Clinical Outcomes and Prognostic Features of Angiosarcoma: Significance of Prior Radiation Therapy

Merfeld, Emily; Gabani, Prashant; Spraker, Matthew B.; Zoberi, Imran; Kim, H.; Tine, Brian A. Van; Chrisinger, John S.A.; Michalski, Jeff M.

doi:10.1016/j.clon.2019.01.006

Cited by 16 publications

(25 citation statements)

References 33 publications

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“…Survival for AS patients is generally poor with reported five-year survival rates of around 40% [2,3,4], dropping to 15% in metastatic patients [2]. There are contradictory findings concerning outcomes for clinical subtypes, as some studies reported either favorable or worse prognosis for RTx induced or soft tissue (ST) AS [4,5,6,7], whilst others did not find prognostic value for clinical subtypes [2,8]. It could be speculated that primary (sporadic) AS has a different mechanism of development than secondary AS.…”

Section: Introductionmentioning

confidence: 99%

Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas

Weidema

Flucke

Graaf

et al. 2019

Cancers

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Angiosarcoma (AS) is a rare sarcoma of endothelial origin, arising spontaneously (primary AS) or after external damage such as radiation therapy or UV exposure (secondary AS). To date, reliable assessment of prognostic factors has proven difficult, due to disease rarity and heterogeneity of study cohorts. Although large registries provide relatively large AS patient series, these cases often lack histological confirmation. This study aimed to analyze AS prognostic factors in a large nationwide cohort of histologically confirmed cases, established through linkage of clinical data from the Netherlands Cancer Registry and pathology data from the Dutch pathology registry (PALGA). All cases were reviewed by an expert pathologist, showing a 16% discordance rate. Multivariable Cox regression survival analysis among 479 confirmed AS patients revealed remarkably poorer overall survival (OS) for primary AS compared to secondary AS (7 vs 21 months, Hazard ratio (HR) = 1.5; 95% confidence interval (CI) = 1.2–1.9). Age above 65 years, male gender, and no surgical treatment also significantly correlated to worse OS. Overall, OS was relatively poor, with a median of 13 months (95% CI = 10–16 months) and 22% five-year survival rate. With this study, we illustrate AS heterogeneity in clinical behavior and show for the first time better survival for secondary AS compared to primary AS.

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Section: Introductionmentioning

confidence: 99%

Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas

Weidema

Flucke

Graaf

et al. 2019

Cancers

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“…Lastly, information on history of prior radiation exposure was only available in 19% of our AS patients, preventing precise evaluation of the prevalence rate of radiation‐associated AS within the AS population. Radiation‐associated AS is associated with a worse prognosis 23 and different molecular features such as c‐ myc amplification compared with primary AS patients 24 . Future studies on AS should describe the proportion of radiation‐associated AS to understand if their outcome will differ from those without radiation association.…”

Section: Discussionmentioning

confidence: 99%

The treatment landscape of advanced angiosarcoma in Asia—A multi‐national collaboration from the Asian Sarcoma Consortium

et al. 2021

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Angiosarcoma (AS) is a rare disease with a dismal prognosis. The treatment landscape and prognostic factors for advanced AS, including locally advanced, unresectable, and metastatic disease remain elusive. The Asian Sarcoma Consortium is an international collaborative effort to understand the sarcoma treatment landscape in Asia. We undertook a retrospective chart review of AS patients seen in 8 sarcoma academic centers across Asia. Patients with complete clinical, treatment, and follow‐up data were enrolled. Overall, 276 advanced AS patients were included into this study; 84 (30%) of the patients had metachronous metastatic AS. The median age was 67 y; primary sites of AS was cutaneous in 55% and visceral in 45% of patients. In total, 143 (52%) patients received at least 1 line of systemic chemotherapy. The most common first‐line chemotherapy regimen used was paclitaxel (47.6%) followed by liposomal doxorubicin (19.6%). The median overall survival (OS) was 7.8 mo. Significant prognostic factors for OS included age > 65 (hazard ratio (HR) 1.54, P = .006), male gender (HR 1.39, P = .02), and a cutaneous primary AS site (HR 0.63, P = .004). The median progression‐free survival (PFS) for first‐line chemotherapy was 3.4 mo. PFS for single vs combination or paclitaxel vs liposomal doxorubicin chemotherapy regimens were comparable. This study provides an insight into the treatment patterns and prognostic factors of advanced AS patients in Asia. Prognosis of advanced AS remains poor. Data from this study serve as a benchmark for future clinical study design.

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“…It has been reported that the median overall survival (OS)varied from 30 to 50 months for AS patients, resulting in a poor prognosis 3 . Previous studies have demonstrated that AS patients with elder age 4 , prior radiation therapy 5 , tumor size > 5cm, primary site from Liver 6 related to a worse prognosis. However, these factors can only serve as a single predictive indicator, which led to limited effect in comprehensively assessing the prognosis of AS.…”

Section: Introductionmentioning

confidence: 98%

Prognostic Nomograms for Predicting Overall Survival and Cancer-Specific Survival in Patients with Angiosarcoma, a SEER population-based study

Jiang¹,

Ye²,

Shao³

et al. 2021

Preprint

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Backgrounds: Angiosarcoma (AS) is a kind of highly aggressive cancer with high occurrence and mortality rates. This study aimed to establish a comprehensive and validated prognostic nomogram with various clinical indicators in patients with AS.Methods: Data of patients with AS diagnosed after surgery between 2010 and 2015 was retrieved from the Surveillance Epidemiology and End Results (SEER) database. Univariate and multivariate Cox proportional hazards regression analysis were performed to identify the independent prognostic factors associated with survival to construct the predictive nomogram of 3- and 5-year overall survival (OS) and cancer-specific survival (CSS) rates. Concordance-index(C-index), calibration plots and receiver operating characteristic (ROC) curves were applied to evaluate the predictive ability of the nomograms. The further decision curve analysis (DCA) was drawn to confirm the clinical usefulness of the nomograms.Results: 323 patients in total with AS were divided into the training group (N =226) and the validation group (N = 97). After the multivariate Cox regression analysis, gender, age, AJCC stage group 7th ed, T, N and M stage 7th ed, histologic grade and primary site were statistically identified as independent factors with OS and CSS (P<0.05). The C-index of the nomograms for OS and CCS in the training cohort was 0.760 (95%CI: 0.674–0.847) and 0.793 (95%CI: 0.687–0.898), meanwhile, the C-index of those in the validation cohort was 0.790 (95%CI: 0.725–0.855) and 0.888 (95%CI: 0.799–0.976) respectively. The results of calibration plots and ROC curve showed the nomograms qualified to measure the risk and prognosis. DCA exhibited good clinical utility of nomograms.Conclusion: Our study has developed novel and practical nomograms for predicting prognosis in patients with AS contributing to cancer management.

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Clinical Outcomes and Prognostic Features of Angiosarcoma: Significance of Prior Radiation Therapy

Cited by 16 publications

References 33 publications

Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas

Prognostic Factors in a Large Nationwide Cohort of Histologically Confirmed Primary and Secondary Angiosarcomas

The treatment landscape of advanced angiosarcoma in Asia—A multi‐national collaboration from the Asian Sarcoma Consortium

Prognostic Nomograms for Predicting Overall Survival and Cancer-Specific Survival in Patients with Angiosarcoma, a SEER population-based study

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