Health-related quality of life in patients with peripheral nerve tumors: results from the German multicentric Peripheral Nerve Tumor Registry.

Objective: Peripheral nerve tumors (PNTs) are rare diseases. So far, no multicenter data on diagnostics, the efficacy of treatment, long-term outcomes, and health-related quality of life (HRQoL) exist. The establishment of the Peripheral Nerve Tumor Registry (PNTR) in 2015 allows for the systematic analysis of patients with tumors associated with peripheral nerves. The present study aims to investigate the impact of PNT on an individual's HRQoL and the effect of surgery. Methods: HRQoL was pre- and postoperatively assessed by the Euro-Qol-5D-5L (EQ-5D-5L) and Euro-Qol visual analog scale (EQ-VAS) survey in the retrospective and prospective study arm in three active participating study centers. An index was calculated based on the EQ-5D-5L for the quantification of health state (0: worst possible state of health, 1: best possible state of health). The EQ-VAS ranges from 0% (worst imaginable health status) to 100% (best possible health status). Patient characteristics (age, sex), as well as disease (histopathological entity) and treatment (pre- and postoperative symptoms, type of treatment)-specific data, were analyzed. Results: Data from 171 patients from three high-volume centers were included, with schwannoma (70.8%, n = 121) and neurofibroma (15.8%, n = 27) being the most prevalent histopathological diagnoses. Both the median health index value (preoperative: 0.887, n = 167; postoperative: 0.910, n = 166) and the median EQ-VAS (preoperative: 75%, n = 167; postoperative: 85%, n = 166) of the entire cohort regarding all histopathological diagnosis improved significantly after surgical therapy (p < 0.001). Preoperatively, 12.3% (n = 21) reached the highest index score of 1.0 in EQ-5D-5L and 100% in the EQ-VAS score in 5.3% (n = 9) of all patients. Postoperatively, the highest index score of 1.0 and 100% in the EQ-VAS score increased significantly and were achieved in 33.3% (n = 57) and 11.1% (n = 19) of the patients, respectively (p < 0.001). Conclusion: For the first time, our study presents multicenter data on life quality and the effect of surgery in primarily benign peripheral nerve tumors. Early surgery at a specialized center could improve neurological outcomes and, in conclusion, better QoL. In summary, surgical therapy significantly improved the entire cohort's QoL, VAS, and analgesia.

Case report: Atypical neurofibromatous neoplasm with uncertain biological potential of the sciatic nerve and a widespread arteriovenous fistula mimicking a malignant peripheral nerve tumor in a young patient with neurofibromatosis type 1.

We report an unusual constellation of diseases in a 32-year-old woman with neurofibromatosis type 1 (NF1) diagnosed with the recently described precursor entity of malignant peripheral nerve sheath tumor (MPNST), the so-called atypical neurofibromatous neoplasm with unknown biological potential (ANNUBP) and a large symptomatic cervical arteriovenous fistula. An [18F] 2-Fluoro-2-deoxy-D-glucose PET/CT (FDG-PET/CT) was performed to detect and stage a conspicuous symptomatic cervical tumor. The FDG-PET/CT showed high FDG uptake in one of the multiple known tumorous lesions associated with peripheral nerves. However, no relevant FDP uptake was observed in this affected cervical area. After digital subtraction angiography, the cervical mass turned out to be a widespread arteriovenous fistula of the vertebral artery. This was successfully treated using endovascular embolization. Subsequently, magnet resonance imaging (MRI) of the FDG-positive tumor revealed a well-enhanced homogeneous mass of the sciatic nerve measuring 5.2×2.4×2.8 cm. Microsurgical gross total tumor resection was performed using ultrasound. The final histopathological diagnosis was ANNUBP transformed from neurofibroma. The patient benefited excellently from the surgery; no recurrence or metastasis has been observed since resection. According to imaging, ANNUBP can be characterized as a well-enhanced homogeneous mass on MRI, displaying high uptake on FDG-PET/CT and hypoechogenic in ultrasound.

Intraoperative Sodium-Fluorescence Imaging in Peripheral Nerve Sheath Tumors (PNST)-A New Additional Promising Diagnostic Tool.

Background: Through the development and implementation of specific fluorophore filters to microscopes in 2012, sodium fluorescein (SF) is currently experiencing a remarkable renaissance in neurosurgery. The present study examines its intraoperative application during surgical removal of peripheral nerve sheath tumors (PNST) and metastases. Methods: This single-center study includes 10 cases of benign and malignant tumors as well as metastases of peripheral nerves (in total 11 PNST). Their surgical resections were all performed under microscope-based fluorescence with SF, which was administered intravenously (0.5-1.0 mg/kg body weight) during anesthesia induction. Microsurgical tumor removals were filmed and the collected data were retrospectively analyzed via ImageJ. Results: Microsurgical tumor preparation was possible under the usage of fluorophore filter. In seven histological confirmed schwannoma (n = 6 patients) tissue differentiation between tumor mass and not involved fascicles was statistically significant for the colors green and red. Schwannoma maximum mean for green reached 254.7 pixel and 179.4 pixel for red, whereas passing healthy fascicles revealed a maximum mean for green 94.91 and for red 120.76 pixel. One case of neurofibroma achieved lower amount of pixel. Similar to schwannoma, the two MPNST cases showed a strong homogeneous fluorescence (max. mean green 215 pixel and red 124.51) involving the whole nerve segment. Subcutaneous tumor remnants were visualized and therefore resected. Via fascicular nerve biopsy a B-cell lymphoma of the tibial nerve could be detected. SF led to variable stain intensities in single fascicles. The resected fascicle revealed a max mean green of 100.54 pixel, whereas surrounding fascicles came up with max. mean green of 63.0 pixel. Conclusions: Intraoperative SF visualization for PNST is feasible and of low risk. During resection of benign PNST, enhanced tissue differentiation between affected and not affected nerve segments is very useful. Tumor remnants can be detected safely and effectively. Its application during resection of malignant PNST is limited. Due to the infiltrative nature of those tumors, intraneural tissue differentiation is not possible. "Fluorescence-guided" biopsy can be regarded as an additional advantage in PNST surgery. Due to the encouraging experience in our institution SF was established as standard visualization tool in PNST surgery.

Multicentric Registry Study on Epidemiological and Biological Disease Profile as Well as Clinical Outcome in Patients with Low-Grade Gliomas: The LoG-Glio Project.

BACKGROUND: World Health Organization (WHO) grade II low-grade gliomas (LGGs) in adults are rare, and patients' mean overall survival (OS) is relatively long. Epidemiological data on factors influencing tumor genesis and progression are scarce, and prospective data on surgical management are still lacking. Because of the molecular heterogeneity of LGG, a comprehensive molecular characterization is required for any clinical and epidemiological research. Further, a detailed radiologic assessment is needed as the only established objective criterion for progressive disease. Both radiologic and molecular assessments have to be standardized to produce comparable data. The aim of the registry is to improve the evidence for surgical management of LGG patients by establishing a multicenter registry with a strong surgical and clinical focus including mandatory biobanking. METHODS: The LoG-Glio project is a prospective national observational multicenter registry that began on November 1, 2015. Inclusion criteria encompass all patients > 18 years of age with a radiologic suspicion of LGG. Patients with severe neurologic or psychiatric disorders that may interfere with their informed consent or if there is no possibility for further follow-up are excluded. Diagnosis of glioblastoma WHO grade IV isocitrate dehydrogenase (IDH) wild type leads to a secondary exclusion of patients. In addition to demographic data, results of the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire, add-on for patients with brain tumors, and National Health Institute Stroke Scale before and after surgery and during regular follow-ups are collected. At each time point a detailed recording of surgical and adjuvant treatment is performed. Radiologic assessment involves three-dimensional (3D) acquisition of T1, fluid-attenuated inversion recovery, and T2 sequences. For the final evaluation, a central detailed neuropathologic and molecular assessment of tumor samples and a radiologic evaluation of imaging sets are part of the study protocol. RESULTS: We report the first 100 consecutively registered patients for LoG-Glio. Three patients dropped out due to loss of follow-up. Of the remaining recruited patients, 8 were classified as wait and scan; 89 had surgery. Using the inclusion criteria described previously, 70 patients had an IDH-mutated glioma, 10 had miscellaneous rare LGGs, and 8 patients had an IDH wild-type WHO grade II or III glioma. CONCLUSION: The LoG-Glio registry has been successfully implemented. Applied selection criteria result in an appropriately balanced patient cohort. Short-term outcome data on epidemiology as well as the influence of current surgical techniques and adjuvant treatment on patient outcomes are expected. In the long run, the aim of the registry is to validate the new molecular-based WHO classification and the influence of the extent of resection on progression-free survival and OS. The registry provides an open platform for future research projects benefiting patients with LGG. TRIAL REGISTRATION: NCT02686229 Clinical trials.

Hypertension and multiple cardiovascular risk factors increase the risk for retinal vein occlusions: results from the Gutenberg Retinal Vein Occlusion Study.

OBJECTIVE: Although several risk factors for retinal vein occlusion (RVO) are known, what triggers RVO is unclear in many cases. We aimed to evaluate the relevance of multiple risk factors in patients with RVO. METHODS: The Gutenberg RVO Study is an observational case-control study that assessed thrombophilic, cardiovascular, ophthalmic, and drug-related risk factors in participants with RVO and the same number of matched controls. Conditional logistic regression analysis was chosen to estimate the risk of RVO due to several risk factors. RESULTS: Of 92 patients with RVO, 46 (50%) had central RVO, 31 (33.7%) had branch RVO, and 15 (16.3) had hemi-RVO. Systemic hypertension was associated with RVO [any RVO: odds ratio (OR): 1.81; 95% confidence interval (CI): 1.14-2.88; branch RVO: OR: 2.56; 95% CI: 1.08-6.10]. The most frequent combinations of risk factors were hypertension with dyslipidemia (33 of 92, 35.9%) and hyperhomocysteinemia and high levels of factor VIII (10 of 92, 10.9%). An increase in the risk sum score by one additional risk factor corresponded to ORs of 1.74 (95% CI: 1.31-2.32) for cardiovascular risk factors, 1.38 (95% CI: 1.04-1.82) for thrombophilic risk factors, and 1.43 (95% CI: 1.20-1.70) for the total number of risk factors for RVO. CONCLUSION: Cardiovascular risk factors are more important than other risk factors for the presence of RVO. The risk of RVO increased by approximately 40% with any additional risk factor and by 70% with any additional cardiovascular risk factor.