CLINICAL CHARACTERISTICS AND PROGNOSTIC FACTORS OF EPITHELIOID HEMANGIOENDOTHELIOMA: A SINGLECENTER RETROSPECTIVE STUDY

Introduction: Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular neoplasm with an estimated prevalence of less than 1 case in 1 million. Due to its rarity, clinical characteristics remain unclear. There is no stablished standard treatment. Aim: We planned to evaluate the clinical characteristics and prognostic factors of EHE. Materials and Methods: Patients with EHE evaluated at our institution between January 2000 and May 2019 were enrolled. Data including demographic characteristics, treatment patterns, pathological results, and survival were gathered from electronic clinical charts, and a retrospective database was constructed for analysis. The primary endpoint was overall survival (OS). Descriptive statistics was used to report population characteristics, OS was calculated with the Kaplan-Meier method, and Fishers exact test was used for associations analysis. Results: Eighteen patients were included. Diagnosis was based on histopathological evaluation and immunohistochemical positivity for CD31, CD34 and factor VIII. The mean age at diagnosis was 41.8 years (17 85). The anatomical site of the primary lesions was visceral in 7 pts. Seven pts (38.8%) had metastasis at diagnosis and the main site of metastatic disease were the lungs (71.4%). Two pts with localized disease evolved with metastatic disease after local treatment; the progression-free survival was 11 and 52.1 months. Seven pts had surgical treatment; 2 pts received adjuvant chemotherapy and 4 pts received adjuvant radiotherapy. Three pts had data regarding first line chemotherapy and included pazopanib, paclitaxel and gemcitabine. After a median follow-up of 51.7 months, median OS for non-metastatic pts at diagnosis was 63.4 months and 18.8 months for metastatic pts (p = 0.348). Median OS for pts with visceral primary disease was 17 months and 63.4 months for pts with non-visceral primary disease (p = 0.025). Visceral primary disease was significantly associated with presence of metastasis at diagnosis (p = 0.002). Conclusion: EHE can occur in any site of the body. Surgery is the primary choice of treatment. Metastasis at diagnosis and visceral primary disease are associated with poor prognosis.

 

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Die Autoren geben an, dass kein Interessenkonflikt besteht.

Publikationsverlauf

Artikel online veröffentlicht:
23. Oktober 2019