Background Meningiomas are the most common primary intracranial tumors in adults. Identification of SMO and AKT1 mutations in meningiomas has raised the hope for targeted therapies. It would be useful to know the precise frequency of these mutations in anatomical subgroups and clarify their prognostic value. Methods We used the Sanger sequencing technique to characterize 79 samples of olfactory groove meningiomas for SMO (L412F and W535L) and AKT1E17K mutations. We reviewed clinical data to assess the prognostic value of these mutations in this anatomical subgroup. Results Out of the 79 patients with olfactory groove meningiomas, we identified targetable mutations in 34 patients (43%) (22 patients [28%] with SMO mutation-L412F almost exclusively-and 12 patients [15%] with AKT1 mutation). Meningiomas in the SMO-mutant group had an overall 36% recurrence rate, significantly higher than in the AKT1-mutant group (16%) and in the "SMO and AKT1 wildtype" group (11%) (χ2 test, P = .04). All late recurrences (after 5 y) occurred in the SMO-mutant group. Among grade I meningiomas, the SMO-mutant group was identified as having a significantly poorer prognosis. World Health Organization histological grade II (P = .006) and incomplete resection (P = .001) were independently associated with shorter recurrence-free survival. Conclusion Molecular diagnosis of SMOL412F/W535L and AKT1E17K mutations improves prognostic evaluation in olfactory groove meningiomas and opens new therapeutic perspectives with SMO or AKT inhibitors for recurrent cases.

/pdf/smo-mutation-status-defines-a-distinct-and-frequent-uhkasw8oja.pdf

SMO mutation status defines a distinct and frequent molecular subgroup in olfactory groove meningiomas.

OBJECTIVE Anterior skull base meningiomas are benign lesions that cause neurological symptoms through mass effect on adjacent neurovascular structures. While traditional transcranial approaches have proven to be effective at removing these tumors, minimally invasive approaches that involve using an endoscope offer the possibility of reducing brain and nerve retraction, minimizing incision size, and speeding patient recovery; however, appropriate case selection and results in large series are lacking. METHODS The authors developed an algorithm for selecting a supraorbital keyhole minicraniotomy (SKM) for olfactory groove meningiomas or an expanded endoscopic endonasal approach (EEA) for tuberculum sella (TS) or planum sphenoidale (PS) meningiomas based on the presence or absence of olfaction and the anatomical extent of the tumor. Where neither approach is appropriate, a standard transcranial approach is utilized. The authors describe rates of gross-total resection (GTR), olfactory outcomes, and visual outcomes, as well as complications, for 7 subgroups of patients. Exceptions to the algorithm are also discussed. RESULTS The series of 57 patients harbored 57 anterior skull base meningiomas; the mean tumor volume was 14.7 ± 15.4 cm3 (range 2.2-66.1 cm3), and the mean follow-up duration was 42.2 ± 37.1 months (range 2-144 months). Of 19 patients with olfactory groove meningiomas, 10 had preserved olfaction and underwent SKM, and preservation of olfaction in was seen in 60%. Of 9 patients who presented without olfaction, 8 had cribriform plate invasion and underwent combined SKM and EEA (n = 3), bifrontal craniotomy (n = 3), or EEA (n = 2), and one patient without both olfaction and cribriform plate invasion underwent SKM. GTR was achieved in 94.7%. Of 38 TS/PS meningiomas, 36 of the lesions were treated according to the algorithm. Of these 36 meningiomas, 30 were treated by EEA and 6 by craniotomy. GTR was achieved in 97.2%, with no visual deterioration and one CSF leak that resolved by placement of a lumbar drain. Two patients with tumors that, based on the algorithm, were not amenable to an EEA underwent EEA nonetheless: one had GTR and the other had a residual tumor that was followed and removed via craniotomy 9 years later. CONCLUSIONS Utilizing a simple algorithm aimed at preserving olfaction and vision and based on maximizing use of minimally invasive approaches and selective use of transcranial approaches, the authors found that excellent outcomes can be achieved for anterior skull base meningiomas.

/pdf/decision-making-algorithm-for-minimally-invasive-approaches-4y3ijd676z.pdf

Decision-making algorithm for minimally invasive approaches to anterior skull base meningiomas.

Head and Neck Cancer: Nasopharynx.- Oropharynx Cancer.- Early Larynx.- Advanced larynx.- Hypopharyngeal Cancer.- Oral Cavity Cancer.- Paranasal Sinuses.- Major Salivary gland.- Thyroid Cancer.- Cranial Nerves.- Unknown Primary.- Head and Neck.- Neck.- Breast Cancer: Early Breast Cancer.- Locally Advanced Breast.- Thorax: Locally Advanced NSCLC &SCLD.- SBRT and post-OP NSCLC.- Gastrointestinal Cancer: Esophagus.- Gastric.- Pancreatic.- HCC/Cholangiocarcinoma.- Rectal Cancer.- Anal Cancer.- Gynecological Cancer: Cervical Cancer.- Endometrial Cancer.- Ovarian Cancer.- Vaginal Cancer.- Vulvar Cancer.- Genitourinary Cancer: Prostate Cancer.- Bladder Cancer.-Seminoma .- CNS: Brain Metastasis.- Benign.- Low and High Grade Glioma.- Lymphoma: Hodgkin's Disease.- Non-Hodgkin's Disease.- Musculoskeletal.- Pediatrics: Sarcoma.- Pediatric CNS.

Target Volume Delineation for Conformal and Intensity-Modulated Radiation Therapy

An understanding of the present standing of surgery, surgical results and the role in altering the future morbidity and mortality of untreated brain arteriovenous malformations (bAVMs) is appropriate considering the myriad alternative management pathways (including radiosurgery, embolization or some combination of treatments), varying risks and selection biases that have contributed to confusion regarding management The purpose of this review is to clarify the link between the incidence of adverse outcomes that are reported from a management pathway of either surgery or no intervention with the projected risks of surgery or no intervention A critical review of the literature was performed on the outcomes of surgery and non-intervention for bAVM An analysis of the biases and how these may have influenced the outcomes was included to attempt to identify reasonable estimates of risks In the absence of treatment, the cumulative risk of future hemorrhage is approximately 16% and 29% at 10 and 20 years after diagnosis of bAVM without hemorrhage and 35% and 45% at 10 and 20 years when presenting with hemorrhage (annualized, this risk would be approximately 18% for unruptured bAVMs and 47% for 8 years for bAVMs presenting with hemorrhage followed by the unruptured bAVM rate) The cumulative outcome of these hemorrhages depends upon whether the patient remains untreated and is allowed to have a further hemorrhage or is treated at this time Overall, approximately 42% will develop a new permanent neurological deficit or death from a hemorrhagic event The presence of an associated proximal intracranial aneurysm (APIA) and restriction of venous outflow may increase the risk for subsequent hemorrhage Other risks for increased risk of hemorrhage (age, pregnancy, female) were examined, and their purported association with hemorrhage is difficult to support Both the Spetzler-Martin grading system (and its compaction into the Spetzler-Ponce tiers) and Lawton-Young supplementary grading system are excellent in predicting the risk of surgery The 8-year risk of unfavorable outcome from surgery (complication leading to a permanent new neurological deficit with a modified Rankin Scale score of greater than one, residual bAVM or recurrence) is dependent on bAVM size, the presence of deep venous drainage (DVD) and location in critical brain (eloquent location) For patients with bAVMs who have neither a DVD nor eloquent location, the 8-year risk for an unfavorable outcome increases with size (increasing from 1 cm to 6 cm) from 1% to 9% For patients with bAVM who have either a DVD or eloquent location (but not both), the 8-year risk for an unfavorable outcome increases with the size (increasing from 1 cm to 6 cm) from 4% to 35% For patients with bAVM who have both a DVD and eloquent location, the 8-year risk for unfavorable outcome increases with size (increasing from 1 cm to 3 cm) from 12% to 38% Patients with a Spetzler-Ponce A bAVM expecting a good quality of life for the next 8 years are likely to do better with surgery in expert centers than remaining untreated Ongoing research is urgently required on the outcome of management pathways for bAVM

Critical review of brain AVM surgery, surgical results and natural history in 2017

Cerebral arteriovenous malformations (AVMs) are composed of a complex tangle of abnormal arteries and veins and are a significant source of cerebral hemorrhage and consequent morbidity and mortality in young adults, representing a diagnostic and therapeutic challenge Current natural-history studies of cerebral AVMs report overall annual rates of 1% and 3% for the risk of epilepsy and hemorrhage, respectively Unruptured AVMs have an annual hemorrhage rate of 22% while ruptured lesions have an annual hemorrhage rate of 45% These hemorrhage rates are can change over time, particularly for hemorrhagic lesions, with the rebleed rate ranging from 6% to 158% in the first year after rupture across several studies Besides hemorrhage, other significant risk factors for AVM hemorrhage include deep location, deep venous drainage, associated aneurysms, and pregnancy Other factors include patient age, sex, and small AVM size, which are not currently considered significant risk factors for AVM hemorrhage In addition to hemorrhage risk and seizure risk, the natural history of an AVM also encompasses the daily psychologic burden that a patient must endure knowing that he or she possesses an untreated AVM This chapter reviews the epidemiology, clinical features, and natural history of cerebral AVMs

The natural history of cerebral arteriovenous malformations.

Anosmia is a common outcome after resection of olfactory groove meningioma(s) (OGM) and for some patients represents a significant disability. To evaluate long term tumor control rates and preservation of subjective olfaction after Gamma Knife (GK) stereotactic radiosurgery (SRS) of OGM. We performed a retrospective chart review and telephone assessments of 41 patients who underwent GK SRS between 1987 and 2008. Clinical outcomes were stratified by full, partial or no subjective olfaction, whereas tumor control was assessed by changes in volume greater or lesser than 25 %. The median clinical and imaging follow-up were 76 and 65 months, respectively. Prior to SRS, 19 (46 %) patients had surgical resections and two (5 %) had received fractionated radiation therapy. Twenty four patients (59 %) reported a normal sense of smell, 12 (29 %) reported a reduced sense of smell and five (12 %) had complete anosmia. The median tumor volume was 8.5 cm3 (range 0.6–56.1), the mean radiation dose at the tumor margin was 13 Gy (range 10–20) and the median estimated dose to the olfactory nerve was 5.1 Gy (range 1.1–18.1). At follow-up, 27 patients (66 %) reported intact olfaction (three (7 %) described return to a normal sense of smell), nine (22 %) described partial anosmia, and five (12 %) had complete anosmia. No patient reported deterioration in olfaction after SRS. Thirteen patients (32 %) showed significant tumor regression, 26 (63 %) had no further growth and two (5 %) had progressed. The progression free tumor control rates were 97 % at 1 year and 95 % at 2, 10 and 20 years. Symptomatic adverse radiation effects occurred in three (7 %) patients. Stereotactic radiosurgery provided both long term tumor control and preservation of olfaction.

Gamma Knife radiosurgery of olfactory groove meningiomas provides a method to preserve subjective olfactory function.

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.

/pdf/cerebellar-liponeurocytoma-a-rare-intracranial-tumor-with-47ie8o7nhr.pdf

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

Vestibular schwannomas (VS) are benign tumors predominantly originating from the balance portion of cranial nerve VIII. These tumors have an incidence of 1-2 per 100,000 people. The growth of these tumors is approximately 1-2 mm per year. A VS can result in significant neurologic dysfunction from continued growth or the management paradigms designed to control this predominantly benign tumor. The impacts on the critical space within the auditory canal and cerebellopontine angle can lead to hearing deficits, tinnitus, vestibular dysfunction, facial nerve deficits, and brain stem compression.

Leksell Radiosurgery for Vestibular Schwannomas.

In most instances, initial surgery to untether a tethered spinal cord is successful. But what happens when it is not? The authors describe the case of a now 18-year-old woman with spina bifida in whom surgery for tethered cord was required on two occasions. In both instances, due to the extent of her underlying lesion and fibrous tissue, only partial detethering was possible without acutely sacrificing significant neurological function. The authors detail the patient's course and review the peer-reviewed scientific literature on outcomes in patients in whom only partial cord detethering is achieved. In their review of all case series and clinical studies pertaining to the surgical treatment of tethered cord syndrome identified during an online search of 2184 scientific abstracts and 2 major neurosurgery textbooks, excluding the present case, the authors identified 53 confirmed or presumed cases of incomplete detethering in eight articles, incorporating 390 patients, for an overall prevalence of roughly 13...

When only partial spinal cord detethering is possible: Case report

The Response of Cystic Acoustic Neuromas to Radiosurgery.

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