Intracranial epithelioid hemangioendothelioma: two cases report and literature review

Abstract

Background Epithelioid hemangioendothelioma is an uncommon low-grade malignant tumor with various biological behaviors. This paper retrospectively analyzed the clinical data of 2 cases with epithelioid hemangioendothelioma which were confirmed by histopathological features, and reviewed relevant literatures, so as to summarize clinical and radiological features, diagnosis and differential diagnosis, treatment and prognosis of this disease.  Methods and Results The tumor in Case 1 was cystic accompanied with hemorrhage. Hematoma clearance, decompressive craniectomy and total removal of the tumor were performed. The tumor in Case 2 was misdiagosed as meningioma firstly, and craniotomy was implemented to remove the tumor. But 8 months later, the tumor relapsed. The tumor was vascularized in operation and subtotally removed with moderate blood loss. Postoperative pathological diagnosis revealed epithelioid hemangioendothelioma in both cases. Immunohistochemical staining showed the tumor cells were positive for CD34, FⅧ RAg in Case 1, while CD31, vimentin (Vim) and Fli-1 in Case 2, and both negative for cytokeratin (CK) and epithelial membrane antigen (EMA). Case 1 was followed up for one year, and no recurrence was found. Case 2 received postoperative auxiliary radiotherapy, but tumor metastasis was found 3 months later. Conclusions Epithelioid hemangioendothelioma is a unique vascular tumor characterized by proliferation of epithelioid or histiocytoid endothelial cells. The histopathological features and biological behaviors are intermediate between hemangioma and angioscarcoma, and its intracranial occurrence is extremely rare. A preoperative diagnosis is usually difficult because of lacking characteristic clinical and radiological features. Diagnosis mainly depends on histopathology and immunohistochemistry. Gross resection is currently the firstline treatment for these tumors. Otherwise, adjuvant therapies are required. The prognosis of this disease has not yet been well defined

DOI: 10.3969/j.issn.1672-6731.2015.09.012