EPID-16. DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR IN PEDIATRIC PATIENTS: A QUANTITATIVE EXPLORATION

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi89-vi89
Author(s):  
Victor Lu ◽  
Long Di ◽  
David Daniels ◽  
Ossama Maher ◽  
Toba Niazi
Keyword(s):  
Overall Survival ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Glioneuronal Tumor ◽  
Surgical Biopsy ◽  
Duration Of Symptoms ◽  
Exact Test ◽  
Entire Cohort ◽  
Prognostic Features ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

Abstract BACKGROUND Diffuse leptomeningeal glioneuronal tumor (DLGNT), also known as oligodendrogliomatosis, is a rare neuro-oncologic condition along the neuraxis that remains poorly understood in children. We sought to describe our institutional experience and quantitively summarize the clinical survival and prognostic features of DLGNT in the pediatric population across the contemporary literature. METHODS We report four institutional cases of pediatric DLGNT diagnosed between 2000-2020 based on retrospective review of our records, and performed a comprehensive literature search for published cases from 2000 onwards to create an integrated cohort for analysis. Kaplan-Meier estimations, Fisher’s exact test, and logistic regression were utilized to interrogate the data. RESULTS Our overall integrated cohort consisted of 54 pediatric DLGNT patients, with 19 (35%) female and 35 (65%) male patients diagnosed at an average age of 6.4 years (range, 1.3-17 years) by means of surgical biopsy. Chemotherapy was used in 45 cases (83%), and mean follow-up time of 54 months (range, 3-204). Across the entire cohort, overall survival 1 month after diagnosis was 96% (95% CI 86-99%), and by 10 years was 69% (95% CI 49-82%). On multivariate analysis of complete data, chemotherapy treatment (HR=0.23, P=0.04) was statistically predictive of longer overall survival. When including limited data, longer duration of symptoms by presentation (HR=1.03, P=0.03) was statistically predictive of shorter overall survival. CONCLUSIONS This is the first quantitative study of pediatric DLGNT clinical course. More than 2-out-of-3 pediatric DLGNT patients survive beyond one decade. Chemotherapy is statistically associated with longer survival in DLGNT pediatric patients and should form the core of any treatment regimen in this setting. Early detection by means of judicious imaging and surgical biopsy for tissue diagnosis can lead to earlier treatment and likely superior outcomes.

scholarly journals Risk stratification in pediatric low-grade glioma and glioneuronal tumor treated with radiation therapy: an integrated clinicopathologic and molecular analysis

2020 ◽  
Vol 22 (8) ◽  
pp. 1203-1213 ◽  
Author(s):  
Sahaja Acharya ◽  
Jo-Fen Liu ◽  
Ruth G Tatevossian ◽  
Jason Chiang ◽  
Ibrahim Qaddoumi ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
High Risk ◽  
Risk Stratification ◽  
Risk Groups ◽  
Low Risk ◽  
Glioneuronal Tumor ◽  
Low Grade ◽  
Diffuse Astrocytoma ◽  
Prognostic Features

Abstract Background Management of unresectable pediatric low-grade glioma and glioneuronal tumor (LGG/LGGNT) is controversial. There are no validated prognostic features to guide use of radiation therapy (RT). Our study aimed to identify negative prognostic features in patients treated with RT using clinicopathologic and molecular data and validate these findings in an external dataset. Methods Children with non-metastatic, biopsy-proven unresectable LGG/LGGNT treated with RT at a single institution between 1997 and 2017 were identified. Recursive partitioning analysis (RPA) was used to stratify patients into low- and high-risk prognostic groups based on overall survival (OS). CNS9702 data were used for validation. Results One hundred and fifty patients met inclusion criteria. Median follow-up was 11.4 years. RPA yielded low- and high-risk groups with 10-year OS of 95.6% versus 76.4% (95% CI: 88.7%–98.4% vs 59.3%–87.1%, P = 0.003), respectively. These risk groups were validated using CNS9702 dataset (n = 48) (4-year OS: low-risk vs high-risk: 100% vs 64%, P < 0.001). High-risk tumors included diffuse astrocytoma or location within thalamus/midbrain. Low-risk tumors included pilocytic astrocytoma/ganglioglioma located outside of the thalamus/midbrain. In the subgroup with known BRAF status (n = 49), risk stratification remained prognostic independently of BRAF alteration (V600E or fusion). Within the high-risk group, delayed RT, defined as RT after at least one line of chemotherapy, was associated with a further decrement in overall survival (P = 0.021). Conclusion A high-risk subgroup of patients, defined by diffuse astrocytoma histology or midbrain/thalamus tumor location, have suboptimal long-term survival and might benefit from timely use of RT. These results require validation.

The transsphenoidal resection of pediatric craniopharyngiomas: a case series

2010 ◽  
Vol 5 (1) ◽  
pp. 49-60 ◽  
Author(s):  
John A. Jane ◽  
Daniel M. Prevedello ◽  
Tord D. Alden ◽  
Edward R. Laws
Keyword(s):  
Transsphenoidal Surgery ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Case Series ◽  
High Rate ◽  
Subtotal Resection ◽  
Consecutive Series ◽  
Entire Cohort ◽  
Transsphenoidal Resection

Object The majority of pediatric craniopharyngiomas are treated using a transcranial approach. Although there is an increasing acceptance of transsphenoidal resection in adults, there are few reports describing this approach in the pediatric population. The purpose of this study is to report the outcomes after transsphenoidal surgery in a consecutive series of pediatric patients with craniopharyngiomas treated at a single institution with the goal of gross-total resection (GTR). Methods Twenty-three patients with pathologically proven craniopharyngiomas were identified who were 18 years of age or less at the time of surgery. The medical records and imaging studies of the patients were retrospectively reviewed. One patient who was lost to follow-up after surgery was excluded. Results Among the 22 patients included in the study, 11 underwent transsphenoidal surgery as the primary procedure and 11 underwent transsphenoidal surgery as a secondary procedure after a previous procedure. All patients had at least some sellar component to their tumor and all had either anterior or posterior pituitary dysfunction at presentation. In the entire cohort, a GTR was achieved in 15 (68%) of 22 patients, a radical subtotal resection in 4 (18%) of 22 patients, a subtotal resection in 1 patient, and a partial resection in 2 patients. The degree of resection was higher in the primary transsphenoidal group. After a mean follow-up of 82 months, 4 patients (18%) experienced recurrence. Recurrence occurred in 13% after GTR compared with 28.5% after all other degrees of resection. Tumor recurred in 9% of the primary transsphenoidal group and in 30% of patients who had undergone other therapies prior to the transsphenoidal operation. No patient who had panhypopituitarism experienced a gain of function postoperatively, 67% developed new panhypopituitarism, and 56% experienced new diabetes insipidus. Vision improved or normalized in 9 (64%) of 14 patients presenting with visual loss. Complications included 1 death 3 weeks postoperatively, 2 CSF leaks, and new obesity in 37%. Conclusions Transsphenoidal resection of pediatric craniopharyngiomas results in a high rate of both visual improvement and GTR with a low associated risk of recurrence. The transsphenoidal approach should be considered in selected pediatric patients with craniopharyngioma, especially those with infradiaphragmatic origin.

scholarly journals Arthroscopic Management of Pigmented Villonodular Synovitis of the Hip in Children and Adolescents

2018 ◽  
Vol 6 (3) ◽  
pp. 232596711876311 ◽  
Author(s):  
S. Clifton Willimon ◽  
Tim Schrader ◽  
Crystal A. Perkins
Keyword(s):  
Pediatric Patients ◽  
Case Reports ◽  
Pediatric Population ◽  
Case Series ◽  
Pigmented Villonodular Synovitis ◽  
Villonodular Synovitis ◽  
Arthroscopic Synovectomy ◽  
Duration Of Symptoms ◽  
Pigmented Villonodular

Background: Pigmented villonodular synovitis (PVNS) is a benign proliferative synovial disorder most commonly described to affect the knee in adults. Literature describing PVNS in the pediatric population is limited to 2 small case series and a handful of single-patient case reports. Within these studies, only 2 patients with PVNS of the hip are described. Purpose: To describe the presentation, management, and outcomes of a single-center series of pediatric patients with PVNS of the hip treated with arthroscopic synovectomy. Study Design: Case series; Level of evidence, 4. Methods: A retrospective review of consecutive pediatric patients treated for PVNS at a single institution was performed. Inclusion criteria consisted of patients younger than 19 years with surgically treated PVNS of the hip. Results: Five pediatric patients with a mean age of 11.0 years were treated for PVNS of the hip from 2011 to 2016. The mean duration of symptoms from onset to surgical treatment was 247 days (range, 3-933 days). Upon review of magnetic resonance imaging (MRI) results, radiologists included PVNS in their differential in 3 patients. Seven surgeries were performed in 5 patients. All therapeutic procedures were arthroscopic synovectomies. Nodular PVNS was present in 4 patients, and diffuse disease was present in 1 patient. At a mean 32-month follow-up (range, 12-63 months), all patients were considered to be free of recurrence based on clinical examination and/or follow-up MRI. Four patients were asymptomatic and returned to all of their previous sports activities. Conclusion: Young age at the time of diagnosis is a point to be highlighted in this cohort, and symptoms may be present for many months prior to diagnosis due to the failure to consider PVNS in children. Therefore, for patients with “atypical” presentations or lack of improvement with treatment for rheumatologic, bleeding, or infectious disorders, PVNS should be strongly considered. MRI with gradient echo sequences is the diagnostic imaging study of choice. One patient with diffuse involvement and preoperative degenerative changes showed progressive changes postoperatively. This type of PVNS may have a worse prognosis, but more diffuse cases are needed before the prognosis can be determined. Arthroscopic synovectomy following a timely diagnosis of PVNS produces good outcomes in nodular cases, with no evidence of symptomatic or radiographic disease persistence among these patients.

Comparison of treatment effect and long-term outcomes for pediatric and adult Ewing sarcoma patients in British Columbia, Canada.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 11531-11531
Author(s):  
Omar Hajjaj ◽  
Lauren Corke ◽  
Caron Strahlendorf ◽  
Christine E. Simmons
Keyword(s):  
British Columbia ◽  
Overall Survival ◽  
Ewing Sarcoma ◽  
Real World ◽  
Pediatric Patients ◽  
Chemotherapy Regimen ◽  
Pediatric Population ◽  
Predictive Biomarkers ◽  
Age At Diagnosis ◽  
Dose Dense

11531 Background: Treatment of Ewing Sarcoma (EWS) is challenging. While it is known to be sensitive to chemotherapy and radiation, the number of lines of therapy available are limited. This disease affects pediatric patients (PP) more often than adults (AP), and reported outcomes are worse for AP onset EWS in the literature. It is unclear if this is due to difference in the biology of disease in AP compared to PP, or if this is due to differences in treatment approach. Furthermore, optimal treatments and real world impact of treatment is unclear in both the PP and AP populations. This study identifies the features of therapy received by AP and PP with EWS in a large, mutli-institutional cohort, and provides real world evidence for the expected outcomes in both AP and PP with EWS. Methods: A cohort study analysis of the Sarcoma Outcomes Unit database at BC Cancer was conducted to identify patients diagnosed with EWS in British Columbia from January 1, 2000 to December 31, 2018. Data on the frequency, amount, and regimen of chemotherapy were collected. Baseline Charlson comorbidity index, age at diagnosis, progression free survival and overall survival were collected. Results: 108 patients with EWS were identified, 66 AP and 42 PP Median age at diagnosis for adults was 37 (19-86) and median age for diagnosis of pediatric patients was 14 (1-18). Real world median PFS and OS for AP were 23 mos and 79 mos, and for the PP were 32 mos and NE. Five year overall survival was 54% in AP and 77% in PP. Overall, there was no difference in the number of lines of therapy received between PP and AP, but the type of therapy was more dose-dense in the PP than in the AP, (85% vs 28% for dose dense chemo). 5 year overall survival was longer for PP who received dose dense regimens compared to non-dose dense regimens (HR 0.87), but was not different in the AP receiving dose dense regimens (HR 0.95), even when controlling for comorbidities. The most common chemotherapy regimen for AP was Vincristine, Adriamycin, cyclophosphamide alternating with Ifosfamide and Etoposide q3weeks, whereas in the pediatric population the most common chemotherapy regimen was the same but alternating q2weeks. Conclusions: The treatment plans for PP with EWS were more often dose dense compared to the AP. Outcomes for PP were vastly better than for APs, despite overall similarities in the number of lines of therapy and types of agents used. Given the lack of difference between dose dense and non-dose dense regimens for APs, this is not the likely cause of difference in survival between PPs and APs. Extrapolating pediatric protocols to the adult setting may not be appropriate given the differences in outcomes. Further work to identify effective therapies and predictive biomarkers in this disease are needed, and my further identify reasons for discrepant outcomes in pediatric and adult populations.

scholarly journals The histomolecular criteria established for adult anaplastic pilocytic astrocytoma are not applicable to the pediatric population

2019 ◽  
Vol 139 (2) ◽  
pp. 287-303 ◽  
Author(s):  
Albane Gareton ◽  
Arnault Tauziède-Espariat ◽  
Volodia Dangouloff-Ros ◽  
Alexandre Roux ◽  
Raphaël Saffroy ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Pilocytic Astrocytoma ◽  
Mapk Pathway ◽  
Case Reports ◽  
Pediatric Population ◽  
Prognostic Significance ◽  
Glioneuronal Tumor ◽  
Diffuse Leptomeningeal Glioneuronal Tumor ◽  
Dna Methylation Profiling ◽  
Methylation Profiling

Abstract Pilocytic astrocytoma (PA) is the most common pediatric glioma, arising from a single driver MAPK pathway alteration. Classified as a grade I tumor according to the 2016 WHO classification, prognosis is excellent with a 10-year survival rate > 95% after surgery. However, rare cases present with anaplastic features, including an unexpected high mitotic/proliferative index, thus posing a diagnostic and therapeutic challenge. Based on small histomolecular series and case reports, such tumors arising at the time of diagnosis or recurrence have been designated by many names including pilocytic astrocytoma with anaplastic features (PAAF). Recent DNA methylation-profiling studies performed mainly on adult cases have revealed that PAAF exhibit a specific methylation signature, thus constituting a distinct methylation class from typical PA [methylation class anaplastic astrocytoma with piloid features—(MC-AAP)]. However, the diagnostic and prognostic significance of MC-AAP remains to be determined in children. We performed an integrative work on the largest pediatric cohort of PAAF, defined according to strict criteria: morphology compatible with the diagnosis of PA, with or without necrosis, ≥ 4 mitoses for 2.3 mm2, and MAPK pathway alteration. We subjected 31 tumors to clinical, imaging, morphological and molecular analyses, including DNA methylation profiling. We identified only one tumor belonging to the MC-AAP (3%), the others exhibiting a methylation profile typical for PA (77%), IDH-wild-type glioblastoma (7%), and diffuse leptomeningeal glioneuronal tumor (3%), while three cases (10%) did not match to a known DNA methylation class. No significant outcome differences were observed between PAAF with necrosis versus no necrosis (p = 0.07), or with 4–6 mitoses versus 7 or more mitoses (p = 0.857). Our findings argue that the diagnostic histomolecular criteria established for anaplasia in adult PA are not of diagnostic or prognostic value in a pediatric setting. Further extensive and comprehensive integrative studies are necessary to accurately define this exceptional entity in children.

scholarly journals Diffuse leptomeningeal glioneuronal tumor with high-grade features masquerading as tubercular meningitis—a case report

2021 ◽  
Vol 52 (1) ◽  
Author(s):  
Sameer Peer ◽  
Vivek Murumkar ◽  
Karthik Kulanthaivelu ◽  
Chandrajit Prasad ◽  
Shilpa Rao ◽  
...  
Keyword(s):  
Case Report ◽  
Early Diagnosis ◽  
Histopathological Examination ◽  
Glioneuronal Tumor ◽  
Communicating Hydrocephalus ◽  
High Grade ◽  
Endemic Region ◽  
Tubercular Meningitis ◽  
Leptomeningeal Enhancement ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

Abstract Background Diffuse leptomeningeal glioneuronal tumor (DLGNT) has been recently described in the literature. The complete neuroimaging spectrum and histopathological characteristics of this entity are yet to be elucidated. In an endemic region, diffuse leptomeningeal enhancement on neuroimaging with associated communicating hydrocephalus is usually suggestive of infective meningitis and the patients are started on empirical anti-microbial therapy. However, it is important to consider other differential diagnosis of leptomeningeal enhancement in such cases, particularly if the clinical condition does not improve on anti-microbial therapy. An early diagnosis of a neoplastic etiology may be of particular importance as the treatment regimens vary considerably depending on the underlying disease condition. Case presentation In this case report, we describe a case of DLGNT with high-grade histopathological features which was initially managed as tubercular meningitis based on the initial neuroimaging findings. Due to worsening of the clinical course and subsequent imaging findings at follow-up, a diagnosis of DLGNT was considered and subsequently proven to be DLGNT with features of anaplasia on histopathological examination of leptomeningeal biopsy specimen. Conclusion This case highlights the importance of recognizing certain subtle finding on MRI which may help in an early diagnosis of DLGNT which is crucial for appropriate treatment.

scholarly journals Paranasal Mass in a Healthy Male Toddler

2021 ◽  
pp. 014556132110079
Author(s):  
Melonie Anne Phillips ◽  
Meredith Lind ◽  
Gerd McGwire ◽  
Diana Rodriguez ◽  
Suzanna Logan
Keyword(s):  
Differential Diagnosis ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Age Group ◽  
Significant Morbidity ◽  
Healthy Male ◽  
Neck Tumors ◽  
Complete Surgical Resection ◽  
Maxilla And Mandible ◽  
Benign Mass

Head and neck tumors are rare in pediatric patients but should be kept in the differential when a patient presents with a new swelling or mass. One of these tumors is a myxoma, which is an insidiously growing, benign mass originating from the mesenchyme. They most commonly arise in the myocardium but can also develop in facial structures, particularly in the maxilla and mandible. When arising in facial structures, ocular, respiratory, and digestive systems can be affected based on local invasion. Complete surgical resection is curative but can lead to significant morbidity as well. Here, we present a case of a 15-month-old toddler presenting with a paranasal mass, which was ultimately diagnosed as a maxillary myxoma. This tumor is very rare in the pediatric population, especially in the toddler age-group, reminding clinicians to broaden the differential diagnosis when a patient’s course is atypical.

scholarly journals 1346. Implementation of a Multidisciplinary 48 Hour Antibiotic Timeout in a Pediatric Population

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S684-S684
Author(s):  
Victoria Konold ◽  
Palak Bhagat ◽  
Jennifer Pisano ◽  
Natasha N Pettit ◽  
Anish Choksi ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Pediatric Population ◽  
Intervention Group ◽  
Post Intervention ◽  
Days Of Therapy ◽  
New Antibiotics ◽  
Core Elements ◽  
Quasi Experimental ◽  
Empirical Antibiotics ◽  
The Impact

Abstract Background To meet the core elements required for antimicrobial stewardship programs, our institution implemented a pharmacy-led antibiotic timeout (ATO) process in 2017 and a multidisciplinary ATO process in 2019. An antibiotic timeout is a discussion and review of the need for ongoing empirical antibiotics 2-4 days after initiation. This study sought to evaluate both the multidisciplinary ATO and the pharmacy-led ATO in a pediatric population, compare the impact of each intervention on antibiotic days of therapy (DOT) to a pre-intervention group without an ATO, and to then compare the impact of the pharmacy-led ATO versus multidisciplinary ATO on antibiotic days of therapy (DOT). Methods This was a retrospective, pre-post, quasi-experimental study of pediatric patients comparing antibiotic DOT prior to ATO implementation (pre-ATO), during the pharmacy-led ATO (pharm-ATO), and during the multidisciplinary ATO (multi-ATO). The pre-ATO group was a patient sample from February-September 2016, prior to the initiation of a formal ATO. The pharmacy-led ATO was implemented from February-September 2018. This was followed by a multidisciplinary ATO led by pediatric residents and nurses from February-September 2019. Both the pharm-ATO and the multi-ATO were implemented as an active non-interruptive alert added to the electronic health record patient list. This alert triggered when new antibiotics had been administered to the patient for 48 hours, at which time, the responsible clinician would discuss the antibiotic and document their decision via the alert workspace. Pediatric patients receiving IV or PO antibiotics administered for at least 48 hours were included. The primary outcome was DOT. Secondary outcomes included length of stay (LOS) and mortality. Results 1284 unique antibiotic orders (n= 572 patients) were reviewed in the pre-ATO group, 868 (n= 323 patients) in the pharm-ATO and 949 (n= 305 patients) in the multi-ATO groups. Average DOT was not significantly different pre vs post intervention for either methodology (Table 1). Mortality was similar between groups, but LOS was longer for both intervention groups (Table 1). Impact of an ATO on DOT, Mortality and LOS Conclusion An ATO had no impact on average antibiotic DOT in a pediatric population, regardless of the ATO methodology. Disclosures All Authors: No reported disclosures

scholarly journals P040: Epidemiology of gun related injuries among Canadian children and youth from 2005-2013: a CHIRPP study

CJEM ◽  
2017 ◽  
Vol 19 (S1) ◽  
pp. S91
Author(s):  
C.M. Cox ◽  
S. Stewart ◽  
K.F. Hurley
Keyword(s):  
Pediatric Population ◽  
Gun Control ◽  
Simple Linear Regression ◽  
Eye Injuries ◽  
Unintentional Injuries ◽  
Exact Test ◽  
Male Predominance ◽  
Self Harm ◽  
Ed Visits ◽  
Injury Reporting

Introduction: Gun related injuries were last reported by the Canadian Hospitals Injury Reporting and Prevention Program (CHIRPP) in 2005. Since that time, Canadian gun control is less stringent and non-powder guns are increasingly popular. We aim to describe trends in pediatric gun related injuries and deaths since 2005. Methods: This is a retrospective review of CHIRPP data. The dataset included pediatric (age 0-19 years) gun-related injuries and deaths reported by participating CHIRPP emergency departments (ED) from 2005-2013. Variables were tested using Fisher’s exact test and simple linear regression. Results: There were 421 records of gun-related injuries in the database. Three hundred and twenty-nine occurred from use of non-powder guns, 85 occurred from use of powder-guns, and in 7 cases the type of gun was not clear. The number of gun-related injuries per 100 000 ED visits remained stable from 2005-2013 with a male predominance (n=366, 87%). Most injuries resulted from non-powder guns and were unintentional. Injuries most often occurred in the context of recreation (n=181) and sport (n=51). One hundred fifty four eye injuries were reported, 98% of which were from a non-powder gun. Forty-six individuals required admission to hospital and 2 died in the ED. Nine of 10 intentional self-harm injuries were inflicted with a powder gun. Conclusion: This study describes the injuries and circumstances in which pediatric gun-related injury and death occur in Canada. Unintentional injuries caused by non-powder guns were most common. Though less fatal than powder guns, non-powder guns can still cause life-altering eye injuries. This evidence can inform injury prevention programs to target specific circumstances in which the pediatric population is most vulnerable.

Longer duration of symptoms at the time of presentation is not associated with worse survival in primary bone sarcoma

2018 ◽  
Vol 100-B (5) ◽  
pp. 652-661 ◽  
Author(s):  
J. M. Lawrenz ◽  
J. F. Styron ◽  
M. Parry ◽  
R. J. Grimer ◽  
N. W. Mesko
Keyword(s):  
Overall Survival ◽  
Proportional Hazards ◽  
Bone Sarcoma ◽  
Axial Skeleton ◽  
Cox Proportional Hazards ◽  
Low Grade ◽  
Duration Of Symptoms ◽  
Negative Effect ◽  
Primary Bone Sarcoma ◽  
Primary Bone

Aims The primary aim of this study was to determine the effect of the duration of symptoms (DOS) prior to diagnosis on the overall survival in patients with a primary bone sarcoma. Patients and Methods In a retrospective analysis of a sarcoma database at a single institution between 1990 and 2014, we identified 1446 patients with non-metastatic and 346 with metastatic bone sarcoma. Low-grade types of tumour were excluded. Our data included the demographics of the patients, the characteristics of the tumour, and the survival outcome of patients. Cox proportional hazards analysis and Kaplan–Meier survival analysis were performed, and the survivorship of the non-metastatic and metastatic cohorts were compared. Results In the non-metastatic cohort, a longer DOS was associated with a slightly more favourable survival (hazard ratio (HR) 0.996, 95% confidence interval (CI) 0.994 to 0.998, p < 0.001). In all types of tumour, there was no difference in survival between patients with a DOS of greater than four months and those with a DOS of less than four months (p = 0.566). There was no correlation between the year of diagnosis and survival (p = 0.741). A diagnosis of chondrosarcoma (HR 0.636, 95% CI 0.474 to 0.854, p = 0.003) had the strongest positive effect on survival, while location in the axial skeleton (HR 1.76, 95% CI 1.36 to 2.29, p < 0.001) had the strongest negative effect on survival. Larger size of tumour (HR 1.05, 95% CI 1.03 to 1.06, p < 0.001) and increased age of the patient (HR 1.02, 95% CI 1.01 to 1.03, p < 0.001) had a slightly negative effect on survival. Metastatic and non-metastatic cohorts had similar median DOS (16 weeks, p = 0.277), although the median survival (15.5 months vs 41 months) and rates of survival at one year (69% vs 89%) and five years (20% vs 59%) were significantly shorter in the metastatic cohort. Conclusion A longer DOS prior to diagnosis is not associated with a poorer overall survival in patients with a primary bone sarcoma. Location in the axial skeleton remains the strongest predictor of a worse prognosis. This may be helpful in counselling patients referred for evaluation on a delayed basis. Cite this article: Bone Joint J 2018;100-B:652–61.

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