Impact of Treatment Modalities Upon Survival Outcomes in Skull Base and Clival Chordoma: An NCDB Analysis

Author(s):  
Ethan Gregory Muhonen ◽  
Tyler M. Yasaka ◽  
Brandon Lehrich ◽  
Khodayar Goshtasbi ◽  
Peter Papagiannopoulos ◽  
...  

Objectives: Skull base chordomas are locally aggressive malignant tumors derived from the notochord remnant. There are limited large-scale studies examining the role and extent of surgery and radiation therapy. Design: Analysis of the National Cancer Database (NCDB) was performed to evaluate the survival outcomes of various treatments, and to assess for predictors of overall survival (OS). Participants: Retrospective, population-based cohort study of patients diagnosed with a clival/skull base chordoma between 2004-2015 in the NCDB. Main Outcome Measures: The primary outcome was overall survival (OS). Results: 468 cases were identified. 49% of patients received surgery and 20.7% had positive margins. Mean age at diagnosis was 48.4 years in the surgical cohort, and 55% were male. Of the surgical cohort, 33.8% had negative margins, 20.7% had positive margins, and 45.5% had unknown margin status. Age ≥ 65 (HR 3.07, 95% CI 1.63-5.76, p<0.001), diagnosis between 2010-2015 (HR 0.49, 95% CI 0.26-0.90, p=0.022), tumor size >5 cm (HR 2.29, 95% CI 1.26-4.15, p=0.007) and government insurance (HR 2.28, 95% CI 1.24-4.2, p=0.008) were independent predictors of OS. When comparing surgery with or without adjuvant radiation, no survival differences were found, regardless of margin status (P=0.66). Conclusions: Surgery remains the mainstay of therapy. Advanced age over 65, large tumor size, and government insurance were predictors of worse OS. While negative margins and the use of adjuvant radiation did not appear to impact OS, these may very well reduce local recurrences. A multidisciplinary approach is critical in achieving optimal outcomes in this challenging disease.

2021 ◽  
Vol 39 (6_suppl) ◽  
pp. 361-361
Author(s):  
Nandini Sharrel Menon ◽  
Devanshi Kalra ◽  
Kumar Prabhash ◽  
Vanita Noronha ◽  
Santosh Menon ◽  
...  

361 Background: Primitive neuroectodermal tumours (PNET) of the kidney are rare tumours with aggressive behaviour. This study was conducted to review the diagnosis and management of patients with renal PNET at our centre. Methods: This was a retrospective study conducted at a tertiary cancer care centre in Mumbai, India. The demographic and clinical data of 17 patients treated by the uro-oncology services were retrieved from electronic medical records. Descriptive analysis was performed for baseline characteristics.Overall & progression-free survival was determined using the Kaplan Meier method. Cox regression was used for multivariate analysis. Results: There were 12 male and 5 female patients in this cohort with a median age of 27 years. At diagnosis 2 patients had metastatic disease and 15 patients had non-metastatic disease. Median follow up in this cohort was 22 months (range 2-30 months). Presenting complaints were hematuria, abdominal pain, flank pain, fever, bone pain, and incidentally detected renal mass. All patients were Mic -2 positive and 13 were FLI-1 positive on immunohistochemistry. Fourteen patients underwent radical nephrectomy. One (5.9%) patient received both neoadjuvant and adjuvant chemotherapy, 8 (47.1%) received adjuvant and 2 (11.8%) received palliative chemotherapy upfront. Eight patients received adjuvant radiation to the renal bed.There was disease progression in12 patients,10 of 15 patients with non metastatic disease at diagnosis eventually developed metastasis.The median progression free survival (PFS) was 10.55 months.The pathological feature that was associated with a shorter PFS was tumor size ⩾10 cm(p = 0.044).The median overall survival was 20.04 months (95% CI 9.49 -not reached). The presence of metastasis and treatment received significantly impacted overall survival (OS). Median OS in patients with non-metastatic disease was not reached versus 14.1 months in those with metastatic disease (p = .019).The median OS in patients treated with multimodality approach was 20.11 months. Patients did not undergo surgery had a median OS of 5.45 months (p < .001) and those who did not receive any chemotherapy had a median OS of 4.57 months (p = .024).Thus, patients who received multimodality treatment had better outcomes. Conclusions: PNET kidney is an aggressive tumor which should be treated with a multimodality approach. Tumor size ⩾10 cm was an adverse prognostic factor.


2021 ◽  
pp. 106689692110560
Author(s):  
Hao Cheng ◽  
Chi Yihebali ◽  
Hongtu Zhang ◽  
Lei Guo ◽  
Susheng Shi

Background Synovial sarcoma (SS) is a rare soft tissue sarcoma. Available data regarding survival outcomes of patients with SS still remains limited. In this study, a single center retrospective analysis was performed to investigate the clinical characteristics, pathology and survival outcomes in patients with SS in China. Methods Patient data were systematically reviewed at the National Cancer Center from January 2015 to December 2020. The general information and treatment condition of patients were collected. Overall survival (OS) was evaluated using the Kaplan-Meier and Cox regression method. Results A total of 237 consecutive patients were included in this study (follow-up cut-off date: December, 2020). The median age of patients involved was 35 years (ranging from 5 to 83 years) and the mean tumor diameter was 5.3 cm (ranging from .2 to 26.0 cm). The main findings of the immunohistochemical staining analyses were EMA (111/156) (71%), keratin (32/64) (50.0%), keratin (12/20) (60%), keratin (42/70) (60%), S-100 (18/160) (11%), BCL-2 (128/134) (96%), CD99 (137/148) (93%) and TLE1 (23/26) (88%). It was found that 109 patients (66%) were presented with monophasic subtype and 55 (34%) with biphasic subtype. A total of 137 patients were tested by FISH method and 119 patients (87%) demonstrated SS18 rearrangement, whereas 18 patients (13%) did not show SS18 rearrangement. Generally, it was found that the 3-year OS rate was 86% and the 3-year DFS was 55%. Results of univariate analysis revealed that age, tumor size, tumor site, radiotherapy and targeted therapy were significantly correlated with the overall survival ( P < .05). Further, multivariate Cox regression analysis revealed that age, tumor size and radiotherapy were significantly associated with OS ( P < .05). Conclusions In conclusion, this study shows that the outcomes of patients with SS significantly decrease with age and tumor size. It was evident that radiotherapy is an independent and positive prognostic factor for patients with SS. In addition, it was shown that the prognosis of SS varies with tumor location. For instance, primary tumors in lower extremities have a higher prognosis, whereas tumors located in thorax have a lower prognosis.


2019 ◽  
Vol 29 (9) ◽  
pp. 1355-1360 ◽  
Author(s):  
Giorgio Bogani ◽  
Daniele Vinti ◽  
Ferdinando Murgia ◽  
Valentina Chiappa ◽  
Umberto Leone Roberti Maggiore ◽  
...  

ObjectiveNodal involvement is one of the most important prognostic factors in cervical cancer patients. We aimed to assess the prognostic role in relation to the burden of nodal disease in stage IIICp cervical cancer.MethodsData on all consecutive patients diagnosed with cervical cancer undergoing primary surgery (radical hysterectomy plus lymphadenectomy) or neoadjuvant chemotherapy followed by radical hysterectomy plus lymphadenectomy, between January 1980 and December 2017, were collected in a dedicated database. Exclusion criteria were: (1) consent withdrawal; (2) synchronous malignancies (within 5 years). Survival outcomes were assessed using Kaplan-Meier and Cox models.ResultsOverall, 177 (14.1%) of 1257 patients with cervical cancer were diagnosed with positive lymph nodes. After a median follow-up of 58 (range 4–175) months, 66 (37.3%) and 37 (20.9%) patients developed recurrent disease and died of disease, respectively. Via multivariate analysis, positive para-aortic nodes (HR 2.62, 95% CI 1.12 to 6.11; p=0.025) and the number of positive nodes (HR 1.06, 95% CI 1.02 to 1.11; p=0.002) correlated with worse disease-free survival. Furthermore, the number of positive nodes (HR 1.06, 95% CI 1.01 to 1.12; p=0.021) correlated with worse overall survival. Number of positive nodes (1, 2 or ≥3) strongly correlated with both disease-free survival (p<0.001, log-rank test) and overall survival (p=0.001, log-rank test). Focusing on patients receiving adjuvant radiation and chemotherapy, the number of positive lymph nodes was associated with response to treatment (p<0.001). Median disease-free survival was 100, 42, and 12 months for patients with one, two, or three or more positive lymph node(s), respectively (p<0.001, log-rank test).ConclusionsIn stage IIICp cervical cancer, adjuvant radiation and chemotherapy provides adequate overall survival in patients diagnosed with only one metastatic node, while survival outcomes are poor in patients with two or more metastatic nodes. This highlights the need for innovative treatments in patients with a high burden of lymphatic disease.


2019 ◽  
Vol 37 (7_suppl) ◽  
pp. 595-595
Author(s):  
Margaret Frances Meagher ◽  
Aaron Bradshaw ◽  
David Anyakora ◽  
Dattatraya H Patil ◽  
Kazutaka Saito ◽  
...  

595 Background: African-Americans have increased incidences of renal cortical tumor subtypes of lower oncological potential in the setting of lower risk disease when compared to other ethno-racial groups. However, survival outcomes are similar. We investigated the impact of African-American race on overall survival, oncological outcomes, functional outcomes, and non-cancer mortality. Methods: Multi-institutional retrospective analysis of patients who underwent partial or radical nephrectomy between 1998-2018. Primary outcome was overall survival (OS). Secondary outcomes included non-cancer mortality (NCM), recurrence free survival (RFS), and estimated glomerular filtration rate (eGFR) decline. Multivariable logistic regression (MVA) were used to elucidate predictive factors for OS, NCM, and RFS, and eGFR <45 and <30 ml/min/1.73m2. Results: 3,088 patients were divided into African American (AA, n=353) and Non-African American (NAA, n=2735) sub-groups. No difference was noted between groups with respect to mean tumor size (p=0.211) or metastases presence (p=0.846). African-American race was an independent risk factor for functional decline to eGFR<45 (OR 4.43, p<0.001) and eGFR<30 (OR 5.15, p<0.001). MVA for worsened NCM demonstrated African-American race (OR=1.72, p=0.042), increasing age (OR=1.03, p=0.001), radical nephrectomy (OR=2.98, p<0.001), and increasing tumor size (OR=1.26, p<0.001) to be independent risk factors. MVA for worsened OS included increasing age (OR=1.04, p<0.001), tumor size (OR=1.182, p<0.001), clear cell histology (OR=1.62, p<0.001), high tumor grade (OR=2.12, p<0.001), and post-operative eGFR <45 (OR=2.12, p<0.001). MVA for worsening RFS demonstrated high tumor grade (OR=2.38, p<0.001) and increasing clinical tumor size (OR=1.152, p<0.001) to be independent factors. Conclusions: African Americans undergoing renal surgery for RCC appear to have similar OS and RFS, but poorer NCM than non-African American patients. The cause of these disparities is multi-faceted and likely associated with functional decline. Nephron-sparing management should be considered in African-Americans presenting with renal cortical tumors.


2021 ◽  
Author(s):  
Walid Ibn Essayed ◽  
Ossama Al-Mefty

Abstract Chondrosarcomas are a spectrum of tumors with variable clinical behavior, histologically classified as grades I, II, and III.1 Maximal surgical resection with adjuvant radiation is the gold standard for grade III malignant tumors.2,3 Lower-grade skull base chondrosarcomas have a slow progressive local growth pattern with an overall benign profile.1,4 In these grade I and II tumors, radical resection can be sufficient in achieving long-term control of the disease without the need for adjuvant therapy, thus avoiding the long-term side effects of irradiation in relatively young patients.4,5 As a slow-growing tumor, skull base chondrosarcoma might reach a giant size with progressive neurological deficits prior to diagnosis. In these cases, to achieve maximal resection, skull base approaches tailored to the patient's anatomy are essential.4,6 Cranial approach is frequently needed due to the calcified nature of part of the tumor that is not amenable to resection through the endonasal approach. The endoscope is extremely helpful combined with the microscopic resection. We demonstrate these techniques through in the surgery of a 39-yr-old female who presented with progressive neurological deficits from a giant ossified chondrosarcoma and underwent a combined petrosal approach for the resection of her tumor. A postoperative small enhancement remained stable on the 4-yr follow-up exam. The pathology confirmed grade II chondrosarcoma, with the patient recovering from her neurological deficit except the third nerve. The patient consented to the surgery and publication of images.  Image at 1:36 reprinted with permission from Al-Mefty O, Operative Atlas of Meningiomas. Vol 1, ©LWW, 1998.


2019 ◽  
Vol 178 (3) ◽  
pp. 523-533 ◽  
Author(s):  
Yara Abdou ◽  
Ahmed Elkhanany ◽  
Kristopher Attwood ◽  
Wenyan Ji ◽  
Kazuaki Takabe ◽  
...  

Abstract Background Primary and secondary breast angiosarcoma is a rare and aggressive malignancy with limited published literature. Optimal management is mostly based on expert opinion. Our study aims to describe a single institution experience with breast angiosarcoma and evaluate other publications on this topic to further clarify prognostic outcomes and treatment modalities in this disease. Methods Twenty two cases of breast angiosarcoma from Roswell Park Comprehensive Cancer Center were retrospectively analyzed. Additionally, a systemic review and meta-analysis was conducted to study the association between survival outcomes, overall survival (OS), and recurrence-free survival (RFS) in both primary (PAS) and secondary breast angiosarcoma (SAS). Results 9 PAS patients (41%) and 13 SAS patients (59%) were retrospectively analyzed. No significant differences were noted in tumor characteristics and survival outcomes between PAS and SAS. Treatment modality had no significant effects on survival outcomes although adjuvant chemotherapy demonstrated a trend towards improved RFS in high grade tumors. 380 PAS and 595 SAS patients were included in the outcome meta-analysis. Survival outcomes were significantly worse with high grade tumors and tumor size of > 5 cm. Adjuvant radiation therapy demonstrated significantly better RFS, while adjuvant chemotherapy had no effect on survival outcomes. Conclusion Tumor size and grade seem to be reliable predictors of survival in both PAS and SAS. Mastectomy does not seem to be adding any additional benefit to BCS. Adjuvant radiation therapy showed statistically significant RFS benefit, while adjuvant chemotherapy can be beneficial in high grade tumors.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e16084-e16084
Author(s):  
Lindsay Kaye Morris ◽  
Alaa Altahan ◽  
John Mays ◽  
Upama Giri ◽  
Eric Wiedower ◽  
...  

e16084 Background: Data is limited regarding outcomes in patients with RCC with positive surgical margins. We sought to evaluate the impact of margin status after radical nephrectomy (RN) on relapse free survival (RFS) and overall survival (OS). Methods: A retrospective study was conducted evaluating patients with RCC having undergone RN at Methodist University Hospital in Memphis, Tennessee, between January 2009 and December 2013. Patients were identified from the tumor registry at this institution, and IRB approval obtained. Patient and tumor characteristics and survival were analyzed by GraphPad Prism, Microsoft Excel and IBM SPSS. Results: 156 patients that underwent RN for RCC were identified; 12 patients (7.7%) had positive margins and 144 had negative margins. Mediation duration of follow-up was 3.4 years. 5 of 12 patients with positive margins relapsed, versus 20 of 144 with negative margins (41.7% v. 13.9%, p = 0.022) with a RR of 3.10 (95% CI 1.417-6.799). Among those who relapsed, there was a statistically significant difference in time to relapse between patients with positive and negative margins (mean number of days to relapse 275 versus 621, respectively, with p = 0.038). On multivariate analysis of age, gender, ethnicity, laterality, tumor histology, margin status, and tumor size, margin status was not a statistically significant determinant of OS at 1, 3, and 5 years (p = 0.051, 0.124 and 0.185 respectively) or RFS at 1, 3, and 5 years (p = 0.372, 0.271 and 0.242 respectively). Pearson correlation analysis showed significant correlation between tumor size and margin status, R = 0.478, p < 0.001. Conclusions: Positive margins were associated with earlier time to relapse among patients following RN. However, in multivariate analysis, margin status was not a statistically significant determinant of OS or RFS. In the current era of multiple available agents in RCC capable of cytoreduction, the risk factors that are predictive of a positive surgical margin at RN should be considered in the design of neoadjuvant systemic therapy trials, with the goal of improving long-term outcomes.


2017 ◽  
Vol 35 (4_suppl) ◽  
pp. 347-347 ◽  
Author(s):  
Mustafa Raoof ◽  
Laleh Golkar Melstrom ◽  
Susanne Warner ◽  
Yanghee Woo ◽  
Gagandeep Singh ◽  
...  

347 Background: American Society of Clinical Oncology guidelines recommend adjuvant chemoradiation (ACR) for margin-positive(R1) and/or node-positive (N+) pancreatic cancers. However, randomized trials and meta-analyses have have not shown superiority of ACR over AC. Methods: National Cancer Database (NCDB) was used to analyze patients with N+ and/or R1 pancreatic adenocarcinoma who underwent ACR or AC over a ten-year period (2004-2014). Patients who received neoadjuvant radiation, no adjuvant treatment or adjuvant radiation alone were excluded. Propensity score nearest-neighbor 1:1 matching (PSM) was performed between ACR and AC groups based on age, sex, race, insurance, comorbidities, T-stage, nodal status, margin status, grade, and neoadjuvant chemotherapy. Primary outcome was overall survival (OS). Results: A total of 9,732 patients were eligible. After PSM two well-balanced groups of 4000 patients each were analyzed. ACR resulted in superior OS in patients with N+ and/or R1 disease as compared to AC alone (HR: 0.83, 95% CI 0.78-0.87; Median OS 22 vs. 19 months, p<0.001). Subset analyses demonstrated overall survival benefit of ACR compared to AC in N+, margin-negative patients (HR: 0.82, 95% CI 0.77-0.88; Median OS 24 vs. 20 months, p<0.001), as well as N+, R1 patients (HR: 0.77, 95% CI 0.68-0.87; Median OS 17 vs. 15 months, p<0.001); but no benefit in node-negative, R1 patients (HR: 1.12, 95% CI 0.84-1.48; Median OS 18 vs. 22 months, p = 0.43). Conclusions: This is the largest study to date that shows superiority of ACR over AC in N+ patients irrespective of margin status. The study failed to show a survival benefit in R1, node-negative patients.


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. 11555-11555
Author(s):  
Hanna Kosela ◽  
Mateusz Spalek ◽  
Aneta Borkowska ◽  
Pawel Teterycz ◽  
Tadeusz Morysinski ◽  
...  

11555 Background: Soft tissue sarcomas (STS) are rare often malignant tumors. The primary treatment of most STS is radical resection with adjuvant radiotherapy. Our previous study showed that the use of preoperative hypofractionated radiotherapy is safe and efficient in the treatment of unselected group of patients with STS. Aim of this study was to assess the use of the treatment scheme in patients with primary STS treated in one institution. Methods: 311 patients (52% female) with primary locally advanced STS participated in this prospective trial conducted from 2010 till 2017. Median age was 55 years (range: 17-91). Median follow up is 57 months (95%CI 55-61). The most common subtypes were pleomorphic sarcoma, liposarcoma and synovial sarcoma. Median tumor size was 11cm. 258 patients (83%) had high grade tumors. The most common tumor localization were lower limbs (72%). 30% of patients received preoperative chemotherapy. For five consecutive days radiotherapy in 5 x 5Gy fraction was applied, with immediate (2-4 days) resection of the tumor. Results: In 260 patients (83.6%) clear surgical margins (R0) were obtained. 107 patients were dead at the time of the analysis.5-year overall survival is 63%. 156 (50%) had a recurrence of the disease. Local recurrence (LR) was found in 13.8% of the patients. Median time from surgery to LR was 14.7 months. In 56% of patients with LR another limb spearing radical surgery could be performed. Factors that had a significant adverse impact on LR were histological subtype (p = 0.017) and surgical margin status (p = 0.013). Factors that had a significant adverse impact on overall survival were tumor size (p < 0.0001), grade (p = 0.0047) and surgical margin status (p = 0.013). 96 patients (30.8%) had any kind of treatment toxicity, factor having negative impact on the toxicity was lower limb location of the tumor (p = 0.0012). 20 patients (6.4%) required surgery for treatment of the complications. 14.6% patients had prolonged healing of the wound ( > 1 month), 8.3% had wound dehiscence, 1.4% required prolonged punctures of the lymph fluid, 2.3% had severe fibrosis leading to contracture of limb, 11% patients prolonged edema of the operated limb. 0.9% of patients had a fracture of the treated limb. Conclusions: In this group, with a big percentage of patients with large, high grade STS use of hypofractionated preoperative radiotherapy was associated with similar local control when compared to published studies. The early toxicity is tolerable, with a small amount of late complications.


2020 ◽  
Vol 38 (6_suppl) ◽  
pp. 749-749
Author(s):  
Margaret Frances Meagher ◽  
Aaron Bradshaw ◽  
Dattatraya H Patil ◽  
Kazutaka Saito ◽  
Brittney Cotta ◽  
...  

749 Background: African-Americans have an increased incidence of renal tumors of lower oncological potential when compared to other ethno-racial groups. Yet, survival outcomes are similar. We investigated the impact of African-American race on overall survival, oncological and functional outcomes, and non-cancer mortality. Methods: Multi-institutional (Emory, TMDU, UCSD) retrospective analysis of patients who underwent partial or radical nephrectomy between 1998-2018. Primary outcome was overall survival (OS). Secondary outcomes were non-cancer mortality (NCM), recurrence free survival (RFS), and eGFR decline. Multivariable logistic regression (MVA) was used to analyze OS, NCM, and RFS, and estimated glomerular filtration rate (eGFR)<45 and <30 ml/min/1.73m2. Results: 3632 patients were grouped into African American (AA, n=531) and Non-African American (NAA, n=3101). No difference was noted between groups with respect to mean tumor size (p=0.31). NAA had a higher proportion of metastases at presentation (9.9% vs. 7.0%, p=0.04). AA race was an independent risk factor for functional decline to de novo eGFR <45 (OR=1.43, p=0.04) and de novo eGFR<30 (OR 2.01, p<0.001). MVA for worsened NCM demonstrated AA race (OR=1.63, p=0.02), increasing age (OR=1.05, p<0.001), male sex (OR=1.56, p=0.01), and hypertension (OR=1.73, p=0.001) to be independent risk factors. Significant factors on MVA for worsened OS included increasing age (OR=1.03, p<0.001), radical nephrectomy (OR=1.47, p=0.01), increasing tumor size (OR=1.11, p<0.001), hypertension (OR=2.63, p<0.001), high tumor grade (OR=1.97, p<0.001), and post-operative eGFR <45 (OR=1.50, p=0.01). MVA for worsening RFS demonstrated high tumor grade (OR=2.04, p<0.001) and increasing clinical tumor size (OR=1.15, p<0.001) to be independent factors. Conclusions: African Americans undergoing surgical management for RCC appear to have similar OS and RFS, but poorer NCM than non-African American patients. The cause of these disparities is multi-faceted but likely is associated with functional decline. Nephron-sparing management when feasible and appropriate should be considered in African-Americans presenting with renal cortical tumors.


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