Endoscopic Endonasal Transsphenoidal Surgery for Intrasellar Mixed Germ Cell Tumors.

A mixed germ cell tumor (MGCT) in the neurohypophysis is very rare, with only a few reported cases1-4 but none with surgical videos. In this report, the endoscopic endonasal transsphenoidal approach for MGCT in the neurohypophysis is presented (Video 1). A 12-year-old girl with ocular pain, fatigue, and nausea presented with gradual onset of quadrant hemianopsia and left oculomotor palsy. Magnetic resonance imaging showed an enhanced mass in the sella turcica with multiple components involving the pituitary gland and stalk. Her endocrinological examination showed decreased levels of pituitary hormones and simultaneously elevated serum levels of alpha-fetoprotein and beta-human chorionic gonadotropin. After pituitary hormone replacement, endoscopic endonasal transsphenoidal surgery was planned. The tumor was strongly adherent to the surrounding structures, and gross total resection was achieved. The histological diagnosis was MGCT with a teratoma component. Postoperatively, her vision and oculomotor palsy improved swiftly, and adjuvant chemotherapy and radiotherapy were administered. In this case, 3-dimensional computer graphics were created from the preoperative computed tomography and magnetic resonance imaging studies. Preoperative simulation with the 3-dimensional computer graphic images and intraoperative verification with indocyanine green images facilitated our understanding of the surrounding anatomy, including the tumor components, pituitary gland, and internal carotid arteries.5 After removal of the tumor, multilayer fascial closure was performed for skull base reconstruction.6 MGCT in the neurohypophysis can be strongly adherent to the surrounding structures, requiring careful dissection and resection under endoscopy. At the last follow-up (8 months after surgery), the tumor was successfully controlled, and the patient had no neurological symptoms with pituitary hormone replacement therapy.

Incidence of and risk factors for chronic subdural hematoma after endoscopic endonasal surgery: quantitative analysis of pneumocephalus.

OBJECTIVE: Chronic subdural hematoma (CSDH) is known to occur after endoscopic endonasal surgery (EES); however, the detailed clinical picture remains unclear. In this study, the authors aimed to examine the incidence of and risk factors for post-EES CSDH, with a focus on the quantitative evaluation of postoperative pneumocephalus. METHODS: The authors retrospectively collected data on consecutive patients who, between November 2016 and December 2022, had undergone EES during which intraoperative cerebrospinal fluid (CSF) leakage occurred. Using CT images obtained immediately after surgery (CT0), the authors measured the extent of pneumocephalus in detail. The locations of pneumocephalus were divided into two groups: remote and local. Remote pneumocephalus was further subdivided into convexity and ventricular. The incidence of post-EES CSDH was calculated, and its risk factors were analyzed. RESULTS: Among the 159 EES patients included in the study, Esposito grade 1, 2, and 3 intraoperative CSF leakage was confirmed in 22 (14%), 27 (17%), and 110 (69%) patients, respectively. CSDH occurred in 6 patients (3.8%). One patient (0.6%) required unilateral burr hole surgery, whereas the hematomas spontaneously disappeared in the others. All CSDHs occurred in patients with Esposito grade 3 CSF leakage and convexity pneumocephalus on CT0. In the multivariate analysis of 149 sides with convexity pneumocephalus on CT0, the product of the diameter and the thickness of convexity pneumocephalus on CT0 was significantly associated with subsequent CSDH (OR 1.21, 95% CI 1.06-1.38, p = 0.004). Using a cutoff value of 10 cm2, CSDH development could be predicted with a sensitivity of 0.82 and specificity of 0.74. CONCLUSIONS: The incidence of post-EES CSDH is acceptably low, and surgery is rarely required. Patients with extensive convexity pneumocephalus on immediate postoperative CT are prone to develop CSDH and thus should be carefully monitored.

Role of stereotactic radiosurgery for recurrent skull base acinic cell carcinoma: illustrative case.

BACKGROUND: Acinic cell carcinomas (AcCCs), rare malignancies of the salivary glands, often recur and metastasize, particularly in the skull base. Conventional radical resection can be invasive for skull base AcCCs adjacent to cranial nerves and major vasculature, and the effectiveness of stereotactic radiosurgery (SRS) as an alternative is not well established. OBSERVATIONS: This case report details the application of SRS for recurrent skull base AcCCs. A 71-year-old male with a history of resection for a right mandibular AcCC 23 years earlier experienced tumor recurrence involving the right cavernous sinus and nasal cavity. He underwent endoscopic transnasal surgery followed by SRS targeting different tumor locations-the cavernous sinus to the pterygopalatine fossa, maxillary sinus, and clivus-each with a prescribed dose of 20 Gy to the 40% to 50% isodose line. After the first skull base metastasis, additional sessions of localized SRS after endoscopic surgery led to a 12-year survival without sequela. LESSONS: This is a report indicating that SRS for skull base AcCCs can achieve favorable local control, functional preservation, and long-term survival. SRS may be suitable for skull base AcCC given the lesion's tendency toward multiple local recurrences. Further investigation is needed to validate the treatment's efficacy.

POLR2A Mutation is a Poor Prognostic Marker of Cerebellopontine Angle Meningioma.

BACKGROUND AND OBJECTIVES: Recent molecular analyses have shown that the driver genetic mutations of meningiomas were associated with the anatomic location. Among these, POLR2A mutation is common among lesions in the skull base, mainly in the cerebellopontine angle (CPA). The objective of this study was to investigate the efficacy of POLR2A mutation as a prognostic marker for CPA meningiomas. METHODS: We retrospectively analyzed the clinical data of 70 patients who had World Health Organization grade I CPA meningiomas. Somatic DNA was analyzed by Sanger sequencing and microsatellite array to examine for NF2, AKT1, KLF4, SMO, and POLR2A mutations and 22q loss. Genetic and clinical parameters were analyzed to identify the factors related with tumor recurrence. RESULTS: We detected clearly the clinical features of the CPA cases with POLR2A mutation. Compared with cases without POLR2A mutation, cases with POLR2A mutation had more meningothelial type (P = 6.9 × 10-4), and higher rate of recurrence (P = .04). We found that the poor prognostic factors associated with the recurrence of CPA meningiomas were POLR2A mutation (P = .03, hazard ratio [HR] 9.38, 95% CI 1.26-70.0) and subtotal resection (STR) (P = 5.1 × 10-4, HR 63.1, 95% CI 6.09-655.0). In addition, in the group that underwent STR, POLR2A mutation was a poor prognostic factor associated with tumor recurrence (P = .03, HR 11.1, 95% CI 1.19-103.7). CONCLUSION: POLR2A mutation and STR were the poor prognostic markers associated with the recurrence of CPA meningioma. For CPA meningioma cases that underwent STR, only POLR2A mutation was a poor prognostic factor. Detecting POLR2A mutation may be a cost-effective, easy, and useful marker for prognostication.

Rise of oligodendroglioma hypermutator phenotype from a subclone harboring TP53 mutation after TMZ treatment.

Oligodendrogliomas characterized and defined by 1p/19q co-deletion are slowly growing tumors showing better prognosis than astrocytomas. TP53 mutation is rare in oligodendrogliomas while the vast majority of astrocytomas harbor the mutation, making TP53 mutation mutually exclusive with 1p/19q codeletion in lower grade gliomas virtually. We report a case of 51-year-old woman with a left fronto-temporal oligodendroglioma that contained a small portion with a TP53 mutation, R248Q, at the initial surgery. On a first, slow-growing recurrence 29 months after radiation and nitrosourea-based chemotherapy, the patient underwent TMZ chemotherapy. The recurrent tumor responded well to TMZ but developed a rapid progression after 6 cycles as a malignant hypermutator tumor with a MSH6 mutation. Most of the recurrent tumor lacked typical oligodendroglioma morphology that was observed in the primary tumor, while it retained the IDH1 mutation and 1p/19q co-deletion. The identical TP53 mutation observed in the small portion of the primary tumor was universal in the recurrence. This case embodied the theoretically understandable clonal expansion of the TP53 mutation with additional mismatch repair gene dysfunction leading to hypermutator phenotype. It thus indicated that TP53 mutation in oligodendroglioma, although not common, may play a critical role in the development of hypermutator after TMZ treatment.

Custom shaping of distal access catheter for navigation of microcatheter into inferolateral and meningohypophyseal trunk feeders.

OBJECTIVE: Navigating a microcatheter to tiny feeding arteries such as the inferolateral trunk (ILT) and meningohypophyseal trunk (MHT) of the internal carotid artery (ICA) is technically challenging because of the anatomical features of steep angulation, small diameter, and significant caliber difference from the ICA. To guide the microcatheter to these ICA side branch feeders, the authors thought that a custom shaping of the intermediate catheter would be helpful to determine the direction of the microcatheter and strongly back it up. The aim of this study was to report the detailed methodology and clinical outcomes of patients who had undergone the embolization of ILT and MHT feeders using this method. METHODS: This was a retrospective study of consecutive patients with intracranial tumors and dural arteriovenous fistulas (DAVFs) who had undergone endovascular embolization. The details of the embolization procedures were examined, especially the technique of guiding a low-profile microcatheter supported by the thermoformed intermediate catheter. The authors assessed the patient background, procedural outcomes, and postoperative complications. RESULTS: Between October 2013 and June 2022, 43 patients with intracranial tumors and 45 with DAVFs underwent embolization at the authors' institutions. Among these cases, embolization of the ICA side branch feeder was attempted in 10 intracranial tumors and 2 DAVFs. Of these 12 patients, the microcatheter was guided deep enough for successful embolization in 11 cases (92%). Two asymptomatic arteriovenous fistulas were complicated during guidewire manipulation deep in the feeder. Yet, both disappeared completely after glue injection into the feeding artery. No spasm or dissection of the parent artery occurred during the procedure. Postoperative radiological evaluation showed no intracranial hematoma or symptomatic cerebral infarction. One patient (9%) developed permanent postoperative cranial neuropathy that gradually improved after embolization. CONCLUSIONS: This study revealed that custom shaping of distal access catheters is an effective technique that enabled the embolization of ILT and MHT feeders with a success rate of more than 90%.

Long-term outcomes of stereotactic radiosurgery for intracranial schwannoma in neurofibromatosis type 2: a genetic analysis perspective.

PURPOSE: Neurofibromatosis type 2 (NF2) is intractable because of multiple tumors involving the nervous system and is clinically diverse and genotype-dependent. Stereotactic radiosurgery (SRS) for NF2-associated schwannomas remains controversial. We aimed to investigate the association between radiosurgical outcomes and mutation types in NF2-associated schwannomas. METHODS: This single-institute retrospective study included consecutive NF2 patients with intracranial schwannomas treated with SRS. The patients' types of germline mutations ("Truncating," "Large deletion," "Splice site," "Missense," and "Mosaic") and Halliday's genetic severity scores were examined, and the associations with progression-free rate (PFR) and overall survival (OS) were analyzed. RESULTS: The study enrolled 14 patients with NF2 with 22 associated intracranial schwannomas (median follow-up, 102 months).　The PFRs in the entire cohort were 95% at 5 years and 90% at 10-20 years. The PFRs tended to be worse in patients with truncating mutation exons 2-13 than in those with other mutation types (91% at 5 years and 82% at 10-20 years vs. 100% at 10-20 years, P = 0.140). The OSs were 89% for patients aged 40 years and 74% for those aged 60 years in the entire cohort and significantly lower in genetic severity group 3 than in the other groups (100% vs. 50% for those aged 35 years; P = 0.016). CONCLUSION: SRS achieved excellent PFR for NF2-associated intracranial schwannomas in the mild (group 2A) and moderate (group 2B) groups. SRS necessitates careful consideration for the severe group (group 3), especially in cases with NF2 truncating mutation exons 2-13.

Generation and application of endogenously floxed alleles for cell-specific knockout in zebrafish.

The zebrafish is amenable to a variety of genetic approaches. However, lack of conditional deletion alleles limits stage- or cell-specific gene knockout. Here, we applied an existing protocol to establish a floxed allele for gata2a but failed to do so due to off-target integration and incomplete knockin. To address these problems, we applied simultaneous co-targeting with Cas12a to insert loxP sites in cis, together with transgenic counterscreening and comprehensive molecular analysis, to identify off-target insertions and confirm targeted knockins. We subsequently used our approach to establish endogenously floxed alleles of foxc1a, rasa1a, and ruvbl1, each in a single generation. We demonstrate the utility of these alleles by verifying Cre-dependent deletion, which yielded expected phenotypes in each case. Finally, we used the floxed gata2a allele to demonstrate an endothelial autonomous requirement in lymphatic valve development. Together, our results provide a framework for routine generation and application of endogenously floxed alleles in zebrafish.

[Surgical Strategy for Skull Base Chordoma:Recent Advent of Neuroendoscopic Surgery].

Skull base chordoma is a rare bone tumor that is predominantly found in the clival area and considered to originate from the notochordal remnants. Chordoma is characterized by an aggressive nature and has a high risk of repeated recurrence despite multimodal treatments, including extensive surgical resection and high-dose radiotherapy. Thus, extensive surgical resection of the tumor and adjacent bony structures is strongly recommended. However, surgery was difficult because of the deep location of the lesion and involvement of important anatomies such as the cranial nerves and internal carotid arteries. Recent advent of endoscopic technology has changed visibility of the surgical field and accessibility, and surgical outcomes of this intractable tumor have dramatically changed. In this article, we present our surgical strategy of skull base chordoma aiming for radical surgical resection, focusing on the neuroendoscopic skull base surgery.

White epidermoid cyst transformation after stereotactic radiosurgery: illustrative case.

BACKGROUND: White epidermoid cysts (WECs) are a rare type of epidermoid cyst with atypical radiological features. The epidemiological aspects and mechanisms of their onset remain unknown. Herein, the authors report a unique case of WEC transformation from a typical epidermoid cyst after stereotactic radiosurgery (SRS), confirmed by radiological and pathological findings. OBSERVATIONS: The case involved a 78-year-old man with a history of 2 surgeries for a left cerebellopontine angle typical epidermoid cyst 23 years earlier and SRS using the CyberKnife for recurrent trigeminal neuralgia (TN) 14 years earlier. The tumor with high intensity on T1-weighted imaging, low intensity on T2-weighted imaging, without restriction on diffusion-weighted imaging had gradually enlarged after SRS. Therefore, a salvage surgery was performed via a left suboccipital craniotomy, and the intraoperative findings showed a cyst with a brown, viscous liquid component, consistent with those of WECs. Histopathologically, keratin calcification and hemorrhage were identified, leading to a diagnosis of WEC. The postoperative course was uneventful, and the TN resolved. No tumor recurrence was recorded at 2 years postoperatively. LESSONS: To the best of the authors' knowledge, this is the first world case of WEC transformation from a typical epidermoid cyst after SRS, confirmed by radiological and pathological findings. Radiation effects could have been involved in this transformation.

Long-Term Outcomes of Stereotactic Radiosurgery for Postoperative World Health Organization Grade I Skull Base Meningioma: Utility of Ki-67 Labeling Index as a Prognostic Indicator.

BACKGROUND: Gross total resection, without causing neurological deficits, is challenging in skull base meningioma (SBM). Therefore, stereotactic radiosurgery (SRS) is an important approach for SBMs; however, it is difficult to predict the long-term prognosis. OBJECTIVE: To identify the predictive factors for tumor progression after SRS for World Health Organization (WHO) grade I SBMs, focusing on the Ki-67 labeling index (LI). METHODS: In this single-center retrospective study, factors affecting progression-free survival rates (PFSs) and neurological outcomes in patients undergoing SRS for postoperative SBMs were evaluated. Based on the Ki-67 LI, patients were classified into 3 groups: low (<4%), intermediate (4%-6%), and high LI (>6%). RESULTS: In the 112 patients enrolled, the cumulative 5- and 10-year PFSs were 93% and 83%, respectively. The PFSs were significantly higher in the low LI group (95% at 10 years) compared with the other groups (intermediate LI, 60% at 10 years, P = .007; high LI, 20% at 10 years, P = .001). Multivariable Cox proportional hazard analysis demonstrated that the Ki-67 LI was significantly associated with the PFSs (low vs intermediate LI; hazard ratio, 6.00; 95% CI, 1.41-25.54; P = .015; low vs high LI; hazard ratio, 31.90; 95% CI, 5.59-181.77; P = .001). CONCLUSION: Ki-67 LI may be a useful predictor of long-term prognosis in SRS for postoperative WHO grade I SBM. SRS provides excellent long- and mid-term PFSs in SBMs with Ki-67 LIs <4% or 4% to 6%, with a low risk of radiation-induced adverse events.

Endoscopic Extended Transsphenoidal Surgery Aiming for Radical Resection of Skull Base Tumors Involving Cavernous Sinus: Assessment of Resectability and Risks of Complications.

BACKGROUND AND OBJECTIVES: Surgical resection of tumors invading the cavernous sinus (CS) still shows therapeutic challenges. For "nonadenomatous" skull base tumors invading in CS, there were only a few reports showing the outcomes of radical resection. Therefore, the outcomes of endoscopic transsphenoidal surgery (ETS) aiming for radical resection thus remain largely unknown regarding resectability and functional results of the cranial nerves. METHODS: We performed ETS aiming for radical resection in 35 skull base tumors involving CS (17 chondrosarcomas, 12 chordomas, 3 meningiomas, and 3 trigeminal schwannomas; median follow-up 36.5 months ranging from 12 to 91 months). Gross total resection (GTR) is attempted in all the cases for real-time findings from electrophysiological monitoring of the cranial nerves. When the tumor was strongly adherent to the cranial nerves or internal carotid artery, maximum volume reduction of the tumor was attempted. RESULTS: GTR was achieved in 28 patients (80.0%), subtotal resection in 3 (8.6%), and partial resection in 4 (11.4%). One patient experienced internal carotid artery injury during surgery. After ETS, 15 patients showed symptom improvement (51.7% in all 29 patients with preoperative cranial nerve symptoms, CNS). Four (11.4%) transiently developed abducens nerve palsy, and one required repair surgery for cerebrospinal leakage. In univariate analyses, extension to the lateral compartment of CS ( P = .04) was significantly associated with reduced achievement of GTR. Previous transcranial surgery was associated with reduced possibility of improvement and worsening in CNS. Eleven patients underwent stereotactic radiosurgery, at a median of 12 months after ETS. 32 patients (91.4%) did not show recurrence at the final follow-up. CONCLUSION: ETS can achieve sufficient surgical resection in most of the patients, with acceptable neurological complications. For patients with CNS, ETS may offer the opportunity for improving CNS. We should also always prioritize avoidance of critical situations by preventing internal carotid artery injury.

Efficacy of a Novel Augmented Reality Navigation System Using 3D Computer Graphic Modeling in Endoscopic Transsphenoidal Surgery for Sellar and Parasellar Tumors.

In endoscopic transsphenoidal skull base surgery, knowledge of tumor location on imaging and the anatomic structures is required simultaneously. However, it is often difficult to accurately reconstruct the endoscopic vision of the surgical field from the pre-surgical radiographic images because the lesion remarkably displaces the geography of normal anatomic structures. We created a precise three-dimensional computer graphic model from preoperative radiographic data that was then superimposed on a visual image of the actual surgical field and displayed on a video monitor during endoscopic transsphenoidal surgery. We evaluated the efficacy of this augmented reality (AR) navigation system in 15 consecutive patients with sellar and parasellar tumors. The average score overall was 4.7 [95% confidence interval: 4.58-4.82], which indicates that the AR navigation system was as useful as or more useful than conventional navigation in certain patients. In two patients, AR navigation was assessed as less useful than conventional navigation because perception of the depth of the lesion was more difficult. The developed system was more useful than conventional navigation for facilitating an immediate three-dimensional understanding of the lesion and surrounding structures.

Long-term Outcomes of Non-vascularized Multilayer Fascial Closure Technique for Dural Repair in Endoscopic Transnasal Surgery: Efficacy, Durability, and Limitations.

OBJECTIVE: Non-vascularized multilayer fascial closure technique (NMFCT) can be used instead of nasoseptal flap reconstruction for dural repair in endoscopic transnasal surgery (ETS); however, due to the lack of blood supply, its long-term durability and possible limitations need to be clarified. METHODS: This was a retrospective study on patients who underwent ETS with intraoperative cerebrospinal fluid (CSF) leakage. We assessed the postoperative and delayed CSF leakage rates and the associated risk factors. RESULTS: Among 200 ETSs with intraoperative CSF leakage, 148 (74.0%) ETSs were performed for skull base pathologies other than pituitary neuroendocrine tumor. The mean follow-up period was 34.4 months. Esposito grade 3 leakage was confirmed in 148 (74.0%) cases. NMFCT was used either with (67 [33.5%]) or without (133 [66.5%]) lumbar drainage. There were 10 cases (5.0%) of postoperative CSF leakage that necessitated reoperation. In 4 other cases (2.0%), CSF leakage was suspected but lumbar drainage alone successfully restored the condition. Multivariate logistic regression analyses revealed that posterior skull base location (P < 0.01, odds ratio 11.5, 95% CI 1.99-2.17 × 102) and craniopharyngioma pathology (P = 0.03, odds ratio 9.4, 95% CI 1.25-1.92 × 102) were significantly associated with postoperative CSF leakage. No delayed leakage occurred during the observation period except for 2 patients who underwent multiple radiotherapies. CONCLUSIONS: NMFCT is a reasonable alternative with long-term durability, though vascularized flap may be a better choice for cases in which vascularity of the surrounding tissues is significantly impaired due to interventions including multiple radiotherapies.

Age-Dependent Hemorrhage Risk and Obliteration Benefit After Radiosurgery for Brain Arteriovenous Malformation.

PURPOSE: Because patients with untreated brain arteriovenous malformations (BAVMs) are at variable risks of cerebral hemorrhage and associated mortality and morbidity, it is essential to identify patient populations who benefit most from prophylactic interventions. This study aimed to examine age-dependent differences in the therapeutic effect of stereotactic radiosurgery (SRS) on BAVMs. METHODS AND MATERIALS: This retrospective observational study enrolled patients with BAVMs who underwent SRS at our institution between 1990 and 2017. The primary outcome was post-SRS hemorrhage, and the secondary outcomes included nidus obliteration, post-SRS early signal changes, and mortality. To investigate age-related differences in outcomes after SRS, we performed age-stratified analyses using the Kaplan-Meier analysis and weighted logistic regression with the inverse probability of censoring weighting (IPCW). To address significant differences in patient baseline characteristics, we also performed inverse probability of treatment weighting (IPTW) adjusted for possible confounders to investigate age-related differences in outcomes after SRS. RESULTS: A total of 735 patients with 738 BAVMs were stratified by age. Age-stratified analysis using a weighted logistic regression model with IPCW showed a direct correlation between patient age and post-SRS hemorrhage (odds ratio [OR], 95% confidence interval [CI], and P value: 2.20, 1.34-3.63, and .002 at 18 months; 1.86, 1.17-2.93, and .008 at 36 months; and 1.61, 1.05-2.48, and .030 at 54 months, respectively). The age-stratified analysis also showed an inverse relationship between age and obliteration over the first 42 months after SRS (OR, 95% CI, and P value: 0.05, 0.02-0.12, and <.001 at 6 months; 0.55, 0.44-0.70, and <.001 at 24 months; and 0.76, 0.63-0.91, and .002 at 42 months, respectively). These results were also confirmed with the IPTW analyses. CONCLUSIONS: Our analysis demonstrated that patients' age at SRS is significantly associated with hemorrhage and the nidus obliteration rate after treatment. In particular, younger patients are more likely to exhibit reduced cerebral hemorrhages and achieve earlier nidus obliteration compared with older patients.

Somatic GJA4 gain-of-function mutation in orbital cavernous venous malformations.

Orbital cavernous venous malformation (OCVM) is a sporadic vascular anomaly of uncertain etiology characterized by abnormally dilated vascular channels. Here, we identify a somatic missense mutation, c.121G > T (p.Gly41Cys) in GJA4, which encodes a transmembrane protein that is a component of gap junctions and hemichannels in the vascular system, in OCVM tissues from 25/26 (96.2%) individuals with OCVM. GJA4 expression was detected in OCVM tissue including endothelial cells and the stroma, through immunohistochemistry. Within OCVM tissue, the mutation allele frequency was higher in endothelial cell-enriched fractions obtained using magnetic-activated cell sorting. Whole-cell voltage clamp analysis in Xenopus oocytes revealed that GJA4 c.121G > T (p.Gly41Cys) is a gain-of-function mutation that leads to the formation of a hyperactive hemichannel. Overexpression of the mutant protein in human umbilical vein endothelial cells led to a loss of cellular integrity, which was rescued by carbenoxolone, a non-specific gap junction/hemichannel inhibitor. Our data suggest that GJA4 c.121G > T (p.Gly41Cys) is a potential driver gene mutation for OCVM. We propose that hyperactive hemichannel plays a role in the development of this vascular phenotype.

Stereotactic radiosurgery for skull base adenoid cystic carcinoma: A report of two cases.

Background: Adenoid cystic carcinoma (ACC) is an uncommon salivary gland tumor with a relatively favorable prognosis. However, treating ACC is potentially challenging because radical resection is usually difficult once the skull base is involved due to the adjacent critical structures. Stereotactic radiosurgery (SRS) is a less invasive alternative for surgically recalcitrant lesions. Case Description: We report two patients with three metastatic skull base ACCs who underwent SRS using the Gamma Knife with a marginal dose of 20 Gy to a 50% isodose line. All tumors were effectively controlled without any adverse events. Conclusion: This case report and our review of the literature suggest that SRS can be considered for local control of ACC invading the skull base when surgical resection is unsuitable or a postoperative residual lesion is suspected. Further, investigations on the accumulated subjects are warranted to confirm the role of SRS for the treatment of ACCs.

Long-term progression-free survival achieved in the skull base metastasis of gastrointestinal stromal tumor with introduction of tyrosine kinase inhibitor: illustrative case.

BACKGROUND: Gastrointestinal stromal tumors (GISTs) are common subepithelial tumors that rarely metastasize to the intracranial space. Because the standard treatment for metastatic intracranial GISTs has not been established, multimodal therapies are needed, especially in the case of skull base metastasis. However, its outcome has not always been favorable. The authors report the longest known surviving case of skull base metastasis of GIST treated with imatinib only. OBSERVATIONS: A 52-year-old male with a history of GIST presented with left facial swelling and numbness. Examinations revealed a 70-mm tumor occupying the left middle cranial fossa and the orbit. The authors performed transnasal endoscopic tumor biopsy for definitive diagnosis and reintroduced imatinib treatment. The tumor significantly decreased in size early after the introduction of imatinib, and symptoms completely disappeared within several weeks. The lesion has remained shrunk radiologically for 63 months, and the patient is continuously being followed up under imatinib treatment. LESSONS: The authors reported a rare case of skull base metastasis of GIST successfully treated solely with systemic therapy with a tyrosine kinase inhibitor, achieving tumor control for over 5 years. This case suggests that tyrosine kinase inhibitors might play a key role in the multidisciplinary treatment for skull base metastases of GIST.