineoplastic drugs or sealed radioactive sources, is predominantly limited to monocystic craniopharyngiomas as secondary therapeutic option. Novel findings in genetic profiling of craniopharyngiomas have unfold new scenarios in the development of targeted therapies based on brand-new biomolecular markers, advancing the hypothesis of introducing neoadjuvant chemotherapy regimens in order to reduce tumor burden prior to resection. Indeed, the rarity of these neoplasms requires a multispecialty approach involving an expert team of endocrinologists, neurosurgeons, neuro-ophthalmologists, neuroradiologists, radiotherapists, and neuro-oncologists, in order to pursue a significant impact on postoperative outcomes and long-term prognosis.

Predictive value of suprasellar extension for intracranial infection after endoscopic transsphenoidal pituitary adenoma resection.

OBJECTIVE: To investigate the relationship between suprasellar extension (SSE) and intracranial infection after endoscopic endonasal transsphenoidal approach (EETA) for pituitary adenoma resection. METHODS: We retrospectively analyzed 94 patients with suprasellar extended pituitary adenoma admitted to the Department of Neurosurgery of the Affiliated Hospital of Guilin Medical College from January 2018 to December 2021. We measured the preoperative magnetic resonance sagittal SSE and collected clinical data and divided the patients into groups according to the presence of postoperative intracranial infection. The critical value for the SSE was calculated by using a working characteristic curve for the subjects. The risk factors for intracranial infection after EETA resection of pituitary adenomas were analyzed by multivariate regression analysis. RESULTS: Among the 94 patients, 12 cases (12.8%) were placed in the infection group and 82 cases (87.2%) in the non-infection group. The cut-off value for the SSE in the sagittal position was 15.6 mm, the sensitivity was 75%, the specificity was 87.8%, and the area under the curve (AUC) was 0.801. The coronary cut-off value for the SSE was 15.8 mm, the sensitivity was 66.7%, the specificity was 79.3%, and the AUC was 0.787. The SSE values in the sagittal and coronal positions were correlated with postoperative intracranial infection (P < 0.05). After univariate analysis, those with significant differences were included in the multivariate regression analysis. It was concluded that the extension distance of the tumor above the sella in the sagittal position was ≥ 15.6 mm, the tumor texture was hard, and the postoperative cerebrospinal fluid leakage were the independent risk factors for intracranial infection after EETA resection of suprasellar extended pituitary tumors (P < 0.05). CONCLUSIONS: The value of SSE on sagittal MRI can predict intracranial infection in patients with suprasellar extended pituitary adenoma after endoscopic endonasal transsphenoidal resection. This finding recommends neurosurgeons pay more attention to the imaging characteristics of pituitary adenomas and select appropriate treatment plans in combination with the intraoperative conditions to reduce the incidence of intracranial infection.

Novel freehand frontal ventriculostomy in the supraorbital keyhole approach: technical note.

BACKGROUND AND OBJECTIVES: The narrow surgical passage provided by the supraorbital keyhole craniotomy restricts the instrument's maneuverability and presents a number of technical challenges. Inadequate brain relaxation may eventually result in unavoidable brain retraction and neurological impairments. The article aims to provide a novel intraoperative ventriculostomy to assist in overcoming the barrier of a narrow surgical corridor and assess its feasibility and safety compared to other techniques. METHODS: The craniometric point was planned on one normal computed tomography (CT) brain. The coordinates were verified on 43 magnetic resonance imaging (MRI) brain images uploaded on the neuronavigation system (StealthStation S8 version 1.0; Medtronic, Louisville, USA). The ventriculostomy point was 3 cm superior to glabella, 2 cm lateral to midline, 6 cm deep to the brain surface in the perpendicular direction, and parallel to the floor of the anterior cranial fossa. Subsequently, the abovementioned radiologically calculated ventriculostomy trajectory was confirmed on 32 consecutive patients (without neuronavigation) of suprasellar mass undergoing supraorbital craniotomy between February 2022 and April 2023. The technical issues, feasibility, and outcomes were assessed. RESULTS: Out of 32 patients, in 29 patients, ventricular hit was attained in a single attempt, and the rest 3 patients needed two attempts. The intraoperative ventricular hit rate was 100% with 90.6% success in a single attempt. No ventriculostomy-related complications occurred. Compared to ELD (external lumbar drainage), performing an intraoperative ventriculostomy had no discernible difference in the perception of the brain retraction force. Intraoperative ventriculostomy fully eliminated the low back pain or radiculopathy that patients with ELD rarely have even after drain removal. CONCLUSION: The novel intraoperative frontal ventriculostomy is a safe trajectory and is a valid alternative to Menovsky's ventriculostomy or external lumbar drainage. The authors recommend this technique be generally utilized in supraorbital keyhole approaches to optimize brain relaxation and minimize secondary adverse events.

Extended endoscopic transsphenoidal approach for suprasellar craniopharyngiomas.

BACKGROUND: Craniopharyngiomas are benign sellar lesions. Surgical excision of craniopharyngiomas is difficult because of the surrounding important neurovascular structures. The choice of surgery depends on the histological type, location, hormonal status, and size of the craniopharyngioma, surrounding neurovascular structures, and invasion of the brain parenchyma. METHODS: We describe the resection of an adamantinomatous craniopharyngioma using an extended endoscopic endonasal approach and discuss the relevant surgical anatomy, indications, limitations, and possible complications. CONCLUSIONS: The extended endoscopic endonasal approach allows successful removal of the craniopharyngioma and poses little risk to surrounding neurovascular structures.

A case report of an intra-optic recurrent craniopharyngioma.

A 46-year old female patient presented with a left-sided superior temporal quadrantanopia due to a recurrent craniopharyngioma. The location of the recurrence was unusual. Imaging showed an enlarged left optic nerve, suggestive of a recurrent intra-optic craniopharyngioma. It was possible to remove the tumour without compromising the visual functions. In this report, we describe the case in further details.

Preoperative embolization of suprasellar hemangioblastoma supplied by artery of foramen rotundum: a case report and review of the literature.

We present the case of 69-year-old woman who underwent preoperative embolization of a suprasellar hemangioblastoma supplied by the artery of foramen rotundum. To our best knowledge, this is the first such report in English. We review the literature focusing on feeding arteries of sellar and suprasellar hemangioblastomas.

Epidemiology, Management, and Long-Term Survival Outcomes of Intracranial Typical Site Germinomas: An Analysis of the Surveillance, Epidemiology, and End-Results (SEER) Database.

BACKGROUND: The correlations of epidemiological characteristics and clinical outcomes with different tumor sites in patients with intracranial typical site germinomas (ICTSGs) have not yet been well established. We analyzed ICTSGs using a multicenter database, focusing on its demographic, management patterns, and long-term survival outcomes. METHODS: Patients diagnosed with ICTSGs were selected from the Surveillance, Epidemiology, and End-Results (SEER) database. Demographic information and management patterns of ICTSGs were extracted for data analysis stratified by different tumor sites. Kaplan-Meier curves were used to evaluate the survival outcome stratified by treatment, tumor site and tumor size. RESULTS: Among the 327 patients enrolled in the study, 16.21% had tumors located in the suprasellar region and 83.79% in the pineal region. The proportion of males was significantly higher among pineal germinomas (94.16 vs 66.04%; P < .001). Smaller tumors (<24 mm) were more common in the suprasellar region (37.74 vs 18.87%; P < .001). A higher percentage of patients with suprasellar germinomas underwent surgery. Radiotherapy (RT) and chemotherapy (CT) was, respectively, administered to 82.97 and 60.61% of patients during the treatment period, with no significant difference between suprasellar and pineal germinomas. CT plus RT was the most common treatment modality for both pituitary (30.19%) and pineal (33.94%) germinomas. Both RT and CT were associated with improved long-term survival. No survival difference was observed between suprasellar and pineal germinomas. CONCLUSIONS: Despite significant differences in epidemiology and management, pineal and suprasellar germinomas had a similar long-term clinical outcome.

Reduction in the cerebrospinal fluid protein level after bevacizumab treatment in patients with optic pathway low-grade gliomas.

Optic pathway gliomas (OPG) can cause elevated cerebrospinal fluid (CSF) protein concentrations. We report on two patients with suprasellar low-grade gliomas and high CSF protein levels (590 and 551 mg/dl) that precluded shunt implantation. After two and three doses of bevacizumab, respectively, the levels dropped dramatically to 191 and 178 mg/dl, respectively. Bevacizumab treatment was associated with a decrease in CSF protein level, allowing successful shunt placement. Our results are consistent with the pharmacological mechanism of bevacizumab, which decreases protein leakage from blood vessels to the ventricles.

Failure of crizotinib based systemic treatment in ALK positive histiocytosis involving the central nervous system: a case report and literature review.

BACKGROUND: Among the histiocytic disorders, anaplastic lymphoma kinase (ALK)-positive histiocytosis emerged in 2008. As more and more cases of the novel entity are reported, our understanding of it is deepened. However, only a few cases with central nervous system (CNS) involvement have been reported. Furthermore, the lesion in the suprasellar region has not been documented.  CASE PRESENTATION: We presented a case of ALK-positive histiocytosis involving the suprasellar region of a one-year-and-four-month-old boy. Through clinical, neuropathological, and genomic analyses, the patient was diagnosed with ALK-positive histiocytosis. After lesions were resected he started treatment with a combination of the three compounds vincristine, prednisolone, and crizotinib, but they did not work. Cytarabine was then added as an additional chemotherapy drug for him, and the lesions in the brain and lungs were shrunk by combining treatment of crizotinib, dexamethasone, vincristine, and cytarabine according to the RECIST (esponse Evaluation Criteria In Solid Tumours). CONCLUSIONS: Additional adjuvant chemotherapy drugs are needed when ALK-inhibitor treatment is ineffective.

Risk factors for postresection shunting in children with suprasellar tumor: a retrospective analysis of 124 patients.

OBJECTIVE: Hydrocephalus is one of the most significant comorbidities of pediatric suprasellar tumors. Up to 37.5-68.0% of patients were diagnosed with hydrocephalus at admission. However, after surgical resection of the tumor, 9.3-51.4% of the hydrocephalus will persist and require a ventriculoperitoneal shunt (VPS) surgery. The purpose of this study was to identify the risk factors associated with postresection shunting in children with suprasellar tumors. METHODS: We conducted a retrospective analysis of children who underwent surgery for suprasellar tumors at our department from February 2011 to December 2020. We used univariate and multivariate analysis to screen the factors that might be correlated with postoperative shunt placement, taking into account patients' characteristics, tumor histology/size/calcification, the severity of preoperative hydrocephalus, the involvement of ventricles, external ventricular drainage (EVD) placement, postoperative intraventricular hematoma, the extent of resection, and other surgical details. RESULTS: A total of 124 children who underwent surgery for suprasellar tumors were included in our study. Hydrocephalus was present in 55 patients (44.3%) at admission; 23 patients (18.5%) received VPS implantation after tumor removal. Univariate analysis showed that the involvement of ventricles (p = 0.002), moderate/severe preoperative hydrocephalus (p = 0.001), postoperative intraventricular hematoma (p = 0.005), and EVD implantation (p = 0.001) were significantly associated with postoperative VPS. Multivariate analysis confirmed that only ventricle involvement (p = 0.002; OR = 5.6; 95%CI 1.8-17.2) and intraventricular hematoma (p = 0.01; OR = 10.7; 95%CI 1.8-64.2) were independent risk factors for postresection shunting. CONCLUSION: Ventricle involvement and intraventricular hematoma can be identified as independent predictors for postoperative shunting in pediatric suprasellar tumors.