Epithelioid Hemangioendothelioma Of Bone Research Articles

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 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.

Surgical treatment and results of 62 patients with epithelioid hemangioendothelioma of bone

Purpose of this retrospective study was (1) to evaluate overall survival and survival to local recurrence of patients with unifocal and multifocal tumor and (2) to evaluate survival to local recurrence after intralesional or wide surgery. We reviewed 62 patients with epithelioid hemangioendothelioma of bone, treated from 1985 to 2010. Histological sections and immunohistochemistry were evaluated. Tumor presented as unifocal in 49 patients and as multifocal in 13. Mean follow-up was 9 years. Five patients (10%) with unifocal tumor progressed to multifocal. Overall survival was 92% at 10 years. Survival with unifocal tumor was significantly higher than survival with multifocal tumor. Survival to local recurrence was 76% at 10 years, significantly higher after wide resection than after intralesional surgery, while there was no statistical difference comparing unifocal and multifocal tumor. At multivariate analysis both variables showed no statistically significance. Wide surgical excision reduces the risk of local recurrence, but functional results and morbidity need to be considered individually when defining surgical indications. Due to the risk of radiation-induced sarcomas, radiation therapy should be reserved to those cases not amenable to wide surgery or when lesions are seated locations difficult to treat.

Multicentric epithelioid hemangioendothelioma of bone. Report of a case with radiologic-pathologic correlation

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of uncertain biologic behavior. Most cases come out as a single lesion of the soft tissue but also may appear in the lung, liver, and other locations. Epithelioid hemangioendothelioma of bone is an extremely rare tumor and more prevalent in the second and third decades of life; its behavior is uncertain, it most commonly is unifocal, and it affects preferentially lower extremities. In this work, we present the clinical, radiologic, and pathologic findings of a 19-year-old man with a multicentric EHE of bone that involved 3 vertebrae and developed lung metastasis.