Pediatric craniopharyngioma with significantly increased intraoperative visual evoked potential amplitude and postoperative visual acuity improvement: a case report.

Visual evoked potential (VEP) is an established modality that allows safe brain tumor resection and preservation of optical function. We herein present a case of a pediatric craniopharyngioma with significant improvement in the VEP amplitude detected during endoscopic transsphenoidal surgery (ETS) and obvious postoperative improvement in visual acuity. A 13-year-old boy presented with visual acuity disturbance in his right eye and was followed up for 5 months by an ophthalmologist. His visual acuity rapidly worsened, and a suprasellar lesion with calcification was found on brain computed tomography. The patient underwent tumor resection during ETS with intraoperative transcranial VEP monitoring. Gross total tumor resection was achieved without injury to the perforators, including the superior hypophyseal arteries. The VEP amplitude was unstable, and significant waves were not detectable before tumor resection; however, a positive wave was detected after removing most of the tumor and exposing the bilateral optic nerves and optic chiasm. Subsequently, negative and positive VEP waves were continuously detected. Visual acuity improved remarkably on postoperative day 10. This case demonstrated both a significant increase in the intraoperative VEP amplitude and rapid postoperative improvement in visual acuity. We surmised that the preoperative rapid worsening of visual dysfunction, intraoperative increase in the VEP amplitude, and significant postoperative improvement in visual acuity were associated with the compression of the optic nerves by the internal carotid artery, anterior cerebral artery, and tumor.

Reconsideration of Surgical Indication for Prolactin-producing Pituitary Tumor Focusing on Visual Impairment.

Prolactin-producing pituitary tumor (PRLoma) is the most prevalent functional pituitary tumor. If the tumor becomes large, vision can be impaired. In contrast to other pituitary tumors, cabergoline (CAB) is extremely effective for PRLoma and has become the first-line treatment. In this study, we examined our experience with the pharmacological and surgical management of PRLomas with visual impairment (VI) to determine whether VI could be a surgical indication. Further, we discussed the function of surgery in situations where the gold standard of PRLoma treatment was CAB administration. Of the 159 patients with PRLomas (age, 13-77 [mean = 36.3] years; men, 29; women, 130) at Tokyo Women's Medical University Hospital from 2009 to 2021, 18 (age, 15-67 [mean = 35.8] years; men, 12; woman, 6) had VI (subjectively, 12; objectively, 6). They started CAB treatment immediately (maximum dose: 0.5 to 6 mg/week; average: 2.17 mg/week). VI improved in 16 patients (88.9%) but did not improve in 2 (11.1%) requiring surgeries. One of the two patients had a parenchymal tumor resistant to CAB, and the other had a cystic tumor due to intratumoral bleeding. Consequently, CAB is the first-line treatment for PRLomas with VI because of its significantly high rate of improvement. However, close and rigorous surveillance is necessary for cases resistant to CAB, and the correct decision is required regarding surgical interventions at proper timing and appropriate surgical approaches considering the purpose of surgery.

Endoscope-assisted Trans-lamina Terminalis Resection of Chordoid Glioma at the Third Ventricle: A Case Report.

As per the 2021 World Health Organization (WHO) Classification of Tumors of the Central Nervous System, chordoid glioma (CG) is defined as a slow-growing glial neoplasm categorized as grade II tumor. This tumor is primarily located in the anterior part of the third ventricle, often adheres to important surrounding structures, and is hemorrhagic in nature. Therefore, dissecting this tumor is extremely difficult. In this study, we present the case of a 44-year-old man who initially complained of mild headache and was diagnosed with a homogeneous gadolinium-enhanced lesion in the third ventricle via magnetic resonance imaging. The pathological diagnosis based on his biopsy at the previous hospital was CG. The patient demonstrated no neurological deficit at that time, but the tumor had gradually grown, hydrocephalus appeared 2 years after the tumor was detected, and the patient developed short memory disorder and daytime sleepiness. We resected the tumor via the anterior interhemispheric trans-lamina terminalis approach using a microscope and an endoscope. The residual tumor at the blind spot of the microscopic view was resected under an angled rigid endoscopic view using dedicated tools for transsphenoidal surgery. The tumor was grossly resected, and the histopathological diagnosis was CG. Postoperative neurological findings included slight memory disorder and hypothalamic adrenal dysfunction. No tumor recurrence was reported 3 years post resection. The endoscope-assisted anterior interhemispheric trans-lamina terminalis approach was determined useful for CG resection with minimal surgical complications and without tumor recurrence.

Effect of GH Deficiency Caused by Nonfunctioning Pituitary Masses on Serum C-reactive Protein Levels.

Context: GH supplementation for GH deficiency (GHD) has been reported to decrease high-sensitivity C-reactive protein (hs-CRP), an inflammatory marker; however, the association between GHD and hs-CRP remains unclear. Objective: We aimed to clarify the impact of impaired GH secretion due to pituitary masses on hs-CRP levels. Methods: We retrospectively examined the association between GH secretion, assessed using GH-releasing peptide-2, and serum hs-CRP levels before and a year after the pituitary surgery in patients with nonfunctioning pituitary neuroendocrine tumor or Rathke cleft cyst. Results: Among 171 patients, 55 (32%) presented with severe GHD (peak GH response to GH-releasing peptide-2 < 9 ng/mL). Serum hs-CRP levels were significantly higher in patients with severe GHD than in those without (P < .001) and significantly correlated with the peak GH (r = -0.50, P < .001). Multiple regression analyses showed that the peak GH significantly and negatively predicted hs-CRP levels (ß = -0.345; 95% CI, -0.533 to -0.158) and the lowest quartile of the peak GH (<5.04 ng/mL) were significantly associated with increase in hs-CRP levels (exp [ß] = 1.840; 95% CI, 1.209 to 2.801), after controlling for other anterior hormones and metabolic parameters. Postoperative change in the peak GH (N = 60) significantly predicted change in hs-CRP levels (ß = -0.391; 95% CI, -0.675 to -0.108), independent of alterations in other anterior hormones and metabolic parameters. Conclusion: The inverse association between GH secretion and hs-CRP levels highlights the protective role of GH in the increase in hs-CRP.

[Endoscopic Transsphenoidal Surgery: Basic Technique].

Transsphenoidal surgery(TSS), a revolutionary approach for treating pituitary lesions, was developed at the beginning of the 20th century. However, it has been disused owing to its high complication and fatality rates. In the 1960s, Hardy et al. introduced microscopy into TSS and established its safety, making it a standard procedure that has spread worldwide. In the 1990s, endoscopes were introduced, and further advances were made. In other words, advances in optical instruments have significantly contributed to the development of TSS. Endoscopic TSS(eTSS)has made significant strides since the introduction of high-definition endoscopes in the 2010s. This report outlines the advantages and disadvantages of eTSS and its basic techniques.

Clinical Features of Pituitary or Parasellar Tumor Onset with Cranial Nerve Palsy: Surgical Intervention Considerations.

OBJECTIVE: This study aimed to clarify the symptoms of pituitary or parasellar tumor onset with cranial nerve palsy (CNP) and to improve our knowledge of this rare symptom and its most appropriate treatment. METHODS: Among 1281 patients with pituitary or parasellar tumors surgically treated from 2003 to 2020, 30 cases (2.34%; 15 men and 15 women; mean age: 55.6 years, range: 6-83 years) first presenting with CNP were reviewed to evaluate the neurological symptoms, histological diagnosis, interval from onset to surgery, and time before complete CNP recovery. RESULTS: Pathological diagnoses comprised 17 pituitary adenomas, including 10 pituitary apoplexies and 4 adrenocorticotropic hormone-positive adenomas, and 13 other tumors, including 3 chordomas, 2 xanthogranulomas, 2 malignant lymphomas, 2 metastatic tumors, 1 Rathke cleft cyst, 1 plasmacytoma, 1 craniopharyngioma, and 1 neuroendocrine carcinoma. The mechanisms causing CNP were pituitary apoplexy (n = 10), cranial nerve compression or involvement (n = 17), and inflammatory changes (n = 9). As the first manifestation, 20 (66.7%) patients presented with oculomotor nerve palsy, 2 (6.7%) with trochlear nerve palsy, and 13 (43.3%) with abducens nerve palsy. Full recovery of CNP was obtained in 25 patients (83.3%) after surgery alone and in 2 patients (6.7%) after adjuvant therapy. Early surgery provided no significant difference in full recovery rates although it reduced the time to reach full recovery. CONCLUSIONS: It is critical to determine the mechanisms of CNP and intervene surgically to improve symptoms, shorten the duration of the disorder, prevent relapses, and obtain the correct pathological diagnosis to select the proper adjuvant therapy.

Pituicytoma with pleomorphism: A case report with cytological findings.

Pituicytoma is a rare neoplasm, arising in the posterior pituitary or in the hypophyseal stalk, and its cytological findings have not yet been well-described. We have experienced a case of pituicytoma, which was difficult to diagnose intraoperatively, because of its cellular pleomorphism. A tumor measuring 18 mm in maximum diameter was found at the sella turcica in a Japanese woman in her forties. Both intraoperative crush cytology and histology of the resected tumor showed pleomorphic spindle or round cells, including multinucleated cells. Tumor cells were positive for TTF-1, S-100 protein, and vimentin, partially positive for glial fibrillary acidic protein and epithelial membrane antigen, and negative for synaptophysin, hormones of the anterior pituitary gland, CD34, Olig2, PAX8, and napsin A. Ki-67 labeling index was 2.0%. Tumors included in the differential diagnosis in general are pituitary adenoma, craniopharyngioma, germinoma, and metastatic tumor on the radiological standpoint, and pilocytic astrocytoma and meningioma on the cytological standpoint. However, our case was difficult to differentiate especially from high-grade glioma only by morphology, and immunohistochemistry including TTF-1 was helpful.

Clinical features and difficulty in diagnosis of Langerhans cell histiocytosis in the hypothalamic-pituitary region.

Langerhans cell histiocytosis (LCH) is a multi-organ disorder that rarely involves the hypothalamic-pituitary region (HPR). HPR-LCH presents with severe progressive pituitary dysfunction and its prognosis is poor. The definitive diagnosis of LCH is considerably difficult and complicated owing to the occurrence of several diseases with similar manifestations in the HPR and its location in the deepest portion of the anterior skull base, in close proximity to important normal structures, severely limiting the size of the biopsy specimen. Chemotherapy is the established treatment modality for LCH; hence, timely and accurate diagnosis of LCH is essential for early therapeutic intervention. We retrospectively reviewed clinical features and biopsy procedures in four patients with HPR-LCH (all female, 28-44 years old) from 2009 to 2020. Maximum diameter of supra-sellar lesions was 23-35 mm and 2 cases had skip lesions. All patients demonstrated central diabetes insipidus, hyper-prolactinemia, and severe anterior pituitary dysfunction. Two of the patients had progressive disease. Furthermore, four patients presented body weight gain, two visual disturbance, and two impaired consciousness. The duration from onset to diagnosis of LCH was 3 to 10 (average 7.25) years. In total, eight operations were performed until final diagnosis. The percentage of correct diagnosis by biopsy was 50% (4/8). Clinical features of HPR-LCH are very similar to those of other HPR diseases, and their symptoms are progressive and irreversible. Clinicians should consider repeated biopsy with a more aggressive approach if the lesion is refractory to steroid therapy, in order to ensure accurate diagnosis and appropriate treatment.

Metabolic Reprogramming Drives Pituitary Tumor Growth through Epigenetic Regulation of TERT.

Pituitary adenomas are common, benign brain tumors. Some tumors show aggressive phenotypes including early recurrence, local invasion and distant metastasis, but the underlying mechanism to drive the progression of pituitary tumors has remained to be clarified. Aerobic glycolysis known as the Warburg effect is one of the emerging hallmarks of cancer, which has an impact on the tumor biology partly through epigenetic regulation of the tumor-promoting genes. Here, we demonstrate metabolic reprogramming in pituitary tumors contributes to tumor cell growth with epigenetic changes such as histone acetylation. Notably, a shift in histone acetylation increases the expression of telomerase reverse transcriptase (TERT) oncogene, which drives metabolism-dependent cell proliferation in pituitary tumors. These indicate that epigenetic changes could be the specific biomarker for predicting the behavior of pituitary tumors and exploitable as a novel target for the aggressive types of the pituitary tumors.

Outcome After Resection of Craniopharyngiomas and the Important Role of Stereotactic Radiosurgery in Their Management.

OBJECTIVE: Experience with management of craniopharyngiomas (CPH) was evaluated retrospectively. METHODS: Between 1981 and 2012, 100 patients underwent removal of a CPH (the main surgical group), and an original tumor grading system was applied to these cases. The mean length of follow-up was 121 months. Additionally, 17 patients underwent removal of a CPH between 2012 and 2017 (the supplementary surgical group), and in 6 of them, CyberKnife radiosurgery was performed on a residual tumor (in 5 cases) or at the time of recurrence (in 1 case). RESULTS: In the main surgical group, the gross total resection (GTR) rate was 81%. The early and late disease-specific postoperative mortality rates were 0% and 2%, respectively. Tumor recurrence was never noted after GTR. There was a statistically significant increase in the Karnofsky Performance Scale (KPS) score after surgery. The tumor surgical grade was inversely associated with both the pre- and postoperative KPS scores, and was lower in cases operated on via the transnasal transsphenoidal approach, but was unrelated to the GTR rate. In the supplementary surgical group, the GTR rate was 65%. CyberKnife radiosurgery consistently resulted in tumor shrinkage. CONCLUSION: GTR is the preferred management option for CPH. The original surgical grading system developed at Tokyo Women's Medical University may be helpful for clinical decision-making. CyberKnife radiosurgery for residual and recurrent CPH is associated with high tumor response rates.

Gamma Knife Radiosurgery for Pituitary Adenomas Invading the Cavernous Sinus: Tokyo Women's Medical University Experience.

Total surgical removal of a pituitary adenoma (PA) invading the cavernous sinus (CS) is challenging and carries a significant risk of postoperative complications. As an alternative treatment strategy, after incomplete resection, such tumors may undergo stereotactic radiosurgery-in particular, Gamma Knife surgery (GKS). Treatment planning based on advanced neuroimaging (e.g., thin-slice 3-dimensional postcontrast constructive interference in steady state (CISS) images) allows clear visualization of the target microanatomy, which results in highly conformal and selective radiation delivery to the lesion with preservation of adjacent functionally important neurovascular structures. In the Tokyo Women's Medical University experience of GKS for 43 nonfunctioning and 46 hormone-secreting PA invading the CS, with a minimum follow-up period of 5 years (mean 76 months, range 60-118 months), the tumor control rate has reached 97%, and a significant volume reduction (≥50%) has been seen in 24% of lesions. In cases of hormone-secreting neoplasms, normalization (in 18 patients; 39%) or improvement (in 22 patients; 48%) of endocrinological function has been noted. Importantly, such effects have been sufficiently durable. Complications have been extremely rare and limited to transient cranial nerve palsy (in 2% of cases). Notably, no patient in our series has had a new pituitary hormone deficit after irradiation. Thus, subtotal resection followed by GKS may be considered a valuable alternative to aggressive surgery for a PA invading the CS.

Novel Bendable Ring Curette for Endoscopic Transsphenoidal Surgery for Pituitary Tumors.

OBJECTIVE: In transsphenoidal resection of pituitary tumors, even dedicated instruments sometimes cannot readily reach the surgical fields and they interfere with the endoscope and other instruments, making instrumental manipulations difficult and often ineffective. To solve these problems, we invented a bendable ring curette to further improve maneuverability and accessibility. METHODS: The newly developed ring curette has a tip (14-mm long) that can bend spanning a range of 90°. Two types of ring curette are constructed; an upward-bending type, and a downward-bending type. We used the ring curette in 222 endoscopic pituitary surgeries. RESULTS: The ring curette was easily operated with one hand, simply by manipulating the slider located at the handle to bend or straighten the tip. The bendable ring curette permits adequate access to sites where regular dedicated instruments such as the fixed-angle ring curette could not reach easily. The ring curette facilitated tumor resection as long as tumors were not too hard. Furthermore, the curette could be used as a dissector because the long tip provides greater horizontal and vertical forces. CONCLUSIONS: Our newly developed bendable ring curette improves maneuverability and accessibility and can be efficiently used as a dissector in the far lateral and frontal sites, compared with the conventional fixed instrument.

Mucosal thickening of the maxillary sinus is frequently associated with diffuse glioma patients and correlates with poor survival prognosis of GBM patients: comparative analysis to meningioma patients.

Glioma patients were frequently associated with mucosal thickening of the maxillary sinus (MTMS), which reflects mucosal inflammation. We suspected that MTMS is associated with impaired mucosal immune response and correlated with dysfunction in the anti-tumor immune response in diffuse glioma patients. Therefore, the aim of this study was to determine whether the occurrence of diffuse glioma is correlated with MTMS compared to meningioma and control groups. Furthermore, we investigated whether MTMS is associated with overall survival (OS) in glioblastoma (GBM) patients. This study included 343 patients with newly diagnosed diffuse gliomas and 218 patients with meningioma treated at our institution between 2015 and 2018. As control, 201 patients with headache who did not have an intracranial organic lesion were included. Using three-axis MR images, we evaluated the incidence of MTMS in all patients. Additionally, we investigated the relationship between MTMS and OS. The incidence of MTMS in patients with diffuse glioma was significantly higher than that in the meningioma (p < .0001) and control groups (p < .0001). In 128 patients with GBM, MTMS status correlated significantly with OS (p = .0064). We revealed that the incidence of MTMS is significantly associated with patients with diffuse glioma. This suggests that MTMS is indirectly involved in the occurrence of diffuse gliomas. Furthermore, the presence of MTMS correlated significantly with shorter OS in GBM patients, indicating that MTMS is involved in suppression of anti-tumor immune response. Preoperative recognition of MTMS might be useful for improving the clinical management of GBM patients.

Development of a Semiautomatic Dura Mater Suturing Device for Preventing Cerebrospinal Fluid Leakage in Transsphenoidal Surgery.

Dural suturing in transsphenoidal surgery requires well-honed technical skills. We have developed a semiautomatic dural suturing device and confirmed its effectiveness by comparing it with the conventional method. This device significantly shortens the suturing time compared with the conventional method. The dural suturing time in transsphenoidal surgery could be decreased significantly by use of this novel device.

Usefulness of the knot-tightener device following dural suturing in endonasal transsphenoidal surgery: technical report.

Transsphenoidal surgery (TSS) has become a well-established standard surgical technique, but the cerebrospinal fluid leakage remains controversial. Direct suturing of the dura, which is a routine procedure within transcranial surgery, can be applied for closure of the sella turcica within TSS. However, as the dura is not accessible by the index finger, knot tying in the narrow and deep surgical corridor following dural suturing is extremely difficult, cumbersome, and time-consuming in TSS. Here, we present a new, simple, and effective technique for knot tying using our newly developed instrument the "knot tightener" (UC-6603: Medical U & A, Inc., Osaka, Japan) to solve this challenge. The knot tightener has a total length of 235 mm and is bayonet shaped. The tip is 5 × 10 mm in diameter and has one long arm and two short curved arms. The long arm has a dimple which can hook and hold a thread, fulfilling the role of an index finger. Together the two short curved arms make a half circle and are able to hook a thread easily. From the 28th of March 2011 to August 2018, we used the knot-tightener device for 566 patients who underwent endonasal TSS, to deliver and tie knots following stitching of the dura using 6-0 nylon. The device was able to easily deliver a knot from outside of the nostril to the sella turcica through the nasal cavity and successfully tighten it firmly. No complications were observed, confirming the safety of the newly designed instrument. The knot tightener can be considered to be an optimal tool for the challenging surgical procedure of knot tying following dural suturing in TSS. Its potential future applications may extend to include other neurosurgical procedures in anatomically restricted areas.

Application of indocyanine green fluorescence endoscopic system in transsphenoidal surgery for pituitary tumors.

BACKGROUND: For the precise removal of pituitary tumors, preserving the surrounding normal structures, we need real-time intraoperative information on tumor location, margins, and surrounding structures. The aim of this study was to evaluate the benefits of a new intraoperative real-time imaging modality using indocyanine green (ICG) fluorescence through an endoscopic system during transsphenoidal surgery (TSS) for pituitary tumors. METHODS: Between August 2013 and October 2014, 20 patients with pituitary and parasellar region tumors underwent TSS using the ICG fluorescence endoscopic system. We used a peripheral vein bolus dose of 6.25 mg/injection of ICG, started with a time counter, and examined how each tissue type increased and decreased in fluorescence through time. RESULTS: A total of 33 investigations were performed for 20 patients: 9 had growth hormone secreting adenomas, 6 non-functioning pituitary adenomas, 3 Rathke's cleft cysts, 1 meningioma, and 1 pituicytoma. After the injection of ICG, the intensity of fluorescence of tumor and normal tissues under near-infrared light showed clear differences. We could differentiate tumor margins from adjacent normal tissues and define clearly the surrounding normal structures using the different fluorescent intensities time changes and tissue-specific fluorescence patterns. CONCLUSIONS: The ICG endoscopic system is simple, user-friendly, quick, cost-effective, and reliable. The method offered real-time information during TSS to delimit pituitary and parasellar region tumor tissue from surrounding normal structures. This method can contribute to the improvement of total removal rates of tumors, reduction of complications after TSS, saving surgical time, and preserving endocrinological functions.

Placental alkaline phosphatase levels in cerebrospinal fluid can have a decisive role in the differential diagnosis of intracranial germ cell tumors.

OBJECTIVE: Placental alkaline phosphatase (PLAP) in CSF can provide a very high diagnostic value in cases of intracranial germ cell tumors (GCTs), especially in pure germinomas, to the level of not requiring histological confirmation. Unlike other tumor markers, reliable data analysis with respect to the diagnostic value of PLAP serum or CSF levels has not been available until now. This is the first systematic and comprehensive study examining the diagnostic value of CSF PLAP in patients with intracranial GCTs. METHODS: From 2004 to 2014, 74 patients (average age 19.6 ± 10.6 years) with intracranial GCTs were evaluated using PLAP from their CSF and histological samples. Chemiluminescent enzyme immunoassay was utilized to measure CSF PLAP in the following tumor sites: pineal (n = 32), pituitary stalk, suprasellar (n = 16), basal ganglia (n = 15), intraventricular (n = 9), and cerebellar (n = 5) regions. In addition to classifying GCT cases, all patients underwent tumor biopsy for correlation with tumor marker data. RESULTS: PLAP in combination with other tumor markers resulted in extremely high sensitivity and specificity of the diagnostic value of intracranial GCTs. Intracranial GCT cases were classified into 1) germinomas, both "pure" and syncytiotrophoblastic giant cell types (n = 38); 2) nongerminomatous GCTs, choriocarcinomas (n = 9) and teratomas (n = 4); and 3) nongerminomas, other kinds of tumors (n = 23). Consequently, all patients received chemoradiation therapy based on elevation of PLAP and the histopathological results. It was also speculated that the level of PLAP could show the amount of intracranial germ cell components of a GCT. PLAP was 100% upregulated in all intracranial germinoma cases. The absence of CSF PLAP proved that the tumor was not a germinoma. CONCLUSIONS: The current study is the first systematic and comprehensive examination of the diagnostic value of the tumor marker PLAP in pediatric patients with intracranial GCT. Using the level of PLAP in CSF, we were able to detect the instances of intracranial germinoma with very high reliability, equivalent to a pathological diagnosis.

A Rare Case of Malignant Craniopharyngioma Reactive to Adjunctive Stereotactic Radiotherapy and Chemotherapy: Case Report and Literature Review.

BACKGROUND: Malignant craniopharyngioma or anaplastic craniopharyngioma was first reported in 1987 by Akachi and colleagues. It has malignant clinical and histological features, remarkably rapid progression, atypical pathology like squamous cell carcinoma, and a poor prognosis. To date, 17 cases of malignant craniopharyngioma have been reported, most of which were secondary malignant tumors. In classic benign craniopharyngioma, adjunctive treatment after gross total removal is not necessary, but in cases of malignant tumors, adjunctive treatment is important. CASE DESCRIPTION: Here we report the first case of malignant craniopharyngioma treated with adjunctive gamma knife stereotactic radiosurgery and chemotherapy (carboplatin and etoposide, as well as temozolomide chemotherapy). Treatment effectively controlled progression of the tumor temporarily. CONCLUSIONS: Adjunctive gamma knife stereotactic radiosurgery and chemotherapy for malignant craniopharyngioma affects follow-up strategies, we propose the need for a revision to the World Health Organization classification regarding the evaluation of malignant craniopharyngioma.

Pediatric Optic Pathway/Hypothalamic Glioma.

Optic pathway/hypothalamic gliomas (OP/HGs) are rare astrocytic tumors that appear more commonly among young children and often are unresectable. They comprise approximately 2% of all central nervous system tumors and account for 3-5% of pediatric intracranial tumors. Initial manifestations are often visual disturbances, endocrinopathies and hypothalamic dysfunction such as the diencephalic syndrome, and sometimes hydrocephalus due to cerebrospinal fluid (CSF) outflow obstruction. In many cases, the tumors are diagnosed late in the clinical course because they silently enlarge. These tumors consist mostly of histologically benign, World Health Organization (WHO) grade I tumors represented by pilocytic astrocytomas (PA), the rest being pilomyxoid astrocytomas (PXA) - WHO grade II tumors. In young pediatric patients, however, can be seen PXA that show aggressive clinical course such as CSF dissemination. Our small series of 14 non-Neurofibromatosis type 1 (NF-1) OP/HGs PA patients underwent extended resection without any adjuvant treatments. The median age at initial treatment was 11.5 ± 6.90 years (range, 1-25 years) and median follow up 85.5 ± 25.0 months. Surgical resection for OP/HGs results in acceptable middle-term survival, tumor control and functional outcome equivalent to chemotherapy. There is, however, no longer doubt that chemotherapy with or without biopsy and as-needed debulking surgery remains the golden standard in management of OP/H. Clinical conditions and treatment plans for OP/HGs vary depending on their structure of origin.

The role of 4D CT angiography for preoperative screening in patients with intracranial tumors.

INTRODUCTION: Four-dimensional computed tomography angiography (4D CTA) is now becoming an often used diagnostic imaging modality for the assessment of patients with intracranial tumors. The purpose of this study was to demonstrate the utility of 4D CTA for preoperative screening in patients with intracranial tumors as well as to examine the correlation between perfusion data and grading of gliomas. METHODS: We performed preoperative screening using 320-row detector CT scanner in 186 patients with intracranial tumors, and 115 patients were finally included in the study. Time-resolved subtracted maximum intensity projection images and volume-rendered images were reconstructed to evaluate vascular structures, tumor staining and incidental lesions. We also evaluated the perfusion functional map for gliomas to find correlations between their vascularity and tumor grading. RESULTS: We evaluated gliomas in 70 patients, meningiomas in 29, and other tumors in 16. Patients with gliomas of a pathologically higher grade showed high cerebral blood volume (p < 0.05). Tumor staining was observed on MIP images for 12 meningiomas, 4 gliomas, and 3 other tumors. Fifty patients showed a mass effect on vascular structures, seven had cerebral venous sinus obstruction, and two had aneurysms. No iatrogenic accidents with the CT procedure were reported. CONCLUSIONS: The 4D CTA technique is effective and safe for depicting vascular structures such as arteries, veins, tumor-related vessels, and direct and indirect anatomical complications such as vascular obstruction or vascular compression. This information is useful for preoperative screening. Although 4D CTA also provides perfusion data correlating with the tumor vascularity and grading of gliomas, its clinical value remains limited.