Malignant progression of cerebellopontine angle solitary fibrous tumors following radiation: illustrative case.

BACKGROUND: Intracranial solitary fibrous tumors (ISFTs) are rare mesenchymal tumors originating in the meninges and constitute a heterogeneous group of clinical and biological behavior. Benign histotypes, such as hemangiopericytomas are now considered as a cellular phenotypic variant of this heterogenous group of rare spindle-cell tumors. IFSTs are poorly recognized and remain a diagnostic challenge due to rarity and resemblance to other brain tumors. Previously, IFSTs were thought to pursue a slow, indolent, and nonaggressive course, however, a growing body of literature based on longer follow-up demonstrates an unpredictable clinical course and an uncertain diagnosis. OBSERVATIONS: A rare case report of malignant transformation of IFST following radiation therapy is reported. In this case a 60-year-old female who underwent gross total resection of the cerebellopontine angle tumor with histopathology consistent with solitary fibrous tumor followed by salvage stereotactic radiosurgery, presented with another recurrence after 2 years of surgery. The authors performed complete removal of the tumor with pathology now consistent with malignant solitary fibrous tumor. A recent follow-up magnetic resonance imaging did not show any recurrence or residual tumor, and the patient reports a generalized well-being. LESSONS: This report will help to understand the natural history and unusual clinical behavior of these intracranial tumors.

Safety and Efficacy of Hypofractionated Stereotactic Radiosurgery in Facial Nerve Schwannoma.

OBJECTIVE: In the current era of modern neurosurgery, the treatment strategies have been shifted to "nerve-preservation approaches" for achieving a higher facial and hearing function preservation rate following facial nerve tumors. We have conducted this novel report on determining the outcome of patients with facial nerve schwannomas (FNS) treated with hypofractionated stereotactic radiosurgery (hfSRS). PATIENTS: Retrospective chart review of a prospectively maintained database search was conducted. INTERVENTION: Patients who underwent hfSRS CyberKnife (Accuray Inc, Sunnyvale, CA, U.S.A.) for FNS were included. MAIN OUTCOME MEASURES: Outcomes consisted of tumor control, facial and hearing nerve function as graded by House-Brackmann and American Academy of Otolaryngology-Head and Neck Surgery recommendations, and adverse radiation effects. RESULTS: With an institutional board review approval, we retrospectively identified five patients with FNS (four intracranial [80%] and one extracranial [20%]) treated with hfSRS (2011-2019). Patients received definitive SRS in three patients (60.0%), whereas adjuvant to surgical resection in two patients (40.0%). A median tumor volume of 7.5 cm 3 (range, 1.5-19.6 cm 3 ) received a median prescription dose of 23.2 Gy (range, 21-25 Gy) administered in median of three fractions (range, three to five sessions). With a median radiographic follow-up of 31.4 months (range, 13.0-71.0 mo) and clinical follow-up of 32.6 months (range, 15.1-72.0 mo), the local tumor control was 100.0%. At the last clinical follow-up, the facial nerve function improved or remained unchanged House-Brackmann I-II in 80.0% of the patients, whereas the hearing nerve function improved or remained stable in 100.0% of the patients. Temporary clinical toxicity was observed in three patients (60.0%), which resolved. None of the patients developed adverse radiation effect. CONCLUSION: From our case series, hfSRS in FNS seems to be safe and efficacious in terms of local tumor control, and improved facial and hearing nerve function.

Segmental spinal neurofibromatosis 1: a novel phenotype.

INTRODUCTION: Segmental neurofibromatosis (SNF) is a rare subtype of neurofibromatosis (NF). The disease is characterized by features circumscribed to one or more body cutaneous and/or subcutaneous segments. This is a classic example of somatic mosaicism which occurs by postzygotic mut ation of the NF1 gene late in the course of embryonic development affecting localized neural crest lines in the fetus. Spinal neurofibromatosis, on the other hand, is characterized by histologically proven bilateral neurofibromas of the spinal roots. METHODS: Hereby we describe a novel manifestation of spinal SNF. RESULTS: Our case report demonstrated one patient who had segmental spinal expression of the disease classified as true mosaic/segmental NF1 along with its management plan treated at one of the largest NF1 centers to exist. CONCLUSION: This will aid in understanding the rare clinical presentation and treatment options for this novel phenotype.

Predictors of hearing functional outcome following surgery for cerebellopontine angle meningioma.

OBJECTIVE: Cerebellopontine angle (CPA) meningiomas can affect hearing function and require expeditious treatment to prevent permanent hearing loss. The authors sought to determine the factors associated with functional hearing outcome in CPA meningioma patients treated with surgery and/or radiation therapy in the form of either stereotactic radiosurgery or stereotactic radiation therapy. METHODS: Consecutive patients with CPA meningiomas who had presented at our hospital from 2008 to 2018 were identified through retrospective chart review. Hearing function (as defined by pure tone average (PTA) and speech discrimination score (SDS) on Audiogram) was assessed before and after surgery for CPA meningioma. Audiograms with PTA > 50 dB and SDS < 69% were defined as poor hearing functional outcome. Multivariable Cox Proportional Hazards Regression Model was used to assess the associations between pre-operative hearing functional assessment and post-operative hearing functional outcomes. RESULTS: The study cohort included 31 patients (80.6% females, with a mean age of 61.3 ± 15.2 years) with a median clinical follow-up of 5 months (range: 1 week-98 months). The mean pre-operative PTA and SDS were 23.8 ± 11.2 dB and 64.4 ± 22.2% respectively. At the last visit, there was significant hearing recovery, with an improvement of 29.7 ± 18.0 dB (p < 0.001) and 87.6 ± 17.8% (p < 0.001) in PTA and SDS respectively. After adjusting for age, gender, tumor volume, location, and tumor classification, Multivariable Cox Proportional Hazards Regression Model was conducted which revealed that patients undergoing surgery through retro sigmoid approach [Hazards Ratio (HR): 32.1, 95% Confidence Interval (CI): 2.11-491.0, p = 0.01] and gross total resection (GTR) (HR: 2.99, 95% CI: 1.09-9.32, p = 0.05) had significantly higher risk of poor hearing functional outcome compared to petrosal approach and near/subtotal resection. Moreover, patients with poor preoperative hearing had 85% higher chance of poor hearing functional outcome postoperatively (HR: 0.15, 95%CI: 0.03-0.59, p = 0.007). CONCLUSION: Postoperative improvement in hearing is a reasonable expectation following surgery for CPA meningioma. Preoperative hearing, surgical approach and extent of surgical resection are predictive factors of postoperative hearing function outcome and can therefore aid in identification of patients at higher risk of hearing loss.