Epithelioid hemangioendothelioma of bone: A survival analysis of 50 cases from the SEER database (1992–2016)
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that may arise in bone. The purpose of this investigation was to determine the clinicopathological features and outcomes of osseous EHE in a large patient series, and to assess whether survival is impacted by demographics, tumor charact...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2021-04-01
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| Series: | Rare Tumors |
| Online Access: | https://doi.org/10.1177/20363613211005593 |
| _version_ | 1818435590805258240 |
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| author | Charles A Gusho Sarah C Tepper Steven Gitelis Alan T Blank |
| author_facet | Charles A Gusho Sarah C Tepper Steven Gitelis Alan T Blank |
| author_sort | Charles A Gusho |
| collection | DOAJ |
| description | Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that may arise in bone. The purpose of this investigation was to determine the clinicopathological features and outcomes of osseous EHE in a large patient series, and to assess whether survival is impacted by demographics, tumor characteristics, or treatment factors. This was a retrospective review of the Surveillance, Epidemiology and End Results (SEER) database from 1992 to 2016. Kaplan-Meier was used to estimate overall survival (OS) and disease-specific survival (DSS). A Cox regression model was used to identify prognostic factors. Fifty patients from 1992 to 2016 with a median age of 54.5 years (IQR, 37–67) were reviewed. For location, 46% ( n = 23) of tumors arose from the appendicular skeleton while 38% ( n = 19) occurred within the axial skeleton (overlapping EHE: 16%, n = 8). Of the cases with recorded treatment factors, 54.8% ( n = 23) had surgery, 26% ( n = 13) received radiation, 22% ( n = 11) were treated with chemotherapy, and 26% ( n = 13) had surgery plus radiation. The 5-year OS probability was 49.2% (95% CI, 23.6–70.6), and the 5-year DSS probability was 63.9% (95% CI, 33.0–83.5). No surgery (surgery: HR, 0.262; 95% CI, 0.07–0.9); p = 0.041) and age older than 50 years (HR, 4.117; 95% CI, 1.1–15.4; p = 0.035) were negative prognostic factors of disease-specific mortality after controlling for confounding variables. There was no association between disease-specific mortality and adjuvant or multimodal therapy. The prognosis of EHE of bone is less than favorable, and the 5-year DSS probability of 64% emphasizes the intermediate grade nature of this tumor subtype. Surgical treatment, when feasible, is associated with a better prognosis. |
| first_indexed | 2024-12-14T16:55:18Z |
| format | Article |
| id | doaj.art-f872351e716447b3b562601793beec91 |
| institution | Directory Open Access Journal |
| issn | 2036-3613 |
| language | English |
| last_indexed | 2024-12-14T16:55:18Z |
| publishDate | 2021-04-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Rare Tumors |
| spelling | doaj.art-f872351e716447b3b562601793beec912022-12-21T22:53:58ZengSAGE PublishingRare Tumors2036-36132021-04-011310.1177/20363613211005593Epithelioid hemangioendothelioma of bone: A survival analysis of 50 cases from the SEER database (1992–2016)Charles A GushoSarah C TepperSteven GitelisAlan T BlankEpithelioid hemangioendothelioma (EHE) is a rare vascular tumor that may arise in bone. The purpose of this investigation was to determine the clinicopathological features and outcomes of osseous EHE in a large patient series, and to assess whether survival is impacted by demographics, tumor characteristics, or treatment factors. This was a retrospective review of the Surveillance, Epidemiology and End Results (SEER) database from 1992 to 2016. Kaplan-Meier was used to estimate overall survival (OS) and disease-specific survival (DSS). A Cox regression model was used to identify prognostic factors. Fifty patients from 1992 to 2016 with a median age of 54.5 years (IQR, 37–67) were reviewed. For location, 46% ( n = 23) of tumors arose from the appendicular skeleton while 38% ( n = 19) occurred within the axial skeleton (overlapping EHE: 16%, n = 8). Of the cases with recorded treatment factors, 54.8% ( n = 23) had surgery, 26% ( n = 13) received radiation, 22% ( n = 11) were treated with chemotherapy, and 26% ( n = 13) had surgery plus radiation. The 5-year OS probability was 49.2% (95% CI, 23.6–70.6), and the 5-year DSS probability was 63.9% (95% CI, 33.0–83.5). No surgery (surgery: HR, 0.262; 95% CI, 0.07–0.9); p = 0.041) and age older than 50 years (HR, 4.117; 95% CI, 1.1–15.4; p = 0.035) were negative prognostic factors of disease-specific mortality after controlling for confounding variables. There was no association between disease-specific mortality and adjuvant or multimodal therapy. The prognosis of EHE of bone is less than favorable, and the 5-year DSS probability of 64% emphasizes the intermediate grade nature of this tumor subtype. Surgical treatment, when feasible, is associated with a better prognosis.https://doi.org/10.1177/20363613211005593 |
| spellingShingle | Charles A Gusho Sarah C Tepper Steven Gitelis Alan T Blank Epithelioid hemangioendothelioma of bone: A survival analysis of 50 cases from the SEER database (1992–2016) Rare Tumors |
| title | Epithelioid hemangioendothelioma of bone: A survival analysis of 50 cases from the SEER database (1992–2016) |
| title_full | Epithelioid hemangioendothelioma of bone: A survival analysis of 50 cases from the SEER database (1992–2016) |
| title_fullStr | Epithelioid hemangioendothelioma of bone: A survival analysis of 50 cases from the SEER database (1992–2016) |
| title_full_unstemmed | Epithelioid hemangioendothelioma of bone: A survival analysis of 50 cases from the SEER database (1992–2016) |
| title_short | Epithelioid hemangioendothelioma of bone: A survival analysis of 50 cases from the SEER database (1992–2016) |
| title_sort | epithelioid hemangioendothelioma of bone a survival analysis of 50 cases from the seer database 1992 2016 |
| url | https://doi.org/10.1177/20363613211005593 |
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