Treatment patterns, and survival of patients with metastatic undifferentiated pleomorphic sarcoma: A National Cancer Database (NCDB) study.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 11561-11561
Author(s):  
Hussain I Rangoonwala ◽  
Mary Elizabeth Dahl ◽  
Jonathan Gootee ◽  
Sai Giridhar Gundepalli ◽  
Sarah J Aurit ◽  
...  

11561 Background: Undifferentiated Pleomorphic Sarcoma (UPS) is regarded as one of the most common types of soft tissue sarcoma. Although prior studies have highlighted its metastatic potential, there have been no database studies that delineate the clinical/social characteristics and outcomes in UPS patients with metastatic disease. Utilizing the National Cancer Database (NCDB), we want to observe the complex interplay between treatment approaches and non-biologic modifiers. Methods: 737 patients diagnosed with metastatic UPS above the age of 17 years between 2004 and 2015 were identified utilizing the NCDB. Patients were identified with ICD-O-3 morphologic code 8830/3. Demographic factors (Race/ethnicity, biological sex, Median household income/education at zip code level, facility type, insurance) were studied in relation to the type of treatment they received: No treatment; chemotherapy only; chemotherapy and radiation therapy; chemotherapy and surgery; chemotherapy, surgery, and radiation; radiation therapy and/or surgery; and other treatments. Survival tables were utilized to generate 1-year and 3-year survival rates, and Kaplan-Meier method with associated log-rank list was used to examine the differences in unadjusted survival. Results: Approximately 17% of our cohort were left untreated, whereas 83% received treatment. Patients who were untreated were likely to be older, on Medicare, and had a Charlson-Deyo (CD) score of 2 or above. Patients who were more likely to receive treatment were younger educated males with private insurance, residing in areas of higher income, and receiving care at an academic program. Patients who only received chemotherapy were more likely to belong to areas with lower income and had a CD score of 0. Patients who received a combination of chemotherapy, surgery, and radiation therapy were more likely to belong to areas of high income, private insurance owners, and received care at an academic facility. Patients who received chemotherapy with surgery and without radiation therapy were more likely to be younger males from areas of highest income, and received care at an academic facility. Patients who had better unadjusted survival were younger educated patients on private insurance or Medicaid, with distant lymph nodes metastases at diagnosis, who received a combination of chemotherapy, radiation therapy and surgery at an academic program. Patients who had poorer unadjusted survival were untreated older patients on Medicare, and with bone metastases. Conclusions: This is one of the most comprehensive studies involving patients with metastatic UPS that analyzes demographic variables in relation to the treatment approach. Some of the major determinants that influence outcomes in these patients included age, insurance status, treatment at academic facility, CD score, and type of metastases at diagnoses.

2017 ◽  
Author(s):  
Ann Y Lee

Both myxofibrosarcoma and undifferentiated pleomorphic sarcoma (UPS) are sarcomas that primarily occur in the extremities of older patients. UPS (previously known as malignant fibrous histiocytoma) is now considered a diagnosis of exclusion, representing a group of high-grade, heterogeneous tumors with no clear line differentiation and characterized by high rates of distant metastases. Myxofibrosarcoma (formerly myxoid variant of malignant fibrous histiocytoma) is a distinct histologic entity that is largely distinguished from UPS by its myxoid stroma and curvilinear vascular pattern. Myxofibrosarcoma tends to have a locally infiltrative behavior that can often be seen as T2 hyperintense “tails” on magnetic resonance imaging. Local recurrence rates can be as high as 61% and are independent of grade. Unlike other sarcomas, it is recommended that tumors be excised with at least a 2 cm margin around both clinically and radiographically evident tumor. Low-grade tumors do not tend to metastasize but can recur as high-grade tumors with increased metastatic potential. Surgery is the mainstay of treatment for localized disease. For high-grade tumors, this may require complex plastics and vascular reconstruction. Selecting cases for multimodality therapy, including radiation and chemotherapy, should be done on a case-by-case basis after review by a multidisciplinary sarcoma team with particular attention to a patient’s risk of local recurrence and distant metastases. Further studies into histology-specific therapeutic targets will help inform future clinical trials. This review contains 4 figures, 4 tables and 54 references Key words: chemotherapy, distant metastases, extremity sarcoma, local recurrence, malignant fibrous histiocytoma, myxofibrosarcoma, radiation, sarcoma, surgical margins, T2 hyperintense tails, undifferentiated pleomorphic sarcoma


Sarcoma ◽  
2019 ◽  
Vol 2019 ◽  
pp. 1-9 ◽  
Author(s):  
Anthony A. Catanzano ◽  
David L. Kerr ◽  
Alexander L. Lazarides ◽  
Brian L. Dial ◽  
Whitney O. Lane ◽  
...  

Background. Although chondrosarcomas (CS) are mostly considered radioresistant, advancements in radiotherapy have brought attention to its use in these patients. Using the largest registry of primary bone tumors, the National Cancer Database (NCDB), we sought to better characterize the current use of radiotherapy in CS patients and identify any potential survival benefit with higher radiation doses and advanced radiation therapies. Methods. We retrospectively analyzed CS patients in the NCDB from 2004 to 2015 who underwent radiotherapy. The Kaplan–Meier method with statistical comparisons was used to identify which individual variables related to dosage and delivery modality were associated with improved 5-year survival rates. Multivariate proportional hazards analyses were performed to determine independent predictors of survival. Results. Of 5,427 patients with a histologic diagnosis of chondrosarcoma, 680 received a form of radiation therapy (13%). The multivariate proportional hazards analysis controlling for various patient, tumor, and treatment variables, including RT dose and modality, demonstrated that while overall radiation therapy (RT) was not associated with improved survival (HR 0.96, 95% CI 0.76–1.20), when examining just the patient cohort with positive surgical margins, RT trended towards improved survival (HR 0.81, 95% CI 0.58–1.13). When comparing advanced and conventional RT modalities, advanced RT was associated with significantly decreased mortality (HR 0.55, 95% CI 0.38–0.80). However, advanced modality and high-dose RT both trended only toward improved survival compared to patients who did not receive any RT (HR 0.74, 95% CI 0.52–1.06 and HR 0.93, 95% CI 0.71–1.21, respectively). Conclusions. Despite the suggested radioresistance of CS, modern radiotherapies may present a treatment option for certain patients. Our results support a role for high-dose, advanced radiation therapies in selected high-risk CS patients with tumors in surgically challenging locations or unplanned positive margins. While there is an associated survival rate benefit, further, prospective studies are needed for validation.


2017 ◽  
Author(s):  
Ann Y Lee

Both myxofibrosarcoma and undifferentiated pleomorphic sarcoma (UPS) are sarcomas that primarily occur in the extremities of older patients. UPS (previously known as malignant fibrous histiocytoma) is now considered a diagnosis of exclusion, representing a group of high-grade, heterogeneous tumors with no clear line differentiation and characterized by high rates of distant metastases. Myxofibrosarcoma (formerly myxoid variant of malignant fibrous histiocytoma) is a distinct histologic entity that is largely distinguished from UPS by its myxoid stroma and curvilinear vascular pattern. Myxofibrosarcoma tends to have a locally infiltrative behavior that can often be seen as T2 hyperintense “tails” on magnetic resonance imaging. Local recurrence rates can be as high as 61% and are independent of grade. Unlike other sarcomas, it is recommended that tumors be excised with at least a 2 cm margin around both clinically and radiographically evident tumor. Low-grade tumors do not tend to metastasize but can recur as high-grade tumors with increased metastatic potential. Surgery is the mainstay of treatment for localized disease. For high-grade tumors, this may require complex plastics and vascular reconstruction. Selecting cases for multimodality therapy, including radiation and chemotherapy, should be done on a case-by-case basis after review by a multidisciplinary sarcoma team with particular attention to a patient’s risk of local recurrence and distant metastases. Further studies into histology-specific therapeutic targets will help inform future clinical trials. This review contains 4 figures, 4 tables and 54 references Key words: chemotherapy, distant metastases, extremity sarcoma, local recurrence, malignant fibrous histiocytoma, myxofibrosarcoma, radiation, sarcoma, surgical margins, T2 hyperintense tails, undifferentiated pleomorphic sarcoma


2021 ◽  
Author(s):  
Christopher Williamson ◽  
Shayla Williamson ◽  
Renjian Jiang ◽  
Lisa Sudmeier ◽  
Natia Esiashvili ◽  
...  

Abstract Purpose Pediatric high-grade glioma (HGG) is a devastating disease with a poor prognosis. The purpose of this analysis is to evaluate the impact of Radiation Therapy (RT) variables on outcomes of pediatric HGG patients in the National Cancer Database (NCDB). Methods The NCDB was used to select patients age < 22 with histologically proven WHO Grade III and IV gliomas treated with ≥ 50Gy and < 76Gy RT between 2004 and 2013. RT variables including RT dose, timing between diagnosis and RT initiation (< 4 weeks, 4–6 weeks, or > 6 weeks), and RT modality were analyzed along with baseline demographic, tumor and treatment variables to assess the impact on overall survival in univariate and multivariable analyses. Results 498 pediatric HGG patients were included. Histologies included glioblastoma (30%), astrocytoma (55%), oligogendroglioma (5%) and gliomas not otherwise specific (10%). The median RT dose was 59.4 Gy (SD 2.9 Gy) starting a median of 4.4 weeks from diagnosis (SD 2.5 weeks). Median follow-up was 19.6 months with 1- and 3-year OS of 78.4% and 40.4%, respectively. On Multivariable analysis, female gender, older age, and private insurance remained independently associated with lower rate of overall death. Radiation initiation ≤ 4 weeks from diagnosis, and glioblastoma histology were significantly associated with higher rate of overall death. There was no relationship between radiation dose or whether radiation was delivered with proton or photon therapy and overall survival. Conclusions Outcomes for pediatric HGG are poor. Early initiation of RT within 4 weeks from diagnosis was negative associated with overall survival and may be related to unknown prognostic factors.


2017 ◽  
Vol 66 (3) ◽  
pp. 168-171 ◽  
Author(s):  
Kazunobu Hirooka ◽  
Masahiro Oonuki ◽  
Susumu Manabe ◽  
Daisuke Hiraoka ◽  
Takashi Yasukawa ◽  
...  

2020 ◽  
Vol 13 (2) ◽  
pp. e232616 ◽  
Author(s):  
Joshua Kong ◽  
Awni D Shahait ◽  
Steve Kim ◽  
Lydia Choi

Breast sarcoma is a rare form of malignancy that arises from connective tissue within the breast, comprising less than 5% of all sarcomas. They develop as primary tumours or as secondary following radiation therapy. Diagnosis can be challenging as breast sarcomas are often asymptomatic and resemble benign breast tissue changes. Radiation-induced breast sarcomas present in various forms with an average latency period of 10–20 years following initial radiation therapy. Angiosarcomas are the most common form, while other types such as undifferentiated pleomorphic sarcomas remain rare. Here, we report a case of radiation-induced undifferentiated pleomorphic breast sarcoma in a 75-year-old woman that developed nearly 20 years following breast conserving surgery and radiation for invasive ductal carcinoma. The patient initially noticed a mass in 2017 on self-examination. The mammogram, ultrasound and biopsy at the time showed a benign 2.2 cm nodular fasciitis without malignancy. The mass grew rapidly in the next 6 months to 5.6 cm and repeat biopsy diagnosed undifferentiated pleomorphic sarcoma. The mass abutted the pectoralis muscle but staging workup ruled out distant metastasis and the patient underwent wide local resection of the mass with clear margins. The patient subsequently underwent further postoperative radiation due to insufficient posterior margin width on wide local excision, as chest wall resection would have been required for a wider posterior margin. Prognosis for postradiation sarcomas is generally poor with 27%–36% 5-year survival, with surgical resection as the main line of treatment. The patient currently remains disease-free after 15 months of surveillance.


Sign in / Sign up

Export Citation Format

Share Document