Second Primary Malignancies After Initial Cutaneous Angiosarcoma: A SEER population-Based Study

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Vignesh Ramachandran
Asad Loya
Kevin Phan


cutaneous angiosarcoma, sarcoma, malignancy, cancer, SPM, SEER, database, CAS, second primary malignancy, second primary neoplasm


Introduction: The epidemiology of second primary malignancies is an under-investigated domain within dermatology. This notion is particularly true for more uncommon cutaneous oncologic diseases. While general epidemiological characteristics and survival data of patients with cutaneous angiosarcoma have been reported before, there is no investigation of the incidence and types of second primary malignancies (SPMs) that these patients face, which has relevance to screening and surveillance. 

Methods: The National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database was utilized in this study. Initial cases of CAS were extracted and analyzed using standardized incidence ratios (SIR) and excess absolute risks (EAR) for SPMs relative to a control population, which was matched by sex, race (white/unknown, black, other), age group (5-year interval), and calendar year (5-year interval). EAR was calculated per 10,000 persons. P-value <0.05 was deemed statistically significant.

Results: Compared to a matched cohort from the general population, patients with CAS demonstrated increased incidence of new malignancies (SIR 1.54; 95% CI, 1.05-2.17; EAR 107.02). Specifically, there was increased risk of soft tissue malignancies, and non-epithelial skin malignancies other than melanoma/basal cell/squamous cell.

Discussion: SPMs may be linked to many etiologies, including genetic susceptibility, treatment-related sequelae, lifestyle/environmental factors, or shared risk factors. Indefinite treatments may induce SPMs. Recently, immunotherapy/immune-modulating drugs have been used to treat CAS4; this therapy may increase the risk of SPMs via immunosuppression. Shared etiology (i.e. blood vessel or soft tissue-derived neoplasms) may also explain SPMs observed. Importantly, CAS is associated with high recurrence even after complete resection.


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2. Surveillance, Epidemiology, and End Results (SEER) Program ( SEER*Stat Database: Incidence - SEER 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, Nov 2018 Sub (2000-2016) - Linked To County Attributes - Total U.S., 1969-2017 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, released April 2019, based on the November 2018 submission.

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4. Ishida Y, Otsuka A, Kabashima K. Cutaneous angiosarcoma: update on biology and latest treatment. Curr Opin Oncol. 2018;30(2):107–112.

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