Incidence, demographics, and survival of malignant hemangioendothelioma in the United States
Abstract Background Malignant hemangioendothelioma is an endothelial cancer with heterogeneous clinical behavior that can range from indolent to aggressive, of which the majority are epithelioid (EHE). Its incidence and demographics have not been previously well defined in a large cohort. Methods Th...
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2023-07-01
|
| Series: | Cancer Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/cam4.6181 |
| _version_ | 1827867241798959104 |
|---|---|
| author | Kelly G. Paulson Vinod Ravi Brian P. Rubin Min Park Elizabeth T. Loggers Lee D. Cranmer Michael J. Wagner |
| author_facet | Kelly G. Paulson Vinod Ravi Brian P. Rubin Min Park Elizabeth T. Loggers Lee D. Cranmer Michael J. Wagner |
| author_sort | Kelly G. Paulson |
| collection | DOAJ |
| description | Abstract Background Malignant hemangioendothelioma is an endothelial cancer with heterogeneous clinical behavior that can range from indolent to aggressive, of which the majority are epithelioid (EHE). Its incidence and demographics have not been previously well defined in a large cohort. Methods This retrospective analysis used the US Cancer Statistics National Program of Cancer Registries – Surveillance Epidemiology End Results (SEER) combined database to identify patients in the US newly diagnosed with hemangioendothelioma between the years of 2001 and 2017 (n = 1986). Survival analyses were performed on a subset of patients within the SEER‐18 database with survival information available (n = 417). Outcomes included incidence, demographics of patients newly diagnosed with hemangioendothelioma, extent of disease at presentation, and overall survival. Results The incidence of hemangioendothelioma in the US is 0.4 cases per million person‐years. Although cases rose to 122 newly diagnosed in the year 2017 (90 EHE, 32 other hemangioendothelioma), incidence rates were stable. Skin and connective tissues were the most common presenting sites (33.4%), followed by liver (24.5%), lung (17.6%), and bone (12.5%). Median age at diagnosis was 55 years; 27.2% of patients were pediatric, adolescent, or young adult (<40 years). At presentation, 36.4% of patients had localized disease; 21.6% presented with regional and 41.7% with distant metastases. Observed survival at 3 years was 79.7%, 70.7%, and 46.0% for patients presenting with local, regional, and distant disease and most deaths occurred within the first 2 years. Conclusions Malignant hemangioendothelioma is ultra‐rare but meaningfully impacts affected patients. These data may provide benchmarks for comparison of new approaches to hemangioendothelioma therapy and highlight poor survival outcomes. |
| first_indexed | 2024-03-12T15:15:17Z |
| format | Article |
| id | doaj.art-b3ec2e3c2c0f43889a2818e3d3728470 |
| institution | Directory Open Access Journal |
| issn | 2045-7634 |
| language | English |
| last_indexed | 2024-03-12T15:15:17Z |
| publishDate | 2023-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | Cancer Medicine |
| spelling | doaj.art-b3ec2e3c2c0f43889a2818e3d37284702023-08-11T14:51:17ZengWileyCancer Medicine2045-76342023-07-011214151011510610.1002/cam4.6181Incidence, demographics, and survival of malignant hemangioendothelioma in the United StatesKelly G. Paulson0Vinod Ravi1Brian P. Rubin2Min Park3Elizabeth T. Loggers4Lee D. Cranmer5Michael J. Wagner6Medical Oncology Providence‐Swedish Cancer Institute Seattle Washington USASarcoma Medical Oncology MD Anderson Cancer Center Houston Texas USARobert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic Cleveland Ohio USAMedical Oncology Providence‐Swedish Cancer Institute Seattle Washington USAClinical Research Division Fred Hutchinson Cancer Center Seattle Washington USAClinical Research Division Fred Hutchinson Cancer Center Seattle Washington USAClinical Research Division Fred Hutchinson Cancer Center Seattle Washington USAAbstract Background Malignant hemangioendothelioma is an endothelial cancer with heterogeneous clinical behavior that can range from indolent to aggressive, of which the majority are epithelioid (EHE). Its incidence and demographics have not been previously well defined in a large cohort. Methods This retrospective analysis used the US Cancer Statistics National Program of Cancer Registries – Surveillance Epidemiology End Results (SEER) combined database to identify patients in the US newly diagnosed with hemangioendothelioma between the years of 2001 and 2017 (n = 1986). Survival analyses were performed on a subset of patients within the SEER‐18 database with survival information available (n = 417). Outcomes included incidence, demographics of patients newly diagnosed with hemangioendothelioma, extent of disease at presentation, and overall survival. Results The incidence of hemangioendothelioma in the US is 0.4 cases per million person‐years. Although cases rose to 122 newly diagnosed in the year 2017 (90 EHE, 32 other hemangioendothelioma), incidence rates were stable. Skin and connective tissues were the most common presenting sites (33.4%), followed by liver (24.5%), lung (17.6%), and bone (12.5%). Median age at diagnosis was 55 years; 27.2% of patients were pediatric, adolescent, or young adult (<40 years). At presentation, 36.4% of patients had localized disease; 21.6% presented with regional and 41.7% with distant metastases. Observed survival at 3 years was 79.7%, 70.7%, and 46.0% for patients presenting with local, regional, and distant disease and most deaths occurred within the first 2 years. Conclusions Malignant hemangioendothelioma is ultra‐rare but meaningfully impacts affected patients. These data may provide benchmarks for comparison of new approaches to hemangioendothelioma therapy and highlight poor survival outcomes.https://doi.org/10.1002/cam4.6181EHEepithelioid hemangioendotheliomahemangioendotheliomarare cancersarcomaSEER |
| spellingShingle | Kelly G. Paulson Vinod Ravi Brian P. Rubin Min Park Elizabeth T. Loggers Lee D. Cranmer Michael J. Wagner Incidence, demographics, and survival of malignant hemangioendothelioma in the United States Cancer Medicine EHE epithelioid hemangioendothelioma hemangioendothelioma rare cancer sarcoma SEER |
| title | Incidence, demographics, and survival of malignant hemangioendothelioma in the United States |
| title_full | Incidence, demographics, and survival of malignant hemangioendothelioma in the United States |
| title_fullStr | Incidence, demographics, and survival of malignant hemangioendothelioma in the United States |
| title_full_unstemmed | Incidence, demographics, and survival of malignant hemangioendothelioma in the United States |
| title_short | Incidence, demographics, and survival of malignant hemangioendothelioma in the United States |
| title_sort | incidence demographics and survival of malignant hemangioendothelioma in the united states |
| topic | EHE epithelioid hemangioendothelioma hemangioendothelioma rare cancer sarcoma SEER |
| url | https://doi.org/10.1002/cam4.6181 |
| work_keys_str_mv | AT kellygpaulson incidencedemographicsandsurvivalofmalignanthemangioendotheliomaintheunitedstates AT vinodravi incidencedemographicsandsurvivalofmalignanthemangioendotheliomaintheunitedstates AT brianprubin incidencedemographicsandsurvivalofmalignanthemangioendotheliomaintheunitedstates AT minpark incidencedemographicsandsurvivalofmalignanthemangioendotheliomaintheunitedstates AT elizabethtloggers incidencedemographicsandsurvivalofmalignanthemangioendotheliomaintheunitedstates AT leedcranmer incidencedemographicsandsurvivalofmalignanthemangioendotheliomaintheunitedstates AT michaeljwagner incidencedemographicsandsurvivalofmalignanthemangioendotheliomaintheunitedstates |