Reducing radiation exposure and costs: CT body scout views with an enhanced protocol versus conventional radiography after shunt surgery.

OBJECTIVE: Ventriculoperitoneal shunt implantation has become standard treatment for cerebrospinal fluid diversion, besides endoscopic third ventriculostomy for certain indications. Postoperative X-ray radiography series of skull, chest and abdomen combined with cranial CT are obtained routinely in many institutions to document the shunt position and valve settings in adult patients. Measures to reduce postoperative radiation exposure are needed, however, there is only limited experience with such efforts. Here, we aim to compare routine postoperative cranial CT plus conventional radiography series (retrospective arm) with cranial CT and body scout views only (prospective arm) concerning both diagnostic quality and radiation exposure. PATIENTS AND METHODS: After introduction of an enhanced CT imaging protocol, routine skull and abdomen radiography was no longer obtained after VP shunt surgery. The image studies of 25 patients with routine postoperative cranial CT and conventional radiography (retrospective arm of study) were then compared to 25 patients with postoperative cranial CT and CT body scout views (prospective arm of study). Patient demographics such as age, sex and primary diagnosis were collected. The image quality of conventional radiographic images and computed tomography scout views images were independently analyzed by one neurosurgeon and one neuroradiologist. RESULTS: There were no differences in quality assessments according to three different factors determined by two independent investigators for both groups. There was a statistically significant difference, however, between the conventional radiography series group and the CT body scout view imaging group with regard to radiation exposure. The effective dose estimation calculation yielded a difference of 0.05 mSv (two-tailed t-test, p = 0.044) in favor of CT body scout view imaging. Furthermore, the new enhanced protocol resulted in a reduction of cost and the use of human resources. CONCLUSION: CT body scout view imaging provides sufficient imaging quality to determine shunt positioning and valve settings. With regard to radiation exposure and costs, we suggest that conventional postoperative shunt series may be abandoned.

[Individualized surgical treatment of sarcomas of the extremities]. / Chirurgische Therapieindividualisierung bei Sarkomen der Extremitäten.

Sarcomas of the extremities are rare entities, the treatment of which requires special expertise. Even if the treatment of patients is always interdisciplinary, the surgical R0 resection is the key point of each curatively intended treatment. In addition to resection in sano, the aim is to preserve the extremities and function, so that defect reconstruction after resection plays a decisive role. Due to the heterogeneity of tumors as well as their localization and extent, reconstruction is always an individually adapted treatment. Modular tumor endoprostheses are often used in this context, which can be constructed according to the size of the defect. The transplantation of autologous or allogeneic bone is also frequently used alone or as an additive procedure. Patient-specific (mega)prostheses are used particularly for pelvic tumors. Defect reconstruction using scaffold-based procedures from the field of tissue engineering is being tested as a promising procedure for the future. This article provides an overview of the treatment principles for sarcomas of the extremities and their individual reconstruction options.

Clinical characterisation of women with persistent genital arousal disorder: the iPGAD-study.

Persistent Genital Arousal Disorder (PGAD) is a rare condition-mostly in women-where patients perceive prolonged genital arousal without any sexual desire or stimulation. Etiopathological considerations reach from peripheral to central issues over local disturbance of the pudendal nerve to neuropathy, psychosocial, and pharmacological theories. Since well controlled clinical studies about PGAD in conjunction with a mental and somatic health status are missing, this study is a detailed clinical investigation of PGAD patients compared to healthy controls. 26 women who fulfilled diagnostic criteria for PGAD were compared to 26 age matched healthy controls. Investigations included comparison of vegetative, gynaecological and sexual history, psychiatric features as well as a (neuro-)radiological, neurophysiological and gynaecological examination. Moreover, a detailed clinical characterisation of PGAD symptoms was performed. PGAD symptoms were mostly characterised as tingling or prickling and were permanently present. In over 80%, PGAD symptoms were located in the clitoris. Almost 70% reported radiations to other regions of the body. Most frequent trigger factors were tight clothes, mental stress, driving a car/bus/bicycle and sexual intercourse. Relieving factors were mainly distraction, relaxation, physical exercise, masturbation and swimming. In group comparisons, PGAD presented with significant higher rates of sexual dysfunctions, spontaneous orgasms, swelling of the genitals, extraordinary lubrication as well as higher rates in depression, agoraphobia, generalized anxiety disorder and lifetime panic disorder. Significantly more PGAD patients were diagnosed with restless legs symptoms. In contrast childhood traumatization, somatization disorder, suicidality, gynaecological as well as neurophysiological examination of the pudendal nerve were not different between the groups. MRI of the brain, pelvis and spinal cord was unsuspicious and incidental findings - including Tarlov cysts or pelvic venous congestion - were equally distributed among the groups. In summary, our study provides a careful characterization of women with PGAD highlighting a serious mental burden, most probably as a consequence of PGAD. With the current set of clinical investigations there was no evidence of a clear causal relationship to a specific clinical finding as it has been previously discussed. Future studies and additional techniques will have to further explore where and how in the peripheral or central nervous systems PGAD develops.

Impaired FADD/BID signaling mediates cross-resistance to immunotherapy in Multiple Myeloma.

The treatment landscape in multiple myeloma (MM) is shifting from genotoxic drugs to immunotherapies. Monoclonal antibodies, immunoconjugates, T-cell engaging antibodies and CART cells have been incorporated into routine treatment algorithms, resulting in improved response rates. Nevertheless, patients continue to relapse and the underlying mechanisms of resistance remain poorly understood. While Impaired death receptor signaling has been reported to mediate resistance to CART in acute lymphoblastic leukemia, this mechanism yet remains to be elucidated in context of novel immunotherapies for MM. Here, we describe impaired death receptor signaling as a novel mechanism of resistance to T-cell mediated immunotherapies in MM. This resistance seems exclusive to novel immunotherapies while sensitivity to conventional anti-tumor therapies being preserved in vitro. As a proof of concept, we present a confirmatory clinical case indicating that the FADD/BID axis is required for meaningful responses to novel immunotherapies thus we report impaired death receptor signaling as a novel resistance mechanism to T-cell mediated immunotherapy in MM.

Demographics and Etiology for Lower Extremity Amputations-Experiences of an University Orthopaedic Center in Germany.

Background and Objectives: Currently, the worldwide incidence of major amputations in the general population is decreasing whereas the incidence of minor amputations is increasing. The purpose of our study was to analyze whether this trend is reflected among orthopaedic patients treated with lower extremity amputation in our orthopaedic university institution. Materials and Methods: We conducted a single-center retrospective study and included patients referred to our orthopaedic department for lower extremity amputation (LEA) between January 2007 and December 2019. Acquired data were the year of amputation, age, sex, level of amputation and cause of amputation. T test and Chi² test were performed to compare age and amputation rates between males and females; significance was defined as p < 0.05. Linear regression and multivariate logistic regression models were used to test time trends and to calculate probabilities for LEA. Results: A total of 114 amputations of the lower extremity were performed, of which 60.5% were major amputations. The number of major amputations increased over time with a rate of 0.6 amputation/year. Men were significantly more often affected by LEA than women. Age of LEA for men was significantly below the age of LEA for women (men: 54.8 ± 2.8 years, women: 64.9 ± 3.2 years, p = 0.021). Main causes leading to LEA were tumors (28.9%) and implant-associated complications (25.4%). Implant-associated complications and age raised the probability for major amputation, whereas malformation, angiopathies and infections were more likely to cause a minor amputation. Conclusions: Among patients in our orthopaedic institution, etiology of amputations of the lower extremity is multifactorial and differs from other surgical specialties. The number of major amputations has increased continuously over the past years. Age and sex, as well as diagnosis, influence the type and level of amputation.

Single-Nucleotide Variants and Epimutations Induce Proteasome Inhibitor Resistance in Multiple Myeloma.

PURPOSE: Proteasome inhibitors (PI) are the backbone of various treatment regimens in multiple myeloma. We recently described the first in-patient point mutations affecting the 20S subunit PSMB5 underlying PI resistance. Notably, in vivo, the incidence of mutations in PSMB5 and other proteasome encoding genes is too low to explain the development of resistance in most of the affected patients. Thus, additional genetic and epigenetic alterations need to be explored. EXPERIMENTAL DESIGN: We performed DNA methylation profiling by Deep Bisulfite Sequencing in PSMB5, PSMC2, PSMC5, PSMC6, PSMD1, and PSMD5, a subset of proteasome subunits that have hitherto been associated with PI resistance, recruited from our own previous research, the literature, or a meta-analysis on the frequency of somatic mutations. Methylation was followed up on gene expression level and by dual-luciferase reporter assay. The KMS11 cell line served as a model to functionally test the impact of demethylating agents. RESULTS: We identified PSMD5 promoter hypermethylation and subsequent epigenetic gene silencing in 24% of PI refractory patients. Hypermethylation correlated with decreased expression and the regulatory impact of this region was functionally confirmed. In contrast, patients with newly diagnosed multiple myeloma, along with peripheral blood mononuclear cells and CD138+ plasma cells from healthy donors, generally show unmethylated profiles. CONCLUSIONS: Under the selective pressure of PI treatment, multiple myeloma cells acquire methylation of the PSMD5 promoter silencing the PSMD5 gene expression. PSMD5 acts as a key orchestrator of proteasome assembly and its downregulation was described to increase the cell's proteolytic capacity. PSMD5 hypermethylation, therefore, represents a novel mechanism of PI tolerance in multiple myeloma.

Sustained response to bevacizumab in a patient with mosaic neurofibromatosis type 2 carrying the NF2:c.784C>T p.(Arg262*) variant.

Neurofibromatosis type 2 (NF2) is a tumor predisposition syndrome characterized by the growth of schwannomas, especially bilateral vestibular schwannomas (VS), meningiomas, and ependymomas. The anti-VEGF antibody bevacizumab has shown efficacy for VS in some NF2 patients. However, there is limited data on the effect of bevacizumab on non-vestibular tumors, and on the correlation between therapy response and genotype. Here, we report on a 33-year-old patient with bilateral VS, 14 additional intracranial or spinal schwannomas, and a meningioma treated with bevacizumab, off-label in the European Union, for 2 years. The genotype of the patient was determined by mutational analysis of NF2, SMARCB1, and LZTR1 on DNA of multiple tissues. Additionally, we performed volumetric measurements of quantifiable non-vestibular tumors (n = 8) on MRI scans from 5 pre-therapeutic and 2 therapeutic years, and pure-tone audiometry of the non-deaf ear. A heterozygous NM_000268.3(NF2):c.784C>T p.(Arg262*) variant was identified in DNA from 3 schwannomas, but not in leukocyte or oral mucosa DNA, and no rare SMARCB1/LZTR1 variants were detected, establishing the diagnosis of definite NF2 mosaicism. While schwannomas had progressed with a mean annual growth rate of 38% pre-therapeutically, volume stabilization or reduction of all schwannomas along with improvement of pain and neurological deficits, including hearing impairment, were observed under 24 months of bevacizumab. In summary, this is the first report of a sustained response to bevacizumab in a patient shown to carry the frequent mosaic NF2:c.784C>T p.(Arg262*) variant. Our results may be of particular relevance to guide treatment decisions in mosaic NF2 patients harboring this variant.

Differences in the MRI Signature and ADC Values of Diffuse Midline Gliomas with H3 K27M Mutation Compared to Midline Glioblastomas.

We conducted a two-center retrospective survey on standard MRI features including apparent diffusion coefficient mapping (ADC) of diffuse midline gliomas H3 K27M-mutant (DMG) compared to midline glioblastomas H3 K27M-wildtype (midGBM-H3wt). We identified 39 intracranial DMG and 18 midGBM-H3wt tumors. Samples were microscopically re-evaluated for microvascular proliferations and necrosis. Image analysis focused on location, peritumoral edema, degree of contrast enhancement and DWI features. Within DMG, MRI features between tumors with or without histomorphological GBM features were compared. DMG occurred in 15/39 samples from the thalamus (38%), in 23/39 samples from the brainstem (59%) and in 1/39 tumors involving primarily the cerebellum (2%). Edema was present in 3/39 DMG cases (8%) versus 78% in the control (midGBM-H3wt) group (p < 0.001). Contrast enhancement at the tumor rim was detected in 17/39 DMG (44%) versus 67% in control (p = 0.155), and necrosis in 24/39 (62%) versus 89% in control (p = 0.060). Strong contrast enhancement was observed in 15/39 DMG (38%) versus 56% in control (p = 0.262). Apparent diffusion coefficient (ADC) histogram analysis showed significantly higher skewness and kurtosis values in the DMG group compared to the controls (p = 0.0016/p = 0.002). Minimum relative ADC (rADC) values, as well as the 10th and 25th rADC-percentiles, were lower in DMGs with GBM features within the DMG group (p < 0.001/p = 0.012/p = 0.027). In conclusion, DMG cases exhibited markedly less edema than midGBM-H3wt, even if histomorphological malignancy was present. Histologically malignant DMGs and midGBM-H3wt more often displayed strong enhancement, as well as rim enhancement, than DMGs without histomorphological malignancy. DMGs showed higher skewness and kurtosis values on ADC-histogram analysis compared to midGBM-H3wt. Lower minimum rADC values in DMGs indicated malignant histomorphological features, likely representing a more complex tissue microstructure.

Stereotactic biopsy of a brain lesion caused by hormographiella aspergillata.

Background: Invasive fungal infections are an increasing problem in immunosuppressed patients. In patients with the central nervous system involvement, there is a high case fatality rate. There is a very limited experience with infections caused by Hormographiella aspergillata (HA) in such cases and most often diagnosis is only confirmed postmortem. Case Description: We report the case of a 53-year-old woman with acute myeloid leukemia. After primary therapy with daunorubicin, cytarabine, and gemtuzumab ozogamicin, the patient developed pneumonia and later neurological symptoms caused by multiple gadolinium-enhancing brain lesions in magnetic resonance imaging (MRI). Stereotactic biopsy of a frontal precentral lesion was performed and revealed HA infection. The patient died in the further course secondary to cardiopulmonary problems. Conclusion: Stereotactic biopsy is a safe way to establish the diagnosis of unclear lesions such as HA infection. We recommend to perform stereotactic biopsy early in immunocompromised patients with brain lesions to guide further treatment.

Cereblon enhancer methylation and IMiD resistance in multiple myeloma.

Cereblon is the direct binding target of the immunomodulatory drugs (IMiDs) that are commonly used to treat multiple myeloma (MM), the second most frequent hematologic malignancy. Patients respond well to initial treatment with IMiDs, but virtually all patients develop drug resistance over time, and the underlying mechanisms are poorly understood. We identified an as yet undescribed DNA hypermethylation in an active intronic CRBN enhancer. Differential hypermethylation in this region was found to be increased in healthy plasma cells, but was more pronounced in IMiD-refractory MM. Methylation significantly correlated with decreased CRBN expression levels. DNA methyltransferase inhibitor (DNTMi) in vitro experiments induced CRBN enhancer demethylation, and sensitizing effects on lenalidomide treatment were observed in 2 MM cell lines. Thus, we provide first evidence that aberrant CRBN DNA methylation is a novel mechanism of IMiD resistance in MM and may predict IMiD response prior to treatment.

Temporal bone primary inverted papilloma - Case Report and review of the literature. / Invertiertes Papillom des Felsenbeins ­ Fallbericht und Literaturübersicht.

Inverted papilloma of middle ear is an extremely rare lesion of the respiratory epithelium that normally occurs in the nasal cavity and paranasal sinuses. So far less than 17 cases were described in literature. A 45-year-old patient was admitted in our Department with hearing loss, otorrhea and pulsing tinnitus on the right ear. The clinical examination showed a granulation tissue on the right eardrum. No tumor formation was seen in the nasal cavity. The MRI showed a tissue formation in the tympanic cavity with an extension in the middle cranial fossa. A mastoidectomy with antrotomy and duraplasty was performed. The histological diagnosis was inverted papilloma of the middle ear. In a second step occurred an eradication of the tumor with a subtotal petrosectomy. The etiology of the inverted papilloma of the middle ear is unknown. Our case is so far the 18nd case described.Our experience has shown that the eradication of the tumor with a subtotal petrosectomy resulted as reasonable procedure. A long-term follow-up is suggested in order to detect possible recurrence or malignant transformation.

Primary and secondary gliosarcoma: differences in treatment and outcome.

INTRODUCTION: There are only few studies comparing differences in the outcome of primary versus secondary gliosarcoma. This study aimed to review the outcome and survival of patients with primary or secondary gliosarcoma following surgical resection and adjuvant treatment. The data were also matched with data of patients with primary and secondary glioblastoma (GBM). PATIENTS AND METHODS: Treatment histories of 10 patients with primary gliosarcoma and 10 patients with secondary gliosarcoma were analysed and compared. Additionally, data of 20 patients with primary and 20 patients with secondary GBM were analysed and compared. All patients underwent surgical resection of the tumour in our department. Follow-up data, progression-free survival (PFS), and median overall survival (mOS) were evaluated. RESULTS: The median PFS in patients with primary gliosarcoma was significantly higher than in patients with secondary gliosarcoma (p = 0.037). The 6-month PFS rates were 80.0% in patients with primary and 30.0% in patients with secondary gliosarcoma. Upon recurrence, five patients with primary gliosarcoma and four patients with secondary gliosarcoma underwent repeat surgical resection. The mOS of patients with primary gliosarcoma was significantly higher than that of patients with secondary gliosarcoma (p = 0.031). The percentage of patients surviving at 1-year/2-year follow-up in primary gliosarcoma was 70%/20%, while it was only 10%/10% in secondary gliosarcoma. When PFS and mOS of primary gliosarcoma was compared to primary GBM, there were no statistically differences (p = 0.509; p = 0.435). The PFS and mOS of secondary gliosarcoma and secondary GBM were also comparable (p = 0.290 and p = 0.390). CONCLUSION: Patients with primary gliosarcoma have a higher PFS and mOS compared to those with secondary gliosarcoma. In the case of tumour recurrence, patients with secondary gliosarcoma harbour an unfavourable prognosis with limited further options. The outcome of patients with primary or secondary gliosarcoma is comparable to that of patients with primary or secondary GBM.

Leptomeningeal Metastasis: The Role of Cerebrospinal Fluid Diagnostics.

Background: Metastatic spread into the cerebrospinal fluid (CSF) represents a severe complication of malignant disease with poor prognosis. Although early diagnosis is crucial, broad spectrums of clinical manifestations, and pitfalls of magnetic resonance imaging (MRI) and CSF diagnostics can be challenging. Data are limited how CSF parameters and MRI findings relate to each other in patients with leptomeningeal metastasis. Methods: Patients with malignant cells in CSF cytology examination diagnosed between 1998 and 2016 at the Department of Neurology in the Hannover Medical School were included in this study. Clinical records, MRI findings and CSF parameters were retrospectively analyzed. Results: One hundred thirteen patients with leptomeningeal metastasis were identified. Seventy-six patients (67%) suffered from a solid malignancy while a hematological malignancy was found in 37 patients (33%). Cerebral signs and symptoms were most frequently found (78% in solid vs. 49% in hematological malignancies) followed by cranial nerve impairment (26% in solid vs. 46% in hematological malignancies) and spinal symptoms (26% in solid vs. 27% in hematological malignancies). In patients with malignant cells in CSF MRI detected signs of leptomeningeal metastasis in 62% of patients with solid and in only 33% of patients with hematological malignancies. Investigations of standard CSF parameters revealed a normal CSF cell count in 21% of patients with solid malignancies and in 8% of patients with hematological malignancies. Blood-CSF-barrier dysfunction was found in most patients (80% in solid vs. 92% in hematological malignancies). Elevated CSF lactate levels occurred in 68% of patients in solid and in 48% of patients with hematological malignancies. A high number of patients (30% in solid vs. 26% in hematological malignancies) exhibited oligoclonal bands in CSF. Significant correlations between the presence of leptomeningeal enhancement demonstrated by MRI and CSF parameters (cell count, lactate levels, and CSF/Serum albumin quotient) were not found in both malignancy groups. Conclusion: CSF examination is helpful to detect leptomeningeal metastasis since the diagnosis can be challenging especially when MRI is negative. CSF cytological investigation is mandatory whenever leptomeningeal metastasis is suspected, even when CSF cell count is normal.

Severe Progressive Multifocal Leukoencephalopathy (PML) and Spontaneous Immune Reconstitution Inflammatory Syndrome (IRIS) in an Immunocompetent Patient.

Background: Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection with JC-virus (JCV), a papova-virus, affecting mostly oligodendrocytes and the white matter of the central nervous system. Progressive Multifocal Leukoencephalopathy (PML) almost exclusively occurs in immunocompromised patients based on different underlying conditions of severe cellular immunodeficiency such as HIV/AIDS, secondary to neoplastic and autoimmune diseases, or during immunosuppressive therapy. Case presentation: We present the case of an otherwise healthy and immunocompetent patient without immunosuppressive therapy who was admitted with hemianopsia to the right side, sensory aphasia and changes of behavior. Magnet resonance imaging (MRI) and laboratory testing confirmed the diagnosis of PML, although functional tests did not show any evidence for cellular immunodeficiency. Extensive immunological tests did not reveal an apparent immunodeficiency. During symptomatic therapy the patient developed seizures which were assumed to be caused by a spontaneous immune reconstitution inflammatory syndrome (IRIS) demonstrated by MRI. We added a high dose of intravenous corticosteroids to the antiepileptic treatment and seizures ended shortly thereafter. However, the impairments of vision, behavior and language persisted. Conclusions: Our case report highlights that an apparently immunocompetent patient can develop PML and IRIS spontaneously. Therefore, MRI should be applied immediately whenever a rapid progression of PML symptoms occurs as treatment of IRIS with corticosteroids can result in a marked clinical improvement.

Severe Subarachnoid Hemorrhage Due to Fusiform Lateral Anterior (A1) Artery Perforator Aneurysm with "Spontaneous Resolution".

Perforating arteries are thin and long vessels which originate from the main cerebral arteries. Subarachnoidal hemorrhage from a perforator aneurysm is rare. Here, we report on a 70-year-old woman who presented with severe subarachnoid hemorrhage from a fusiform lateral anterior (A1) artery perforator aneurysm. Unexpectedly, digital subtraction angiography was non-diagnostic. MR imaging, however, demonstrated occlusion of the aneurysm secondary to thrombosis. Surgery was performed to remove the associated hematoma, and histopathological examination verified intraaneurysmal thrombosis.

Rare ADAR and RNASEH2B variants and a type I interferon signature in glioma and prostate carcinoma risk and tumorigenesis.

In search of novel germline alterations predisposing to tumors, in particular to gliomas, we studied a family with two brothers affected by anaplastic gliomas, and their father and paternal great-uncle diagnosed with prostate carcinoma. In this family, whole-exome sequencing yielded rare, simultaneously heterozygous variants in the Aicardi-Goutières syndrome (AGS) genes ADAR and RNASEH2B co-segregating with the tumor phenotype. AGS is a genetically induced inflammatory disease particularly of the brain, which has not been associated with a consistently increased cancer risk to date. By targeted sequencing, we identified novel ADAR and RNASEH2B variants, and a 3- to 17-fold frequency increase of the AGS mutations ADAR,c.577C>G;p.(P193A) and RNASEH2B,c.529G>A;p.(A177T) in the germline of familial glioma patients as well as in test and validation cohorts of glioblastomas and prostate carcinomas versus ethnicity-matched controls, whereby rare RNASEH2B variants were significantly more frequent in familial glioma patients. Tumors with ADAR or RNASEH2B variants recapitulated features of AGS, such as calcification and increased type I interferon expression. Patients carrying ADAR or RNASEH2B variants showed upregulation of interferon-stimulated gene (ISG) transcripts in peripheral blood as seen in AGS. An increased ISG expression was also induced by ADAR and RNASEH2B variants in tumor cells and was blocked by the JAK inhibitor Ruxolitinib. Our data implicate rare variants in the AGS genes ADAR and RNASEH2B and a type I interferon signature in glioma and prostate carcinoma risk and tumorigenesis, consistent with a genetic basis underlying inflammation-driven malignant transformation in glioma and prostate carcinoma development.

Severe Cerebral Complications Secondary to Perforation Injury of the Anterior Skull Base During Sinonasal Surgery: An Underappreciated Problem?

OBJECTIVE: Functional endonasal sinus surgery (FESS) is widely practiced and is considered a generally safe procedure. Skull base injuries occur in <1% of procedures and are typically associated with cerebrospinal fluid leaks. Rarely, skull base injuries might result in cerebral lesions. Here we present a series of 4 patients with iatrogenic perforating injuries of the anterior skull base and cerebral lesions after routine FESS. METHODS: Four patients with iatrogenic perforating cerebral lesions after routine FESS, performed at other institutions, were referred to a tertiary neurosurgery department. Within a 10-year period these procedures were performed in 3 patients as endoscopic FESS and as a microscopic FESS in 1 patient. RESULTS: There were 3 men and 1 woman. Mean age at the time of surgery was 50 years. In 3 instances (in which an endoscope was used), the ear, nose, and throat physician had noted perforation of the skull base during surgery, but it went unnoticed in 1 patient operated with the microscope. Frontal lobe hematoma occurred in all patients, and in 3 of them cerebral infarction developed secondary to injury of branches of the anterior cerebral artery. Three patients developed acute hydrocephalus. Two had rapid global brain swelling and they succumbed within days. The other 2 patients survived without apparent neurological deficits. CONCLUSIONS: Cerebral lesions during FESS still occur in contemporary surgery and they are possibly underreported. Even with prompt conservative and surgical measures, these lesions may result in catastrophic outcome. Associated vascular injuries have a worse prognosis. The only risk factor associated with lethal outcome in our series was younger age.

Contribution of QSM Imaging to the Diagnosis of the Rare Syndrome of Leukoencephalopathy with Cysts and Calcification (LCC).

We describe the typical computed tomography (CT) and magnetic resonance imaging (MRI) characteristics in a rare case of adult leukoencephalopathy with calcifications and cysts (LCC). In addition, we describe the specific findings of quantified susceptibility mapping (QSM) in this case, relate it to known histopathological findings, and point out possible advantages of this method.

Spectroscopic Characterization of Gliosarcomas-Do They Differ From Glioblastomas and Metastases?

OBJECTIVE: To evaluate the spectroscopic pattern of gliosarcomas for differentiation from glioblastomas or metastases. METHODS: H-nuclear magnetic resonance (NMR) spectroscopic intermediate echo time data of 5 patients with histologically proven gliosarcomas were compared with data of 17 metastases and 54 glioblastomas. Specialized H-NMR spectroscopy analysis software was used offline. Lipid and macromolecular resonances between 0.9 ppm and 1.4 ppm were compared besides the main metabolites using the Mann-Whitney U test. RESULTS: Gliosarcomas showed higher lipid and macromolecule resonances and a higher lipid-choline ratio compared with glioblastomas (P < 0.024 and P < 0.036). Glioblastomas showed higher creatine concentrations compared with metastases (P < 0.007) but not compared with gliosarcomas. We found no significant differences between metastases and gliosarcomas. CONCLUSIONS: Gliosarcomas may mimic metastases on H NMR spectroscopy showing high signal intensities from lipid and macromolecule resonances. This tumor type should be suspected if conventional imaging suggests an intra-axial brain neoplasm in combination with high lipids in solid tumor parts.

The sellar and suprasellar region: A "hideaway" of rare lesions. Clinical aspects, imaging findings, surgical outcome and comparative analysis.

OBJECTIVE: Apart from the "common" lesions (e.g. pituitary adenomas, Rathke's cleft cysts, meningiomas and craniopharyngiomas), there is a plethora of rare tumors or tumor-mimicking lesions in the sellar and suprasellar region (SSR). Due to a lack of characteristic imaging features, there is a dilemma in distinguishing these rare lesions from the more "common" ones preoperatively. Consequently, both diagnosis and definition of surgical goals, as well as further treatment strategies continue to be challenging for all attending physicians. To replenish the scarce data on this issue, we analysed all patients with infrequent non-adenomatous pathologies in the SSR treated in our clinic, providing a database for further studies. METHODS: A retrospective study was performed including 223 patients who were operated on lesions within the SSR at the Department of Neurosurgery, Hannover Medical School, between 2006 and 2014. The patients' charts were analysed with regard to the results of pre-/postoperative endocrinological and neuroophthalmological examinations. Preoperative T2WI were analysed with special focus on distinct growth patterns within four quadrants constituting the (supra-)sellar region. In this way, a comparative analysis between the diverse lesions regarding their clinical features, resectability and the final outcome was possible. RESULTS: After exclusion of cases with "common" lesions, a collective of 20 patients with rare lesions within the SSR was obtained. The histopathological diagnosis revealed xanthogranulomas (n=6), metastatic tumors (n=5), colloid/epidermal cysts (n=3), pilocytic astrocytomas (n=2), and one case each of gangliocytoma, lymphocytic hypophysitis or concomitant germ cell tumor/rhabdomyosarcoma. In comparison to non-infiltrative lesions, those of infiltrative nature caused more frequently diplopia and deterioration of visual acuity (4 cases; p<0,05) that were less prone to improve postoperatively. Regarding growth pattern, metastatic tumors demonstrated main growth within the third quadrant with destructive remodelling of the dorsum sellae (p<0,05). While patients harbouring large lesions (>20mm) showed a significantly worse outcome regarding hormonal deficits (p=0,0313), the overall prognosis was heavily linked to the histopathological diagnosis. CONCLUSION: The correlation of the subtle radiological findings demonstrated with the specific clinical features may facilitate the differential diagnosis of rare lesions of the SSR and aid in establishing an interdisciplinary diagnostic and therapeutic procedure for these lesions.