Kiyofuji, Satoshi

Abstract Background Clinical behavior of pituitary neoplasms is peculiar and notoriously difficult to predict. While aggressive tumors are common, metastasis is very rare, can be highly delayed, and there are no histological or clinical features to meaningfully predict this happening. Endocrinologically silent tumors are particularly difficult, as there is less opportunity to detect early metastasis. Together, this amounts to a situation of uncertainty over the appropriate management of such tumors before and after metastasis. Case Description The authors report two cases of nonfunctioning aggressive pituitary adenoma (APA) each requiring two transsphenoidal surgeries, a transcranial resection and radiotherapy. Both these tumors subsequently metastasized caudally along the neuraxis, years later, as a null cell carcinoma associated with a germline CHEK2 mutation and a silent Crooke's cell carcinoma. The former represents a novel oncogenetic association. Conclusion Delayed drop dural metastasis of pituitary carcinoma is becoming increasingly recognized. Surgical resection of the distant disease to confirm the diagnosis and relieve the mass effect, followed by temozolomide chemotherapy, is the current treatment of choice. The need for both long-term follow-up in patients with APA, and a high degree of suspicion toward dural-based radiographic findings is emphasized.

Abstract Background The far-lateral approach is an effective skull base technique that provides access to the lower clivus and premedullary area. This approach is also useful for maximal visualization and resection of large posterior fossa tumors with extensive medial extension, especially when aggressive resection is ideal for a malignant tumor in a young patient, or it is technically challenging because of tumor calcification. We demonstrate a microsurgical operative video to describe technical pearls in this difficult situation. Case Presentation A 45-year-old man with history of “hoarseness” for up to two decades was presented with imbalance and mild dysphagia over 3 years. Imaging demonstrated a 55-mm left cerebellopontine angle (CPA) tumor extending medially across the midline with severe calcification (Figs. 1 and 2). His neurological examination revealed left facial numbness, complete left facial weakness, left deafness, complete left vocal paralysis, as well as severe left hemibody ataxia. The tumor was resected via a left suboccipital craniotomy with far lateral approach including drilling to the occipital condyle and C1 laminectomy. Initial manipulation of the inferior pole of the tumor resulted in asystole which was managed successfully with glycol pyrolate. Pathology demonstrated IDH-1 wild type, MGMT-methylated glioblastoma. The patient subsequently underwent adjuvant chemoradiation. Conclusion The far-lateral approach is an effective approach for maximal safe resection of a malignant brainstem, cerebellar, and CPA tumor.The link to the video can be found at: https://youtu.be/AIGebJPJEnw.

Abstract BACKGROUND Giant fusiform and dolichoectatic aneurysms of the basilar trunk and vertebrobasilar junction (BTVBJ-GFDA) are extremely difficult to treat. OBJECTIVE To evaluate factors influencing survival and outcome of BTVBJ-GFDA by performing a retrospective multicenter cohort study. METHODS A total of 32 patients with BTVBJ-GFDA were included in this study. Clinicopathological characteristics, treatment measures, and outcomes were collected from medical records and imaging studies. Autopsy and histological findings of the aneurysm and adjacent brain tissue were also obtained in 9 cases. RESULTS A total of 11 patients did not undergo surgery, of whom 10 died; 3 from progressive brainstem compression, 4 from subarachnoid hemorrhage, 2 from brainstem infarction, and 1 from associated atherosclerotic disease. The remaining 21 patients underwent a surgical treatment, consisting of immediately proximal parent artery occlusion, remotely proximal parent artery occlusion, clip reconstruction, and distal bypass and achieved significantly longer overall survival compared with those who received conservative therapy (adjusted hazard ratio 1.508, 95% CI 1.058-2.148, P = .02). Histological examination of the aneurysms demonstrated staged clots, open lumen, and intrathrombotic channels with endothelial lining. The patients younger than 45 yr of age showed statistically longer survival than those equal and older than 45 yr ( P = .03). CONCLUSION Surgical intervention achieved greater survival than conservative management in BTVBJ-GFDA. Narrow ideal treatment window of the blood flow within the aneurysm to maintain sufficient but not excess supply should be targeted based on the hemodynamics of both the posterior communicating arteries and perforating vessel collaterals.

Abstract Background Consensus in timing of radiotherapy is yet to be established in esthesioneuroblastoma (ENB). Objective This study was aimed to investigate if planned adjuvant radiotherapy improves tumor control after complete margin negative resection of low Hyams' grade (1 or 2) ENB. Methods A retrospective review of patients with pathologically confirmed negative margin resection of Kadish's stage B or C and Hyams' grade 1 and 2 ENBs was conducted. Seventeen patients meeting the criteria were divided into the following two groups for cohort study: (1) those who underwent planned immediate postoperative adjuvant radiotherapy (IR group) and (2) those who did not (delayed radiotherapy [DR] group). Results The IR group included nine patients (Kadish's stage B in one and stage C in eight; Hyams' grade 1 in two and grade 2 in seven). Mean follow-up was 140.8 months. Seven patients (78%) had disease progression (DP) at a median of 88 months (four with cervical lymph node metastasis [CLNM], one with distant metastasis, and two with both local recurrence and CLNM). One patient experienced frontal lobe abscess. The DR group included eight patients (Kadish's stage B in six and stage C in two; all Hyams' grade 2). Mean follow-up was 123.3 months. Four (50%) patients who developed DP (all local recurrence) were salvaged with surgery and adjuvant radiotherapy at a median of 37.5 months. There was no statistically significant difference in DP rate (p = 0.23), time to DP (p = 0.26), or the local tumor control rate (p = 0.23). Conclusion In our limited cohort, immediate postoperative radiotherapy did not demonstrate superiority in tumor control, although risk of radiotherapy toxicity appears low.

A 60-year-old man with a history of four prior operations for a left cerebellar/middle cerebellar peduncle hemangioblastoma presented with hearing loss, imbalance, and ataxia (de la Monte and Horowitz, 1989). Magnetic resonance imaging (MRI) demonstrated a 3-cm cystic mass with heterogeneous enhancement in the same location. We resected the mass via reopening of the retrosigmoid approach (Lee et al., 2014). Left cranial nerves IV, V, VII, VIII, IX, X, and XI were all well identified and preserved, and feeding arteries from the brainstem were meticulously coagulated and transected without violating the tumor-brainstem interface (Chen et al., 2013). Preoperative embolization greatly aided safe resection of the mass, whose pathology revealed recurrence of hemangioblastoma (Eskridge et al., 1996; Kim et al., 2006; Sakamoto et al., 2012). The video can be found here: https://youtu.be/3mZgY15xOZc .

OBJECTIVESphenoorbital meningioma (SOM) is a unique skull base tumor, characterized by infiltrative involvement and hyperostosis primarily of the lesser wing of sphenoid bone, with frequent involvement of the orbital compartment. SOM often manifests with proptosis and visual impairment. Surgical technique and outcome are highly variable among studies reported in the literature. The authors present a single-surgeon experience with SOM.METHODSA retrospective review of a prospectively maintained institutional database was performed. A blinded imaging review by 2 study team members was completed to confirm SOM, after which chart review was carried out to capture demographics and outcomes. All statistical testing was completed using JMP Pro version 14.1.0, with significance defined as p < 0.05.RESULTSForty-seven patients who underwent surgery between 2000 and 2017 were included. The median age at surgery was 47 years (range 36–70 years), 81% of patients were female, and the median follow-up was 43 months (range 0–175 months). All operations were performed via a frontotemporal craniotomy, orbitooptic osteotomy, and anterior clinoidectomy, with extensive resection of all involved bone and soft tissue. Preoperatively, proptosis was noted in 44 patients, 98% of whom improved. Twenty-eight patients (60%) had visual deficits before surgery, 21 (75%) of whom improved during follow-up. Visual field defect other than a central scotoma was the only prognostic factor for improvement in vision on multivariate analysis (p = 0.0062). Nine patients (19%) had recurrence or progression during follow-up.CONCLUSIONSSOM is a unique skull base tumor that needs careful planning to optimize outcome. Aggressive removal of involved bone and periorbita is crucial, and proptosis and visual field defect other than a central scotoma can improve after surgery.