Amparo Wolf

Cerebellar liponeurocytoma: a rare intracranial tumor with possible familial predisposition. Case report.

The biological origin of cerebellar liponeurocytomas is unknown, and hereditary forms of this disease have not been described. Here, the authors present clinical and histopathological findings of a young patient with a cerebellar liponeurocytoma who had multiple immediate family members who harbored similar intracranial tumors. A 37-year-old otherwise healthy woman presented with a history of progressive headaches. Lipomatous medulloblastoma had been diagnosed previously in her mother and maternal grandfather, and her maternal uncle had a supratentorial liponeurocytoma. MRI revealed a large, poorly enhancing, lipomatous mass emanating from the superior vermis that produced marked compression of posterior fossa structures. An uncomplicated supracerebellar infratentorial approach was used to resect the lesion. Genetic and histopathological analyses of the lesion revealed neuronal, glial, and lipomatous differentiation and confirmed the diagnosis of cerebellar liponeurocytoma. A comparison of the tumors resected from the patient and, 22 years previously, her mother revealed similar features. Cerebellar liponeurocytoma is a poorly understood entity. This report provides novel evidence of an inheritable predisposition for tumor development. Accurate diagnosis and reporting of clinical outcomes and associated genetic and histopathological changes are necessary for guiding prognosis and developing recommendations for patient care.

Toward the complete control of brain metastases using surveillance screening and stereotactic radiosurgery

OBJECTIVE The incidence of brain metastases is increasing with improved systemic therapies, many of which have a limited impact on intracranial disease. Stereotactic radiosurgery (SRS) is a first-line management option for brain metastases. The purpose of this study was to determine if there is a threshold tumor size below which local control (LC) rates approach 100%, and to relate these findings to the use of routine surveillance brain imaging. METHODS From a prospective registry, 200 patients with 1237 brain metastases were identified who underwent SRS between December 2012 and May 2015. The median imaging follow-up duration was 7.9 months, and the median margin dose was 18 Gy. The maximal diameter and volume of tumors were measured. Histological analysis included 96 patients with non-small cell lung cancers (NSCLCs), 40 with melanoma, 35 with breast cancer, and 29 with other histologies. RESULTS Almost 50% of brain metastases were NSCLCs and commonly measured less than 6 mm in maximal diameter or 70 mm3 in volume. Thirty-three of 1237 tumors had local progression at a median of 8.8 months. The 1- and 2-year actuarial LC rates were 97% and 93%, respectively. LC of 100% was achieved for all intracranial metastases less than 100 mm3 in volume or 6 mm in diameter. Patients whose tumors at first SRS were less than 10 mm maximal diameter or a volume of 250 mm3 had improved overall survival. CONCLUSIONS SRS can achieve LC rates approaching 100% for subcentimeter metastases. The earlier initial detection and prompt treatment of small intracranial metastases may prevent the development of neurological symptoms and the need for resection, and improve overall survival. To identify tumors when they are small, routine surveillance brain imaging should be considered as part of the standard of care for lung, breast, and melanoma metastases. ■ CLASSIFICATION OF EVIDENCE Type of question: prognostic; study design: retrospective cohort; evidence: Class II.

The relationship of dose to nerve volume in predicting pain recurrence after stereotactic radiosurgery in trigeminal neuralgia

OBJECTIVE Approximately 75%-92% of patients with trigeminal neuralgia (TN) achieve pain relief after Gamma Knife surgery (GKS), although a proportion of these patients will experience recurrence of their pain. To evaluate the reasons for durability or recurrence, this study determined the impact of trigeminal nerve length and volume, the nerve dose-volume relationship, and the presence of neurovascular compression (NVC) on pain outcomes after GKS for TN. METHODS Fifty-eight patients with 60 symptomatic nerves underwent GKS for TN between 2013 and 2015, including 15 symptomatic nerves secondary to multiple sclerosis (MS). High-resolution MRI was acquired the day of GKS. The median maximum dose was 80 Gy for initial GKS and 65 Gy for repeat GKS. NVC, length and volume of the trigeminal nerve within the subarachnoid space of the posterior fossa, and the ratio of dose to nerve volume were assessed as predictors of recurrence. RESULTS Follow-up was available on 55 patients. Forty-nine patients (89.1%) reported pain relief (Barrow Neurological Institute [BNI] Grades I-IIIb) after GKS at a median duration of 1.9 months. The probability of maintaining pain relief (BNI Grades I-IIIb) without requiring resumption or an increase in medication was 93% at 1 year and 84% at 2 years for patients without MS, and 68% at 1 year and 51% at 2 years for all patients. The nerve length, nerve volume, target distance from the brainstem, and presence of NVC were not predictive of pain recurrence. Patients with a smaller volume of nerve (< 35% of the total nerve volume) that received a high dose (≥ 80% isodose) were less likely to experience recurrence of their TN pain after 1 year (mean time to recurrence: < 35%, 32.2 ± 4.0 months; > 35%, 17.9 ± 2.8 months, log-rank test, χ2 = 4.3, p = 0.039). CONCLUSIONS The ratio of dose to nerve volume may predict recurrence of TN pain after GKS. Prospective studies are needed to determine the optimal dose to nerve volume ratio and whether this will result in longer pain-free outcomes.

Bilateral caudate nucleus infarcts: A case report of a rare complication following endoscopic resection of a tuberculum sellae meningioma

Background: We present a rare complication of bilateral caudate infarcts and necrosed nasoseptal flaps after endoscopic transsphenoidal resection of tuberculum sellae meningioma. This case highlights the importance of early and accurate diagnosis and treatment of a postoperative cerebrospinal fluid (CSF) leak and associated bacterial meningitis, and reviews any existing guidelines regarding its management. Case Description: A 54-year-old otherwise healthy man presented with progressive bitemporal hemianopsia. Magnetic resonance imaging of the head revealed a large, homogeneously enhancing sellar and suprasellar mass consistent with a meningioma. An endoscopic endonasal transsphenoidal approach was performed to resect the tuberculum sellae meningioma. The patient developed basal bacterial meningitis secondary to a CSF leak, requiring repair on two separate occasions. At the time of both repairs, there was evidence of necrosis of the nasoseptal flaps used for the repairs. Soon after the diagnosis of meningitis, the patient developed bilateral caudate infarcts. Conclusion: This report discusses the possible underlying etiologies for the bilateral caudate infarcts and necrosed flaps including bacterial meningitis with associated local vasospasm of nearby vessels resulting in infarction. This case emphasizes the importance of concise management of postendoscopic CSF leak and discusses the guidelines regarding antimicrobial therapy and the management of lumbar drains.

Quantitative evaluation of vision-related and health-related quality of life after endoscopic transsphenoidal surgery for pituitary adenoma

OBJECTIVE Endoscopic resection of pituitary adenomas has been reported to improve vision function in up to 80%-90% of patients with visual impairment due to these adenomas. It is unclear how these reported rates translate into improvement in visual outcomes and general health as perceived by the patients. The authors evaluated self-assessed health-related quality of life (HR-QOL) and vision-related QOL (VR-QOL) in patients before and after endoscopic resection of pituitary adenomas. METHODS The authors prospectively collected data from 50 patients who underwent endoscopic resection of pituitary adenomas. This cohort included 32 patients (64%) with visual impairment preoperatively. Twenty-seven patients (54%) had pituitary dysfunction, including 17 (34%) with hormone-producing tumors. Patients completed the National Eye Institute Visual Functioning Questionnaire and the 36-Item Short Form Health Survey preoperatively and 6 weeks and 6 months after surgery. RESULTS Patients with preoperative visual impairment reported a significant impact of this condition on VR-QOL preoperatively, including general vision, near activities, and peripheral vision; they also noted vision-specific impacts on mental health, role difficulties, dependency, and driving. After endoscopic resection of adenomas, patients reported improvement across all these categories 6 weeks postoperatively, and this improvement was maintained by 6 months postoperatively. Patients with preoperative pituitary dysfunction, including hormone-producing tumors, perceived their general health and physical function as poorer, with some of these patients reporting improvement in perceived general health after the endoscopic surgery. All patients noted that their ability to work or perform activities of daily living was transiently reduced 6 weeks postoperatively, followed by significant improvement by 6 months after the surgery. CONCLUSIONS Both VR-QOL and patients perceptions of their ability to do work and perform other daily activities as a result of their physical health significantly improved by 6 months after endoscopic resection of pituitary adenoma. The use of multidimensional QOL questionnaires provides a precise assessment of perceived outcomes after endoscopic surgery.

195 How Well Do Neuroradiologists Predict the Side of Trigeminal Neuralgia

INTRODUCTION With improving MRI techniques, physicians can better visualize neurovascular compression (NVC) of the trigeminal nerve, as well as proximal and distal nerve atrophy, nerve distortion, and demyelinating plaques. This study aimed to determine the interrater reliability of these different anatomical variables, as well as the accuracy in predicting the side of trigeminal neuralgia (TN) for 2 neuroradiologists. METHODS High-resolution MRI sequences including T1-Gadolinium and constructive interference in steady state (CISS) sequences were reviewed in 43 patients, with symptomatic TN in 44 nerves, who subsequently underwent Gamma Knife radiosurgery. Thirteen patients had multiple sclerosis (MS)-associated TN. Two neuroradiologists (NR1, NR2), blinded to the side of TN, assessed for the presence or absence of NVC, proximal or distal atrophy, distorted nerve course, as well as predicted the side of TN. RESULTS The presence of ipsilateral NVC was reported in 79.5% (NR1) and 70.5% (NR2) of nerves, proximal nerve atrophy in 36.4% (NR1) and 40.9% (NR2), distal nerve atrophy in 20.5% (NR1) and 9.1% (NR2), and nerve distortion in 56.8% (NR1) and 38.6% (NR2). Moderate interrater reliability was seen for the assessment of NVC (κ = 0.52, P < .001), while there was only fair reliability for proximal and distal nerve atrophy and nerve distortion (κ = 0.14-0.24, P > .05). MS patients were less likely to have ipsilateral NVC. Sensitivity and specificity of predicting the side of TN was 75.6% and 58.0% for NR1 and 61.4% and 58.2% for NR2, respectively. Interrater reliability on predicting the side of TN was also moderate (κ = 0.42, P < .001). CONCLUSION Despite improving MRI, the accuracy of predicting the side of TN is limited with moderate interrater reliability. Newer MRI techniques assessing the trigeminal nerve pathway are needed to improve diagnostic accuracy and to better understand the underlying etiology of TN.

Brain metastases: radiosurgery

Stereotactic radiosurgery has revolutionized the management of brain metastases. It delivers focused, highly conformal, ionizing radiation to a tumor delineated using high-resolution imaging, with low toxicity to adjacent brain structures. Randomized controlled and prospective trials have demonstrated a survival advantage and high local control rates after stereotactic radiosurgery for metastatic disease to the central nervous system, including for up to 10 brain metastases. Its minimal-access nature makes it an attractive alternative to surgical resection. Furthermore, in addition to chemotherapy, newer targeted therapies and immunotherapies with improved side-effect profiles allow for the concurrent delivery of systemic therapy with radiosurgery, with possible additive or synergistic effects, expediting the treatment of both extracranial and intracranial disease. The modern management of brain metastasis patients should include consideration of routine staging and surveillance magnetic resonance imaging scans in patients with higher-stage cancer to detect intracranial metastases earlier and treat promptly with radiosurgery in order to prevent the development of neurologic symptoms and the need for surgical resection.

Radiosurgery for Arteriovenous Malformations

Stereotactic radiosurgery (SRS) is an important therapeutic option for the care of patients with an intracranial arteriovenous malformation (AVM). It can achieve complete obliteration of blood flow within the AVM, thereby eliminating the risk of future intracranial hemorrhage. The overall morbidity of SRS in AVMs is relatively low. However, complications can arise subsequent to SRS of AVMs and include most commonly delayed hemorrhage, hemodynamic effects on the regional brain from AVM vessel closure, radiation injury to the adjacent brain, and delayed cyst formation. This chapter reviews the principles of complication avoidance and management in the radiosurgical management of AVMs.

Repeat Stereotactic Radiosurgery for Progressive or Recurrent Vestibular Schwannomas

BACKGROUND Stereotactic radiosurgery (SRS) is a highly effective management approach for patients with vestibular schwannomas (VS), with 10-yr control rates up 98%. When it fails, however, few data are available to guide management. OBJECTIVE To perform a retrospective analysis of patients who underwent 2 SRS procedures on the same VS to assess the safety and efficacy of this practice. METHODS This study was opened to centers of the International Gamma Knife Research Foundation (IGKRF). Data collected included patient characteristics, clinical symptoms at the time of SRS, radiosurgery dosimetric data, imaging response, clinical evolution, and survival. Actuarial analyses of tumor responses were performed. RESULTS Seventy-six patients from 8 IGKRF centers were identified. Median follow-up from the second SRS was 51.7 mo. Progression after the first SRS occurred at a median of 43 mo. Repeat SRS was performed using a median dose of 12 Gy. Actuarial tumor control rates at 2, 5, and 10 yr following the second SRS were 98.6%, 92.2%, and 92.2%, respectively. Useful hearing was present in 30%, 8%, and 5% of patients at first SRS, second SRS, and last follow-up, respectively. Seventy-five percent of patients reported stable or improved symptoms following the second SRS. Worsening of facial nerve function attributable to SRS occurred in 7% of cases. There were no reports of radionecrosis, radiation-associated edema requiring corticosteroids, radiation-related neoplasia, or death attributable to the repeat SRS procedure. CONCLUSION Patients with progressing VS after radiosurgery can be safely and effectively managed using a second SRS procedure.

Commentary: Ten-Year Follow-up on Tumor Growth and Hearing in Patients Observed With an Intracanalicular Vestibular Schwannoma

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