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There remains an unmet need to identify molecular biomarkers in Ewing sarcoma (ES). We sought to assess the influence of the OData of advanced ES patients, treated with IT were retrospectively collected. Patients were required to have progression after prior VDC-IE.A total of 20 ES patients underwent

Irinotecan and temozolomide (IT) is a widely used regimen for relapsed Ewing sarcoma (ES), although studies are largely limited to paediatric populations. We retrospectively reviewed paediatric (

Metastatic carcinomas in the nasopharynx are a rarity. We report a case of a 54-year-old male patient who presented with a history of recurrent epistaxis. On evaluation, a mass in the right Rosenmüller fossa was detected, which was biopsied and diagnosed as a poorly differentiated adenocarcinoma, immunoreactive for thyroid transcription factor-1, consistent with metastatic pulmonary adenocarcinoma. Fluorodeoxyglucose–positron emission tomography/computed tomography (FDG-PET/CT) scan confirmed the presence of a mass in the upper lobe of the left lung, proven pathologically to be a poorly differentiated pulmonary adenocarcinoma, with an immunoprofile similar to the nasopharyngeal tumor. The patient underwent palliative chemotherapy, and was then shifted to immunotherapy. He is currently alive and disease free 50 months after the initial diagnosis was made. The unusual presentation of a metastatic carcinoma in the nasopharynx will be discussed, along with a review of literature. The role of immunotherapy in cancer control and greater longevity will also be presented.

AIM: To assess the impact of delay in local control on survival outcomes of Ewing sarcoma (ES) patients. BACKGROUND: The cornerstone of therapy of localized ES includes chemotherapy and local control with surgery or radiotherapy. We sought to assess the impact of delay (>15 weeks) in timing of local control on survival outcomes of ES patients. METHODS: Data of consecutive patients with primary non-metastatic ES of the extremities, treated at a single institution were collected. The impact of delay of timing for local control, demographics, and disease characteristics on overall survival (OS) was analyzed. RESULTS: A total of 43 patients with ES of the extremity were included. All patients received neoadjuvant chemotherapy. Local control was by surgery in 36 patients and definitive radiation in 7. A total of 16 patients had delay in local control. At a median follow of up of 48 months, patients with delay in local control had significantly inferior OS compared to those with optimal local control timing (5-year OS 56% vs. 80%, respectively, p = 0.044). Other factors that predicted inferior OS included definitive radiation as opposed to definitive surgery (5-year OS 25% vs. 79%, respectively, p = 0.041) and tumor necrosis

Purpose To compare the outcomes of extraskeletal and skeletal Ewing sarcomas treated with standard chemotherapy protocol. Methods We retrospectively collected data on primary localized skeletal and extraskeletal ES patients. Demographics and disease characteristics were compared between the two groups. The influence of presentation (skeletal vs. extraskeletal) on overall survival (OS) and local recurrence-free survival (LRFS) was assessed and compared by the log-rank test. Results A total of 120 patients were included; 29 (24%) had extraskeletal and 91 (76%) had skeletal ES. All patients received vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (VDC-IE) chemotherapy, with a plan for local control at week 12. At a median follow-up of 38 months, there was no difference in OS between skeletal and extraskeletal ES; 5-year OS 70% and 67% respectively, p = 0.96. Patients with extraskeletal ES had inferior 5-year LRFS compared to skeletal ES; 74% vs. 83%; p = 0.042. Local recurrence occurred at a higher frequency in the extraskeletal group; 28% vs. 11%, p = 0.034, although more extraskeletal patients received adjuvant radiotherapy; 73% vs. 36%, p = 0.01. Among patients who underwent surgery (n = 76), there was no difference in R0 resection rate (skeletal: 89%, extraskeletal: 86%, p = 0.52, or good ( ≥ 90%) tumor necrosis; skeletal: 54%, extraskeletal: 38%, p = 0.31. Conclusion Patients with localized extraskeletal ES have comparable OS outcomes to patients with skeletal ES utilizing the standard VDC-IE chemotherapy. However, extraskeletal patients are at significantly higher risk for local recurrence.

e23507 Background: There remains an unmet need to identify prognostic and predictive molecular biomarkers in advance Ewing sarcoma (ES). We sought to assess the influence of MGMT promoter methylation status on response rate, time to progression (TTP), and overall survival (OS) following salvage irinotecan and temozolomide (IT) chemotherapy. Methods: Data of advanced ES patients, treated with IT chemotherapy from Jan, 2014 to Jan, 2020 were retrospectively collected. Patients were required to have previously received and progressed after vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (VDC-IE). MGMT promoter methylation status was assessed by Methylation Sensitive Restriction Enzyme quantitative-PCR (MSRE-qPCR) on non-decalcified Formalin-fixed paraffin embedded (FFPE) tissue using internally developed primers and the OneStep qMethyl Kit (ZYMO RESEARCH CORP). Responses were assessed by response evaluation criteria in solid tumors (RECIST v. 1.1). TTP and OS were assessed by the Kaplan-Meier method. Survival comparisons were performed by the Log-rank test. Results: Herein, we present data of the preliminary analysis of the first 18 patients who underwent MGMT promoter methylation testing. Patients had a median age of 18 years (range: 5-34 years), and were predominantly male ( n=11; 61%). The primary tumor was located in the pelvis in 9 patients (50%), femur in 3 (17%), tibia in 2 (11%), kidney in 2 (11%), chest wall in one (6%), and scapula in one patient (6%). IT was given in a second ( n=15) or third-line setting ( n=3). At the time of initiation of IT, 13 patients (72%) had distant metastasis, and 5 (28%) had unresectable local progression in the pelvis ( n=4) or chest wall ( n=1). The mean percentage of MGMT promoter methylation was 29% (range: 3-98%). Five patients (28%) had methylated MGMT promoter, whereas the remaining had partially methylated ( n=6; 33%) or unmethylated ( n=7;39%) promoter. Five patients (28%) had objective response, with no observed difference according to MGMT promoter methylation ( p=0.58 for comparison between methylated and unmethylated/ partially methylated). Median TTP was 4.9 and 2.2 months for patients with methylated and partially methylated/ unmethylated MGMT respectively; p=0.76. The corresponding median OS was 69.4 and 14.3 months in favor of the methylated group; p=0.3. Conclusions: This preliminary data suggests a possible prognostic role for MGMT promoter methylation, with a markedly extended median OS for the methylated group. Nevertheless, the median OS difference did not reach statistical significance in this preliminary analysis. We plan to report data of the final analysis after finalizing MGMT testing for the rest of our study patients.

A 37-year-old female with unusual presentation of metastatic non-small cell lung cancer (NSCLC), as she presented with symptoms related to clival bone metastases. This case highlights the unpredictable presentations and the variety of metastatic sites of which metastatic NSCLC could be presented.

The aim of this study was to report the outcomes and prognostic factors for a cohort of patients with osteosarcoma who presented with metastasis at initial diagnosis. Data of consecutive patients with osteosarcoma and metastatic disease at initial presentation, who were treated and followed up at a single institution, were retrospectively reviewed. The effect of potential factors on overall survival (OS) was analyzed through univariate and multivariate analysis. Between January, 2000 and March, 2013, a total of 135 patients with osteosarcoma, of whom 21 (16.0%) had distant metastasis at initial presentation, were diagnosed and treated at our center and were included in this analysis. The patients were treated with a strategy that integrates multi-agent chemotherapy and resection of all sites of gross disease whenever feasible. The 5-year OS and event-free survival (EFS) were 23.0% and 11.0%, respectively. The factors associated with inferior OS in the univariate analysis included the osteoblastic variant, extrapulmonary metastasis and failure to achieve complete remission. In the multivariate analysis, the osteoblastic variant [hazard ratio (HR)=4.83, 95% confidence interval (CI): 1.16–20.0, P=0.038] and extrapulmonary metastasis (HR=5.0, 95% CI: 1.40–17.94, P=0.018) were the only independent prognostic factors. The current outcomes of patients with osteosarcoma and metastasis at initial diagnosis remained poor. The osteoblastic subtype and extrapulmonary metastatic sites predicted poor survival in our series. To the best of our knowledge, this is the first report of the correlation between the histological subtype and survival for patients with metastatic disease at initial presentation; therefore, confirmation in future studies is required.

11027 Background: Extra-skeletal ewing sarcomas (ES) are rare, and data on outcomes following standard ES chemotherapy protocols are very limited. Methods: We retrospectively collected data on skeletal and extra-skeletal ES patients who presented with localized disease from January, 2006 to June, 2018. Disease and treatment characteristics were compared between the two groups by the chi-square test. Overall survival (OS) and local recurrence free survival (LRFS) were estimated by the Kaplan-Meier method and compared by the Log-rank test. Results: A total of 120 patients were included. Twenty-nine (24%) had extra-skeletal and 91 (76%) had skeletal ES. Location was in the extremity in 51 (43%) and non-extremity in 69 (57%). For extra-skeletal ES, tumors originated from soft tissue in 23 (79%), and viscera in 6 (21%). All patients received standard vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (VDC-IE), with a plan for local control at week 12 of the protocol. Local control was by surgery in 76 (63%) and radiotherapy in 44 (37%). At a median follow up of 38 months, there was no difference in 5-year OS between extra-skeletal and skeletal ES patients (67% and 70% respectively, p = 0.96). Patients with visceral ES had inferior 5-year OS compared to all others (soft-tissue extra-skeletal and skeletal ES); 33% vs. 72%; p = 0.013. Resectability rate was not different between extra-skeletal and skeletal ES patients (54% and 69% respectively, p= 0.11). Furthermore, among patients who underwent surgery, there was no difference between extra-skeletal and skeletal ES patients in R0 resection rate (86% and 89% respectively, p= 0.52) and poor ( < 90%) tumor necrosis rate (62% and 46% respectively, p= 0.31). However, more local recurrences (28% vs. 10%, p= 0.034) and inferior 5-year LRFS (74% vs. 83%; p = 0.042) were observed in the extra-skeletal group, although more extra-skeletal patients received adjuvant radiotherapy; 11 (73%) vs. 21 (36%), p = 0.01. Conclusions: Patients with localized extra-skeletal ES have OS outcomes that are comparable to skeletal ES treated with standard VDC-IE chemotherapy. However, extra-skeletal ES patients are at significantly higher risk of local recurrence.

e22500 Background: Irinotecan and temozolomide (IT) is a widely used regimen for relapsed Ewing Sarcoma (ES), although studies are largely limited to the paediatric population. We aimed to compare the tolerability and efficacy of IT between paediatric and adult patients. Methods: We retrospectively reviewed paediatric (< 18 years) and adult patients treated with salvage IT chemotherapy at two institutions from March, 2010 to June, 2018. Toxicities were graded according to common terminology criteria of adverse events (CTCAE v. 4.03) and compared using the Chi Square test. Responses were interpreted by Response Evaluation Criteria in Solid Tumors (RECIST). The Kaplan-Meyer method was used to estimate progression free survival (PFS); survival comparisons were carried out by the Log-rank test. Results: Fifty-three patients were included ( n= 16 paediatric; n= 37 adult). Median age was 20 (range, 5 – 45 years). A total 236 IT cycles were delivered (median = 4, range:1-7). IT was given as second-line ( n= 34; 64%) or ≥third-line ( n= 19; 36%). There was no difference in ≥grade 3/4 haematologic toxicity between paediatric and adult patients (31% vs. 35% respectively; p= 0.76), whilst febrile neutropenia was observed in two (4%) patients (one adults and one paediatric). The frequency of diarrhoea of any grade was similar (38% in each group). Of 43 patients assessable for response, 12 (28%) had objective response (1 CR, 11 PR), 19 (44%) had disease progression and 12 (28%) had stable disease. Objective response rate did not differ between the two groups (36% in paediatrics vs. 25% in adults; p = 0.47). Median PFS was superior in paediatrics vs. adults (7.4 vs. 2.1 months, p = 0.001). Superior PFS for the paediatric population was observed in both, the second-line (6.2 vs. 2.2 months; p= 0.060) and ≥third-line setting (7.4 vs. 1.2 months, p= 0.014). Conclusions: IT is an effective salvage regimen for ES, with favourable toxicity and equally observed objective responses in paediatric and adult populations. The observed superior PFS for the paediatric cohort requires further confirmation in a larger prospective study.

OBJECTIVES: Pulmonary metastasectomy for sarcoma is a widely accepted practice. Nevertheless, no previous studies has been reported the outcomes following metastasectomy compared with chemotherapy for patients with resectable and isolated pulmonary metastases. Our aim is to compare these modalities for the subset of patients with resectable metastases. Furthermore, the outcomes for patients with unresectable lung metastases are reported. METHODS: Sarcoma patients with isolated lung metastases were identified and their computed axial tomography scans were reviewed by a thoracic surgeons’ committee. Patients were divided into three groups: A: patients with resectable metastases treated with metastasectomy (n= 29), B: patients with resectable metastases who received systemic therapy (n= 17) and C: patients with unresectable metastases (n= 25). Survival outcomes were plotted and compared through log-rank test for osteosarcoma and non-osteosarcoma patients. RESULTS: Seventy-one patients (32 with osteosarcoma and 39 with non-osteosarcoma) were eligible. Progression-free survival (PFS) was superior in patients who belonged to Group A compared with Groups B and C (8.0, 4.3 and 2.2 months, respectively, P= 0.0002). Furthermore, overall survival (OS) was superior in patients who belonged to Group A compared with Groups B and C (39.6, 20.0 and 7.8 months, respectively, P< 0.0001). A subanalysis for osteosarcoma patients showed superior PFS and OS for Group A vs B (median PFS 21.6 and 3.65 months, respectively, P= 0.011 and median OS 34.0 and 12.4 months, respectively, P= 0.0044). For non-osteosarcoma patients, there were no such significant survival differences between Groups A and B. Overall, patients who belonged to Group A had significantly lower mean percentage of their follow-up time spent admitted at hospital, and a trend towards lower requirements for home oxygen therapy. CONCLUSIONS: Pulmonary metastasectomy is associated with improved survival of osteosarcoma patients with resectable lung metastases. For non-osteosarcoma patients, the survival benefit of metastasectomy over chemotherapy is uncertain and warrants further evaluation. Patients with unresectable metastases have poor prognosis.

A 22-year-old female patient, with synovial sarcoma of the left thigh, was treated with marginal resection and adjuvant chemoradiotherapy. Two years later, she developed sub-pleural metastatic nodules that were resected. Follow-up chest CT-scans revealed small yet new left lower lobe sub-pleural nodules, which were observed closely. These lesions progressed over time and, 20 months later, the patient underwent pulmonary metastasectomy. Histopathology examination of the lung tissue showed changes consistent with a sarcoid-like reaction. There were no systemic manifestations of sarcoidosis. To date, there have been no reported cases of sarcoid-like reaction associated with synovial sarcoma. Reporting this case, therefore, may help to improve our overall understanding of the relationship between malignancy and sarcoid-like reactions in the lungs.

Synovial sarcoma is a rare type of sarcoma with poor prognosis. Data on relevant prognostic factors are inconsistent. The objective of this study was to determine the independent prognostic factors that govern local recurrence, distant metastasis and overall survival. A retrospective analysis of 51 patients treated for localized synovial sarcoma at a single institution by a multidisciplinary/multimodality approach over a period of 12 years. Patients, tumor and treatment characteristics were collected including age, sex, tumor site, location, depth, size, status of margins, histology subtype and involvement of bone or lymph nodes. Type of surgery, adjuvant chemotherapy and radiotherapy were also examined. The endpoints analyzed were local recurrence-free survival (LRFS), metastasis-free survival (MFS) and overall survival (OS). Median age of patients was 26 years (range 3–64 years) with 73 % above the age of 20 year. All patients received surgery, 57 % received adjuvant radiotherapy and 45 % adjuvant chemotherapy. The median survival was 111 months, and 5-year OS was 73 %. Deep seatedness of the tumor was linked to OS as the only independent risk factor. Twelve patients had local recurrence, and the 5-year LRFS was 61 %. Multivariate analysis determined negative margins and adjuvant radiotherapy as independent predicting factors for LRFS. Five-year MFS was 48 %; multivariate analysis identified monophasic subtype and site other than lower extremity as the only independent factors associated with inferior MFS. The most important factors that govern LRFS and MFS are negative margins and adjuvant radiotherapy for LRFS and tumor histology and site for MFS, while deep seatedness of the tumor is the sole independent factor that governs OS.

Metastatic synovial sarcoma (SS) is associated with poor prognosis. Nevertheless, data addressing prognostic factors for patients with metastatic disease are very limited. We identified patients with SS who presented with or developed metastases at our institution from January 2000 to October 2012. Potential demographic and disease-related factors were analyzed for possible influence on survival. A second analysis for patients who received chemotherapy was undertaken to assess efficacy of first-line regimens. Thirty-three patients with metastatic SS were included in this analysis. The following factors were associated with inferior overall survival (OS); age >30 years, presence of extra-pulmonary metastases, lymph node (LN) involvement, presence of uncontrolled primary site, and treatment not including pulmonary metastasectomy. Multivariate analysis identified LN metastases (HR 6.06, 95 % CI 1.18–31) and extra-pulmonary metastases (HR 4.06, 95 % CI 1.22–13.57) as the only independent factors associated with inferior OS. Assessment of efficacy of first-line chemotherapy showed superiority in progression-free survival (PFS) for ifosfamide-containing regimens versus non-ifosfamide-containing regimens (median PFS of 8.3 and 2.5 months, respectively, p = 0.002). No such significant difference in PFS was detected for comparison between doxorubicin- and non-doxorubicin-containing regimens (p = 0.45). The current study highlights that the pattern of metastases at first detection of metastatic disease is an important determinant of survival. Future studies evaluating therapeutic strategies for metastatic SS should address the comparability of those factors among study arms. In addition, our results suggest that high-dose ifosfamide should be an integral component of first-line chemotherapy regimen.

Introduction: Panobinostat (PAN) is a potent pan-deacetylase inhibitor that targets multiple myeloma (MM) cells via its epigenetic effects as well as its effect on the aggresome. In the PANORAMA 1 phase 3 trial, the combination of PAN, bortezomib (BTZ), and dexamethasone (Dex; PAN+BTZ+Dex) significantly increased progression-free survival compared with placebo plus BTZ and Dex, leading to approval in Europe of the combination for the treatment of patients with MM who have received ≥ 2 prior regimens, including BTZ and an immunomodulatory agent. The purpose of this expanded treatment protocol (ETP) is to further evaluate safety and to provide panobinostat prior to commercial availability to patients with relapsed/refractory MM who have received ≥ 2 prior lines of therapy but for whom satisfactory treatment alternatives are not available. Methods: Panobinostat-ETP is a multicenter, open-label, ETP study of PAN+BTZ+Dex in adult patients with MM relapsed and/or refractory to ≥ 2 prior lines of therapy. During treatment phase (TP) 1 of the study, patients received oral PAN 20 mg (days 1, 3, 5, 8, 10, and 12) plus intravenous or subcutaneous BTZ 1.3 mg/m2 (days 1, 4, 8, and 11) for eight 21-day cycles. Patients with ≥ stable disease proceeded to TP2, with maintained PAN and less frequent BTZ dosing (days 1 and 8) for an additional 8 cycles. In both phases, oral Dex 20 mg was administered on the days of and after BTZ treatment. Reduction to once-weekly BTZ was allowed prior to TP2 as a dose reduction strategy. Results: A total of 49 patients with a median age of 67 years (range, 45-85 years) were enrolled in the study. Patients were heavily pretreated; 73.5% received ≥ 3 prior lines of therapy. Most patients (n = 43 [87.8%]) received subcutaneous BTZ, while 3 (6.1%) received intravenous BTZ, and another 3 (6.1%) received both. The median duration of treatment with PAN+BTZ+Dex was 67 days (range, 12-305 days). The most common grade 3/4 hematologic laboratory abnormalities were thrombocytopenia (51.0%), anemia (12.2%), and neutropenia (10.2%). The most common nonhematologic grade 3/4 treatment-emergent adverse events (AEs) were diarrhea (14.3%), fatigue (8.2%), infection (6.1%), and nausea (6.1%). Common gastrointestinal treatment-emergent AEs of any grade included diarrhea (51.0%), constipation (16.3%), and nausea (16.3%). Interestingly, in the patients receiving subcutaneous BTZ, the rates of grade 3/4 diarrhea (11.6%), thrombocytopenia (48.8%), anemia (14.0%), and neutropenia (11.6%) were lower than those seen with intravenous BTZ administration in the phase 3 PANORAMA 1 trial (25%, 57%, 16.8%, and 24.1%, respectively). Two patients died while on treatment; one due to disease progression and the other due to infection. Common serious AEs included diarrhea (12.2%) and thrombocytopenia, atrial fibrillation, infection, pneumonia, and syncope (all 6.1%); serious AEs of atrial fibrillation and syncope were higher than in PANORAMA 1 (1.0% and 1.3%, respectively). AEs led to PAN, BTZ, and Dex dose adjustment in 36.7%, 42.9%, and 16.3% of patients, respectively; the most common AE leading to dose adjustment or temporary interruption was thrombocytopenia (all-grade, 30.6%; grade 3/4, 28.6%). Efficacy data will be reported after sufficient follow-up. Conclusions: Overall, the safety results from panobinostat-ETP, albeit in an older population of patients with more advanced MM, support those generated in PANORAMA 1, with only a slight increase in atrial fibrillation, potentially because of the older population. In the subgroup of patients receiving subcutaneous BTZ, rates of diarrhea and hematologic toxicities, AEs of interest with PAN+BTZ+Dex therapy, appear to be reduced compared with PANORAMA 1 data with intravenous BTZ administration; however, because of the small size of this study, these results should be interpreted with caution. Further clinical experience with the use of subcutaneous BTZ in this combination will help to determine the potential impact of the route of BTZ administration on tolerability. Disclosures Guenther: Takeda: Consultancy, Honoraria; Celgene: Honoraria; Novartis: Consultancy, Honoraria. Mügge:Celgene: Honoraria, Research Funding; Janssen: Honoraria; Cilag: Honoraria; Novartis: Honoraria; Amgen: Honoraria; Bristol Myers-Suibb: Honoraria. Schjesvold:Janssen: Research Funding; Novartis: Consultancy, Honoraria, Research Funding. Lechner:Novartis: Honoraria. Gisslinger:Novartis: Consultancy, Honoraria; AOP Orphan: Consultancy, Honoraria; Baxalta: Consultancy, Honoraria. Greil:Novartis: Honoraria, Research Funding; Janssen Cilag: Honoraria, Research Funding. Gunsilius:Takeda: Honoraria, Membership on an entity's Board of Directors or advisory committees; Janssen: Honoraria, Membership on an entity's Board of Directors or advisory committees; Amgen: Honoraria, Membership on an entity's Board of Directors or advisory committees; BMS: Honoraria; Novartis: Membership on an entity's Board of Directors or advisory committees. Weisel:Amgen: Consultancy, Honoraria; Bristol Myer-Squibb: Consultancy, Honoraria; Celgene: Consultancy, Honoraria, Research Funding; Novartis: Consultancy, Honoraria; Janssen: Consultancy, Honoraria, Research Funding; Takeda: Consultancy, Honoraria. Munder:Janssen: Honoraria; Amgen: Honoraria; Bristol Myers Squibb: Honoraria; Takeda: Honoraria. Kiani:Novartis: Consultancy, Honoraria, Speakers Bureau. Campello-Iddison:Novartis: Employment, Equity Ownership. Einsele:Janssen: Consultancy, Honoraria, Speakers Bureau; Novartis: Consultancy, Honoraria; Celgene: Consultancy, Honoraria, Speakers Bureau; Amgen: Consultancy, Honoraria, Speakers Bureau.

Purpose To compare clinical features and outcomes of adults and children reported to have rhabdomyosarcoma. Patients and Methods We analyzed data from 1,071 adults (age > 19 years) and 1,529 children (age ≤ 19 years) reported in the public-access Surveillance, Epidemiology and End Results database as having rhabdomyosarcoma, diagnosed from 1973 to 2005. Survival estimates were determined using survival time with the end point being death from any cause. Results Adults with rhabdomyosarcoma had significantly worse outcome than children (5-year overall survival rates, 27% ± 1.4% and 61% ± 1.4%, respectively; P < .0001). Tumors in adults were more likely to be at an unfavorable site (65% v 55%; P < .0001) and to have histologies that are unusual during childhood, particularly the pleomorphic subtype (19%) and not otherwise specified (43%). Regional and distant spread was not more frequent in adults. Adults had significantly worse outcome than children with similar tumors. The most significant difference was in localized disease; 5-year survival estimates were 82% ± 2.0% for children and 47% ± 2.9% for adults (P < .0001). Multivariate analysis showed that age, histologic subtype, primary site location, stage, and local control with surgery and/or radiation were significant predictors of survival. However, alveolar subtype and unfavorable primary site lost significance when analysis was restricted to adults. Conclusion Adults reported to have rhabdomyosarcoma had worse survival than children with similar tumors. Predictors of poor outcome in children were valid in adults except for alveolar histology and unfavorable tumor site.