Resection Grade Research Articles

Demographics and outcome of histologically confirmed intracranial meningiomas

Objective: Meningiomas represent the most common intracranial extraaxial neoplasia in adults, accounting for a third of all diagnosed primary tumors of the brain. Despite decades of research, relatively little data on demographics of meningiomas exist. The aim of our study was to undertake an analysis of demographics and outcome of patients who underwent meningioma surgery over an 8-year time period at our institution. Methods: We reviewed 187 consecutive patients with histologically confirmed meningioma. Demographic data, tumor location and side, surgical resection grade, and histopathological and radiological data were collected and assessed for all patients. Furthermore, recurrence, morbidity, and mortality were evaluated. Results: Of the 187 consecutive patients undergoing meningioma resection over a period of 8 years, 131 (70.1%) were women ( p < 0.001). Meningiomas were classified as World Health Organization (WHO) grade I, grade II, and grade III in 66.8%, 31.0%, and 2.1%, of the cases, respectively ( p < 0.001). MIB-1 proliferation index was <1 in 7.5%, 1–5 in 52.9%, 6–10 in 22.4%, >10 in 11.8% of the lesions ( p < 0.001). In 82.4% of the cases, gross total resection was achieved. Recurrence occurred in 23 patients (12.3%), while overall morbidity and mortality rate was 41.2% and 7.7%, respectively. Conclusion: Based on our results, women are more than twice as likely to be affected, and the peak age is between 60 years and 70 years. Recurrence rate in our cohort was relatively low when compared to the data in the literature. The diagnosis of WHO grade II meningiomas, 31% in our cohort, is increasing since the 2007 WHO criteria have been published.

Comparative genomic analysis of driver mutations in matched primary and recurrent meningiomas.

A significant proportion of low-grade WHO grade I and higher-grade WHO grade II or III meningiomas are at risk to develop post-resection recurrence. Though recent studies investigated genomic alterations within histological subtypes of meningiomas, few have compared genomic profiles of primary meningiomas matched to their recurrences. The present study aimed to identify oncogenic driver mutations that may indicate risk of meningioma recurrence and aggressive clinical course. Seventeen patients treated for low-grade (n = 8) or high-grade (n = 9) meningioma and underwent both primary and recurrent resection between 2007–2017 were reviewed. Tumor specimens (n = 38) underwent genomic sequencing of known oncogenic driver mutations. Primary and recurrent tumors were compared using matched-pair analyses for mutational associations with clinical outcomes including functional status, progression-free survival (PFS) and overall survival (OS). Most common driver mutations included POLE and NF2. There was no enrichment for any driver mutation from primary to recurrent tumor specimen. NF2 mutant meningiomas were associated with larger tumor size (8-fold increase), presence of vasogenic edema, and higher mitotic proliferation on univariate and independently on multivariate regression (p’s < 0.05) after controlling for preoperative and tumor features. Tumors with POLE driver mutations were associated with decreased functional status at last postoperative follow-up (p = 0.022) relative to presentation. Mutation status was not associated with PFS or OS on multivariate Cox regression, but rather with grade of resection (p = 0.046) for PFS. While primary and recurrent tumors exhibited similar driver mutations within patients, the identification of driver mutations associated with clinical outcomes is crucial for guiding potential targeted treatments in recurrent meningiomas.

Perioperative (P) UGT1A1 genotype guided irinotecan (iri) dosing ‘gFOLFIRINOX’ for gastroesophageal adenocarcinoma (GEA).

4050 Background: Complete resection (R0) and pathologic response grade (PRG) correlate with long-term GEA outcome. FOLFIRINOX demonstrated efficacy in advanced GEA; gFOLFIRINOX improved tolerability. We evaluated R0, PRG and tolerability in this pilot P study. Methods: Gastric body (GB) + esophagogastric (EGJ) GEA patients (pts) with ≥T3Nx or TxN+ were enrolled &amp; treated with 4 pre + 4 postoperative biweekly cycles of gFOLFIRINOX (5-FU 2400mg/m2 over 46 hrs; oxaliplatin 85mg/m2; iri: 180mg/m2 for UGT1A1 genotype 6/6, 135mg/m2 for 6/7, 90mg/m2 for 7/7) (+ trastuzumab (T) 6mg/kg then 4mg/kg for HER2+) with prophylactic peg-filgastrim. 1°endpoint R0 resection required 36 pts to assess for a 90% R0 rate (intention to treat (ITT)) with 90% power + 0.05 alpha; ≥30/36 R0 considered positive. Co-1°endpoint was PRG (Becker); 36 pts provided 85% power with 0.05 alpha for a complete (pCR G1a) rate of 16%. 2°endpoints were safety/toxicity, PET response, &amp; R0/PRG by tumor site, histologic subtype, HER2 status, &amp; UGT1A1 genotype. We report efficacy and toxicity data from the neoadjuvant (Neo) portion of the study; postop data &amp; survival outcomes will be presented at the meeting. Results: 4 sites enrolled 36 ITT pts between 2/2014-8/2018; 75% male, median age 66 (range 27-85). All pts completed all 4 cycles of Neo therapy: 10% had any dose reduction of iri (16%/0%/25% by genotype 6/6, 6/7, 7/7); any G3+ toxicity occurred in 35% of pts (32% 6/6, 29% 6/7, 75% 7/7). G3+ toxicity in ≥5% of pts: diarrhea (17.5%; 6/6 21%, 6/7 11%, 7/7 25%), anemia (5%), vomiting (5%). Efficacy is shown in the Table. Of pts going to surgery, both R1 resections were GB linitus. PRG1(a+b) was achieved in 36% of ITT pts, 46% of intestinal type histology. Conclusions: Neo gFOLFIRINOX was tolerable with surrogate efficacy comparable to FLOT. Clinical trial information: NCT02366819. [Table: see text]

Efficacy of Detoxsan® powder on diarrhea caused by gastrointestinal neuroendocrine tumors.

BACKGROUNDPatients with neuroendocrine tumors (NETs) of the gastrointestinal tract suffer frequently from chronic diarrhea. A well characterized medical advice containing zeolite (Detoxsan® powder) was applied to patients suffered from therapy-refractory diarrhea either by its frequency or by watery stool, despite receiving standard pharmacotherapy according to the guidelines for carcinoid syndrome and comorbidities. Detoxsan® powder acts as an adsorbent and might reduce significantly symptoms of diarrhea in patients suffering from NETs.AIMTo overcome the therapy-refractory diarrhea of patients with NETs by the zeolite containing medical advice Detoxsan® powder.METHODSA total of 20 patients (12 female and 8 male) suffering from diarrhea either by its frequency or from watery stool caused by NETs were included. In each patient, the diagnosis had been confirmed by histology and somatostatin receptors expression proven by positron emission tomography/computed tomography using Ga-68-labeled somatostatin analogs. All patients received standard-of-care pharmacotherapy and were additionally given Detoxsan® powder as an extemporaneous drug containing 90% natural Cuban zeolite and 10% magnesium aspartate. Recommended daily dosage ranges between 3 g once to three times per day. Each day dose and bowel movements were documented by the patients themselves in a pre-defined table. Additionally to the bowel movements quantitative determinations of serotonin, urea, creatinine and single ions were performed within the serum of the patients by commercially available equipment used as a matter of routine in the clinic.RESULTSAll patients enrolled in this pilot study did not only suffer from NETs, but also from comorbidities and treatment-resistant diarrhea. There was insufficient control of diarrhea, most probably due to the secretion of hormones like serotonin produced by the slowly growing and highly differentiated NETs. All patients only took Detoxsan® powder as an antidiarrheal drug. In general, response effects need several days to become perceptible and require an intake of Detoxsan® powder for an extended time period or intermittently, if persisting stabilization of bowel movements could not be achieved. A correlation between NET grade, part and size of bowel resection and functionality of the tumor could not be demonstrated. Therefore, diarrhea seemed to be based on the metabolic activity of the well-differentiated NETs, which eventually led to treatment resistance. In summary, 14 out of the 20 patients (70%) declared to be very content with using Detoxsan® powder and observed a significant reduction of diarrhea, while the effective dose and intake period that resulted in a symptom relief varied individually.CONCLUSIONDetoxsan® powder is able to reduce significantly symptoms of NET-related diarrhea in the majority of patients. The duration of taking Detoxsan® powder and its dosage vary individually.

Neurological and neuropsychological outcome after resection of craniopharyngiomas.

Craniopharyngiomas are rare and benign tumors of the sellar and/or parasellar region. Primary treatment involves resection followed by adjuvant radiotherapy. While the grade of resection was frequently analyzed following surgery, the neurological outcome and especially neuropsychological deficits and quality of life have been neglected for many decades. Therefore, the authors retrospectively analyzed their patient series and prospectively assessed neuropsychological outcome and quality of life following resection of craniopharyngiomas in adults. In total, 71 patients (39 men and 32 women) with a mean age of 49 years were enrolled in the retrospective analysis. In addition, 36 of the 71 patients were included in the prospective arm of the study and underwent neurological and neuropsychological testing as well as quality of life (36-Item Short-Form Health Survey; SF-36) assessment. Factors influencing outcome were identified and correlations calculated. Resection was performed mostly using a pterional (41.6%, 47/113 surgical procedures) or bifrontal translamina terminalis (30.1%, 34/113 surgical procedures) approach. Following surgery, visual acuity was significantly improved (> 0.2 diopters) in 32.4% (23/71) of patients, or remained stable in 45.1% (32/71) of patients. During long-term follow up, 80.3% (57/71) of patients developed pituitary insufficiency, particularly involving the corticotropic and thyrotrophic axes. In total, 75% (27/36) of patients showed neuropsychological deviations in at least 1 test item. In particular, attentiveness, cognitive speed, and short-term memory were affected. Referring to the SF-36 score, quality of life was affected in both the mental and physical score in 19.4% (7/36) and 33.3% (12/36), respectively. The risk factors that were identified were a tumor volume larger than 9 cm3, tumor extension toward/into the third ventricle or the brainstem, and resection using a bifrontal translamina terminalis or left-sided approach. This study demonstrated that resection of craniopharyngiomas is frequently associated with postoperative neuropsychological deficits and hence an impaired quality of life. In addition to tumor size and extension toward/into the third ventricle or the brainstem, selection of the surgical approach may play a crucial role in the patient's neuropsychological outcome and quality of life.

Clinical features and outcomes of young patients with epithelioid sarcoma: an analysis from the Children's Oncology Group and the European paediatric soft tissue Sarcoma Study Group prospective clinical trials

BackgroundData on the clinical features, optimal treatment and outcomes of paediatric patients with epithelioid sarcoma (ES) are limited and mostly retrospective. MethodsA subset analysis of ES patients < 30 years of age enrolled on two international prospective clinical trials conducted between 7/2005 and 11/2015 was performed. Risk-adapted therapy was based on tumour diameter, histologic grade, extent of surgery and presence/absence of metastases and included surgery ± radiotherapy for all patients with the addition of ifosfamide/doxorubicin chemotherapy for intermediate-/high-risk patients. Response to therapy, event-free and overall survival and pattern and predictors of treatment failure were evaluated. ResultsSixty-three ES patients (median age 13.1 years, 52% male) were eligible. Clinical features included the following: 68% extremity, median tumour diameter 3.5 cm, 56% high histologic grade, 14% nodal metastases, 14% distant metastases. Thirty-four low-risk patients underwent surgery (n = 30) or surgery/radiotherapy (n = 4); 16 intermediate-risk and 13 high-risk patients received chemotherapy ± surgery ± radiotherapy. Partial response was observed in 11/22 (50%) patients receiving neoadjuvant therapy. Events were local recurrence (n = 10) and distant recurrence (n = 15); estimated 5-year survival was 86.4%, 63.5% and 0%, respectively, for low-, intermediate- and high-risk patients. Locoregional nodal involvement, invasive tumour, high grade and lesser extent of resection predicted event-free survival in patients without metastases. ConclusionsMost low-risk ES patients who have undergone an adequate resection fare well without adjuvant therapy. Large tumour size, high histologic grade, tumour invasiveness, inadequate tumour resection and metastatic disease predict poorer outcomes in higher risk ES patients, for whom more effective therapies are needed. Clinical trial registrationCOG ARST0332: ClinicalTrials.gov Identifier NCT00346164, EpSSG NRSTS 2005: European Union Drug Regulating Authorities Clinical Trials No. 2005-001139-31.

Clinical and Pathologic Features and Prognostic Factors for Recurrent Gliomas

To explore related factors that influence time to recurrence and prognosis of gliomas. A retrospective analysis of pathologic and clinical data of patients with glioma who underwent surgery for the first time and had a recurrence between 2009 and 2018 in West China Hospital was performed. Clinical characteristics of patients were reviewed, and survival analysis was performed to identify prognostic factors for the recurrent time. Molecules with differential changes in the paired samples were included in the survival analysis. A total of 84 patients met our inclusion requirements and were included in the study; other related factors were also considered in detail in the integrated analysis. Significant differences among O6-methylguanine-DNA methyltransferase (positive/negative), isocitrate dehydrogenase 1 (positive/negative), and Ki-67 were determined by statistical analysis of paired samples (P= 0.013, P= 0.014, P= 0.017). Univariate analysis demonstrated that Ki-67 (low expression, medium expression, high expression), initial World Health Organization grade (low or high), tumor side (left, right, middle), age (≥50 years, <50 years), and extent of resection were significantly correlated with time to recurrence (log-rank P= 0.008, P < 0.001, P= 0.015, P < 0.001, P= 0.001). Multivariate analysis results showed that Ki-67 lower expression (hazard ratio [HR]= 0.585, 95% confidence interval [CI]= 0.146-2.336, P= 0.448), medium expression (HR= 0.256, 95% CI= 0.084-0.784, P= 0.017), and high expression (HR= 1 as a reference) together with the initial World Health Organization grade (HR= 0.148, 95% CI= 0.029-0.749, P= 0.021) were independent predictive factors for glioma recurrence. This comprehensive analysis revealed that initial World Health Organization grade and Ki-67 proliferative index were independent prognostic factors that predict the time to recurrence of glioma in patients after first surgery.

Intraoperative 3D ultrasound-guided resection of diffuse low-grade gliomas: radiological and clinical results.

Extent of resection (EOR) and residual tumor volume are linked to prognosis in low-grade glioma (LGG) and there are various methods for facilitating safe maximal resection in such patients. In this prospective study the authors assess radiological and clinical results in consecutive patients with LGG treated with 3D ultrasound (US)-guided resection under general anesthesia. Consecutive LGGs undergoing primary surgery guided with 3D US between 2008 and 2015 were included. All LGGs were classified according to the WHO 2016 classification system. Pre- and postoperative volumetric assessments were performed, and volumetric results were linked to overall and malignant-free survival. Pre- and postoperative health-related quality of life (HRQoL) was evaluated. Forty-seven consecutive patients were included. Twenty LGGs (43%) were isocitrate dehydrogenase (IDH)-mutated, 7 (14%) were IDH wild-type, 19 (40%) had both IDH mutation and 1p/19q codeletion, and 1 had IDH mutation and inconclusive 1p/19q status. Median resection grade was 93.4%, with gross-total resection achieved in 14 patients (30%). An additional 24 patients (51%) had small tumor remnants < 10 ml. A more conspicuous tumor border (p = 0.02) and lower University of California San Francisco prognostic score (p = 0.01) were associated with less remnant tumor tissue, and overall survival was significantly better with remnants < 10 ml (p = 0.03). HRQoL was maintained or improved in 86% of patients at 1 month. In both cases with severe permanent deficits, relevant ischemia was present on diffusion-weighted postoperative MRI. Three-dimensional US-guided LGG resections under general anesthesia are safe and HRQoL is preserved in most patients. Effectiveness in terms of EOR appears to be consistent with published studies using other advanced neurosurgical tools. Avoiding intraoperative vascular injury is a key factor for achieving good functional outcome.

Surgical management of spinal meningiomas: focus on unilateral posterior approach and anterior localization.

OBJECTIVESpinal meningiomas (sMNGs) are relatively rare in comparison to intracranial MNGs. sMNGs localized anterior to the denticulate ligament (aMNGs) represent a surgically challenging subgroup. A high perioperative complication rate due to the need for complex surgical approaches has been described. In the present study, the authors report on their surgical experience that involves two institutions in which 207 patients underwent surgery for sMNGs. Special focus was placed on patients with aMNGs that were treated via a unilateral posterior approach (ULPA).METHODSBetween 2005 and 2017, 207 patients underwent resection of sMNGs at one of two institutions. The following characteristics were assessed: tumor size and localization, surgical approach, duration of surgery, grade of resection, peri- and postoperative complication rates, and neurological outcome. Data were compared between the subgroups of patients according to the lesion's relationship to the denticulate ligament and to surgical approach.RESULTSThe authors identified 48 patients with aMNGs, 86 patients with lateral MNGs, and 76 patients with posterior MNGs (pMNGs). Overall, 66.6% of aMNGs and 64% of pMNGs were reached via a ULPA. aMNGs that were approached via a ULPA showed reduced duration of surgery (131 vs 224 minutes, p < 0.0001) and had surgical complication rates and neurological outcomes comparable to those of lesions that were approached via a bilateral approach. No significant differences in complication rate, outcomes, and extent of resection were seen between aMNGs and pMNGs.CONCLUSIONSThe duration of surgery, extent of resection, and outcomes are comparable between aMNGs and pMNGs when removed via a ULPA. Thus, ULPA represents a safe route to achieve a gross-total resection, even in cases of aMNG.

Synthesis of Amine Terminated Pegylated Iron Oxide Nanoparticles for Prospective Astrocytoma Resection Grade Improvement

Synthesis of Amine Terminated Pegylated Iron Oxide Nanoparticles for Prospective Astrocytoma Resection Grade Improvement

AB186. 148. Concomitant primary leiomyosarcoma of jejunum and renal oncocytoma: a case report and literature review

: Leiomyosarcoma of small bowel is quite rare, comprising 2–3% of all small bowel tumours. It usually presents with non-specific symptoms and early recognition is crucial. Here we report a case of jejunal leiomyosarcoma with concurrent renal oncocytoma and review of the literature. A 51-year-old gentleman presented to emergency department with abdominal pain and right sided abdominal tenderness, with no associated systemic features. After initial workup, CT scan of abdomen and pelvis that showed a 12-cm mass in the right abdomen, and an incidental 5 cm left renal mass. After multi-disciplinary discussion, he underwent laparotomy and en bloc resection of jejunal mass with primary anastomosis, and left nephrectomy. Histology revealed an intermediate grade leiomyosarcoma with clear margins and confirmatory immunohistochemistry. Renal mass was an oncocytoma. No evidence of distant metastases was found on CT scanning. Patient recovered fully. Multi-disciplinary team recommended 6 monthly imaging follow-up and no adjuvant treatment. Leiomyosarcoma of small bowel are quite rare with only 26 cases reported between 2000 and 2012. Only after the advent of novel immunohistochemical techniques in late 1990s, their differentiation form gastrointestinal stromal tumours (GISTs) became possible. Also no case of a co-existing renal oncocytoma has been reported to date. Oncocytoma is benign and surgical resection is curative. National Comprehensive Cancer Network (NCCN) guidelines recommend complete oncological resection, intense follow-up and selective use of adjuvant chemo-radiation. The management of small bowel leiomyosarcoma is centred on surgical resection with adjuvant treatment in selected cases and requires regular follow up. Tumour grade and extent of surgical resection are main predictors of prognosis.

Clinical features, radiological findings, and treatment outcomes of high-grade lateral ventricular meningiomas: a report of 26 cases.

High-grade meningiomas in ventricles are rare, where most published series only include a few patients. A retrospective analysis was performed on the clinical features, radiological findings, and treatment outcomes of 26 patients with high-grade meningiomas in lateral ventricles who were surgically treated in our hospital between July 2008 and July 2016. A female predilection (female/male = 1.4:1) was observed with a mean age of 42.4years. Headache and/or vomiting (65.3%) were the most common initial symptom, and with symptom duration time ranging between 7days and 5years (mean 8.5months). The lateral ventricle trigone area was the most common site (80.7%). Twenty-two patients (84.6%) obtained gross total resection. The 2007 WHO classification was used to classify 22 (84.6%) meningiomas as grade II and the remaining four tumors were graded III. These tumors accounted for a recurrence rate of 38.5% (10 of 26 patients) and a mortality rate of 11.5% (3 deaths) during the follow-up periods. The recurrence rate after the gross total resection was 27.3% (6 of 22 patients). Radiotherapy was administered as an adjuvant treatment in 12 patients (46.2%) after surgery. There were 4 recurrences out of the 12 patients who received radiotherapy and 6 of the 14 patients relapsed without radiotherapy (p = 0.58). The subtotal resection was considered a risk factor for recurrence. The postoperative radiotherapy seemed to have little significance for the high-grade meningiomas in the lateral ventricles. Long-term follow-up is required, regardless of the resection grade, and reoperation is feasible for patients with recurrence.

Resection of complex pancreatic injuries: Benchmarking postoperative morbidity using the Accordion Severity Grading System

Background: This study benchmarked the severity of complications using the Accordion Severity Grading System (ASGS) in patients undergoing resection for severe pancreatic injuries. Methods: A prospective database of 461 patients with pancreatic injuries treated from 1990 to 2015 was reviewed. 130 patients with AAST grade 3, 4 or 5 pancreatic injuries underwent resection (pancreatoduodenectomy (PD) n=20, distal pancreatectomy n=110), including 30 who had damage control laparotomy (DCL) and later definitive surgery. Uni- and multivariate logistic regression analysis was applied. Results: Overall 238 complications occurred in 95 (73%) patients of which 73% were ASGS grades 3-6. 19 patients (14.6%) died. Patients more likely to have complications after pancreatic resection were older, had RTS <7.8, were shocked on admission, had grade 5 pancreatic injuries with vascular and duodenal injuries, required a DCL, received a larger blood transfusion, had a PD and repeat laparotomies. Applying univariate logistic regression analysis, mechanism of injury, RTS <7.8, shock on admission, DCL, increasing AAST grade and type of pancreatic resection were significant variables for complications. Multivariate logistic regression analysis however showed that only age and type of pancreatic resection (PD) were significant. Conclusion: ASGS-assessed morbidity after pancreatic resection for trauma was high. This detailed outcome analysis serves as a reference for future institutional comparisons.

Resection of complex pancreatic injuries: benchmarking postoperative morbidity using the accordion severity grading system

Background: This study benchmarked the severity of complications using the Accordion Severity Grading System (ASGS) in patients undergoing resection for severe pancreatic injuries. Methods: A prospective database of 461 patients with pancreatic injuries treated from 1990 to 2015 was reviewed. 130 patients with AAST grade 3, 4 or 5 pancreatic injuries underwent resection (pancreatoduodenectomy (PD) n=20, distal pancreatectomy n=110), including 30 who had damage control laparotomy (DCL) and later definitive surgery. Uni- and multivariate logistic regression analysis was applied. Results: Overall 238 complications occurred in 95 (73%) patients of which 73% were ASGS grades 3-6. 19 patients (14.6%) died. Patients more likely to have complications after pancreatic resection were older, had RTS <7.8, were shocked on admission, had grade 5 pancreatic injuries with vascular and duodenal injuries, required a DCL, received a larger blood transfusion, had a PD and repeat laparotomies. Applying univariate logistic regression analysis, mechanism of injury, RTS <7.8, shock on admission, DCL, increasing AAST grade and type of pancreatic resection were significant variables for complications. Multivariate logistic regression analysis however showed that only age and type of pancreatic resection (PD) were significant. Conclusion: ASGS-assessed morbidity after pancreatic resection for trauma was high. This detailed outcome analysis serves as a reference for future institutional comparisons.

A management dilemma: adjuvant radiotherapy after gross total resection of atypical meningioma

Weber et al . recently published initial results of a prospective phase II trial of patients with grade II and III meningiomas treated with adjuvant radiotherapy (RT) after subtotal or gross total resection (GTR) (1). This initial publication of EORTC 22042 reports outcomes of the 56 enrolled patients with grade II meningiomas who underwent GTR followed by adjuvant RT, and shows favorable 3-year progression free survival (PFS) with moderate toxicity. Together with RTOG 0539 intermediate risk cohort (2), these two trials provide the only prospective data on outcomes of adjuvant RT for atypical meningiomas. Despite meeting the primary endpoints for both of the aforementioned trials, adjuvant RT remains controversial in the management of gross totally resected grade II meningiomas.

Radiation Therapy in Extracranial Chondrosarcomas: A Multicenter French Sarcoma Group and Rare Cancer Network Study

Chondrosarcomas (CHS) are considered as radio-resistant tumors requiring high-dose radiation therapy (RT). Skull base CHS are a validated indication for protontherapy. CHS from other anatomic sites are systematically contra-indicated for RT on an institution-dependent way, regardless of grade and quality of resection. The main objective of our study was to assess the impact of RT in extracranial CHS. Patients were extracted from the CONTICABASE, the database from the French Sarcoma Group (where all samples are reviewed for proper diagnosis), and additional data from the Rare Cancer Network were considered. All consecutive non metastatic extracranial CHS treated in a curative intent were included, regardless of RT. Between 2005 and 2014, 226 patients (125 men, 55%; median age 52 [range 11-90]), met inclusion criteria. Performance status was 0 in 50%, 1 in 46% and 2 in 4% of patients. ASA operability score was 0-1 in 65%, 2 in 20%, or >2. Primary site was a limb in 57%, thorax/spine in 24%, head and neck in 10% and abdomen/pelvis in 9%. It affected bone in 58% or soft-tissues in 42%. Grade was 1, 2, and 3 in 43%, 42%, and 15%; respectively. CHS was myxoïd, mesenchymal, dedifferentiated, periosteal in 28%, 10%, 5%, or 4%, respectively and NOS/other in most cases (53%). Surgery was performed in 89% with en bloc resection, and 76% of patients were considered as R0. RT was performed in 85 (37,6%) patients combined to surgery or as exclusive local treatment. Among irradiated patients 46 (54%), 18 (21%), 15 (18%) and 6 (7%) were grade 2, 1, 3 and unclassified respectively. Mean dose was 54Gy (range 26-70Gy). Three-dimensional RT, Intensity-modulated RT and protontherapy were used in 71%, 24% and 5% respectively. Thirty-nine (17%) patients had adjuvant chemotherapy. With a median follow-up period of 54,5 months (range 1-532), there were 84 (37,1%) relapses including 49 local relapses within median 48 months, 13 regional within 71 months, and 65 metastatic ones within 42 months. At last follow-up, 56% of the patients were alive without disease; and 11% and 29% of the patients were alive or dead with disease, respectively. Of the 62 locoregional relapsing patients, there were 15 (24%) in the irradiated vs 47 (76%) in the non-irradiated patients. Late grade 3 or more toxicity was noted in 3 patients. Extracranial chondrosarcoma is a rare entity but this large database study seems to show that these patients have a better outcome when RT (postoperative or exclusive) is administered.

Predictors of recurrence and postoperative outcomes in patients with non-skull base meningiomas based on modern neurosurgical standards

Abstract Background Advances in neurosurgical techniques and neuroimaging resolution questions the modern-day reliability of the Simpson grade for predicting meningioma recurrence. Therefore, we evaluated the reliability of predictors for recurrence and outcomes in detail in patients with non-skull base meningiomas (NSBMs). Methods We retrospectively analyzed data from consecutive 175 NSBMs underwent surgical resection. We performed Kaplan–Meier analyses of recurrence-free survival (RFS) according to Simpson and World Health Organization (WHO) grades. Predictors of RFS and clinical deterioration were estimated by univariate and multivariate analyses. Correlation between the Simpson grade and change in Karnofsky Performance Scale scores was assessed by Fisher's exact test. Results Log-rank tests revealed significant correlations of both the Simpson and WHO grades with RFS for the overall cohort, convexity, and falx/tentorium meningioma. Unlike patients undergoing Simpson grade I and II resections, RFS in patients with WHO grade I and II/III tumors differed significantly from the early postoperative stage. Multivariate analysis identified tumor size, Simpson grade, and MIB-1 labeling index as significant predictors of RFS. Clinical deterioration was more frequent among patients undergoing less aggressive resection. Tumor location was the only significant predictor of clinical deterioration. Conclusions Our findings indicate that tumor size, Simpson and WHO grades, and MIB-1 labeling index are significant predictors of NSBM recurrence. Moreover, the risk of recurrence markedly decreases within the follow-up duration of 80 months. Aggressive resection appears to minimize the risk of recurrence without evidence of clinical deterioration. Follow-up schedules should be based on the WHO grade and extent of resection.

Surgical resection for recurrent retroperitoneal leiomyosarcoma and liposarcoma

BackgroundRetroperitoneal soft tissue sarcomas (STS) include a number of histologies but are rare, with approximately 3000 cases in the USA per year. Retroperitoneal STS have a high incidence of local and distant recurrence. The purpose of this study was to review the University of Maryland Medical Center’s (UMMC) treatment experience of retroperitoneal STS, where the patient population served represents a diverse socioeconomic and ethnic catchment.MethodsIRB approval was obtained. We constructed a de-identified database of patients diagnosed with retroperitoneal liposarcomas (LPS) or leiomyosarcomas (LMS) treated at UMMC between 2000 and 2013. A total of 49 patients (Pts) with retroperitoneal STS met our eligibility criteria. Kaplan-Meier plots were used to graphically portray progression-free survival (PFS) and overall survival (OS). The log-rank test was used to compare time-to-event distributions.ResultsThe median OS for all patients (Pts) was 6.3 years, and the 2-year OS rate was 81%. The median PFS for all Pts was 1.8 years, and the 2-year PFS rate was 45%. There was no difference in OS and PFS among LMS and LPS patients; the median OS for LMS was 3.8 years vs. LPS 6.4 years (p = 0.33), and the median PFS for LMS was 1.2 years vs. LPS 2.5 years (p = 0.28). There was a significant difference between histology and race (p = 0.001). LPS were primarily Caucasian 86% vs. 14% black, whereas LMS were primarily black 52% vs. 33% Caucasian. OS was influenced by functional status, gender, American Joint Committee on Cancer (AJCC) stage, grade, histology, tumor size, and extent of resection. PFS was influenced by AJCC stage, grade, and extent of resection. Neither adjuvant chemotherapy (1 Pt) nor neoadjuvant/adjuvant radiation therapy (18 Pts) influenced OS or PFS. There was a non-significant difference that Pts who could undergo resection of local recurrence had improved 2-year OS, with 100% LMS and LPS compared to 2-year OS of 71% (LMS) and 78% (LPS) not undergoing resection of local recurrence.ConclusionsThis study suggests a higher incidence of leiomyosarcoma in the African-American population. This study confirms the prognostic importance of grade, tumor size, AJCC stage, histology, and extent of resection in patient outcomes, at a large substantially diverse academic medical center. Future research into the biological features of liposarcoma and leiomyosarcoma Pts imparting these characteristics will be important to define.

Outcomes of tumour related epilepsy in a specialised epilepsy surgery unit

IntroductionSeizures are an important cause of morbidity in patients with low grade gliomas with approximately 40% of cases drug resistant. The pathogenesis is quite complex and poorly understood. The treatment aims vary between almost purely epilepsy considerations and those that are primarily oncologic. AimTo determine whether patients who present with seizures and are found to have a low grade glioma have better outcomes when managed through a specialized epilepsy unit compared to the general neurosurgical service. MethodsA review of the prospectively collected database was performed over a 10 year period to identify 48 adult patients who present with a seizure and were subsequently found to have a low grade glioma. These patients were analysed with respect to management through the specialized epilepsy service or the general neurosurgical service. The primary outcome was Engel classification between the two groups. Secondary outcomes included recurrence, postoperative deficits, delay to surgery, histology, grade and extent of resection. OutcomesThe patients managed through the epilepsy service had significantly higher rate of favourable Engel outcomes (I and II) compared to the general neurosurgery service (OR: 13.2, 95% CI: 1.239–140.679; P = 0.033). The epilepsy surgery group patients had a significantly higher delay to surgery (P < 0.001). The patients in the epilepsy service had a significantly higher resection ratio compared to the general neurosurgery service (73% vs 127%, P = 0.014). Rates of recurrence were not different between the two groups. ConclusionPatients with tumour related epilepsy who undergo an intensive presurgical evaluation may obtain better seizure related outcomes.

Preoperative and postoperative prediction of long-term meningioma outcomes.

BackgroundMeningiomas are stratified according to tumor grade and extent of resection, often in isolation of other clinical variables. Here, we use machine learning (ML) to integrate demographic, clinical, radiographic and pathologic data to develop predictive models for meningioma outcomes.Methods and findingsWe developed a comprehensive database containing information from 235 patients who underwent surgery for 257 meningiomas at a single institution from 1990 to 2015. The median follow-up was 4.3 years, and resection specimens were re-evaluated according to current diagnostic criteria, revealing 128 WHO grade I, 104 grade II and 25 grade III meningiomas. A series of ML algorithms were trained and tuned by nested resampling to create models based on preoperative features, conventional postoperative features, or both. We compared different algorithms’ accuracy as well as the unique insights they offered into the data. Machine learning models restricted to preoperative information, such as patient demographics and radiographic features, had similar accuracy for predicting local failure (AUC = 0.74) or overall survival (AUC = 0.68) as models based on meningioma grade and extent of resection (AUC = 0.73 and AUC = 0.72, respectively). Integrated models incorporating all available demographic, clinical, radiographic and pathologic data provided the most accurate estimates (AUC = 0.78 and AUC = 0.74, respectively). From these models, we developed decision trees and nomograms to estimate the risks of local failure or overall survival for meningioma patients.ConclusionsClinical information has been historically underutilized in the prediction of meningioma outcomes. Predictive models trained on preoperative clinical data perform comparably to conventional models trained on meningioma grade and extent of resection. Combination of all available information can help stratify meningioma patients more accurately.