Clinico-pathologic Evaluation of Patients with Hemangioendothelioma: A 10-year Experience from Shiraz, Southern of Iran

Abstract

Background: Hemangioendothelioma (HE) represents a group of rare, borderline malignant vascular tumors with diverse clinical presentations and diagnostic challenges. This study aims to investigate the demographic, clinical, radiological data, histological findings, treatment modalities, survival rates, and other key characteristics of HE cases in both pediatric and adult patients. Materials and Methods: This is a case series cross-sectional study conducted at Namazi Teaching Hospital and Abuali Sina Hospital, Shiraz University of Medical Sciences. The study involved a retrospective review of medical records from 2009 to 2019, focusing on 22 patients diagnosed with HE. Data were collected using a standardized collection form. For precise histological examination, tissue slides were extracted and analyzed, incorporating pathological variables and immunohistochemical markers. Results: In this study, a total of 22 patients diagnosed with HE was examined. Their mean age was 40.6 years with male-to-female ratio of 12:10. The majority of cases in this study were diagnosed with hepatic epithelioid HE (59%). The most commonly reported symptoms included abdominal pain and palpable masses. Radiologically, the tumors primarily appeared as hypo-dense lesions, often accompanied by peripheral rim enhancement. Pathologically, the features such as intracytoplasmic vacuolization, eosinophilic cytoplasm, and specific markers (CD31, CD34) were crucial in accurate diagnosis. Importantly, the study also revealed a positive outcome in terms of patient survival, with only 10% of surgically treated patients succumbing to the disease. Conclusion: This study provides a detailed clinico-pathologic evaluation of HE from Southern Iran, underscoring the critical relationship between tumor location and clinical presentation. The research highlights that HE, though rare, can occur in pediatric and adults and must be included in the differential diagnosis of vascular lesions across all age groups. From a therapeutic perspective, surgical intervention emerged as the most effective treatment modality, associated with a notably high survival rate of 90% among operated patients.