Primary intracranial epithelioid hemangioendothelioma: a low-proliferation tumor exhibiting clinically malignant behavior

Abstract

Epithelioid hemangioendothelioma is an extremely rare intracranial tumor and is regarded as a low-proliferation tumor. We present two cases of primary intracranial epithelioid hemangioendothelioma and give an overview of the English literature pertaining to this disease. We described two new cases of primary intracranial epithelioid hemangioendothelioma and performed a search of MEDLINE (PubMed) using the words "epithelioid hemangioendothelioma". Only cases in the English language that were intracranially located and contained clinical information pertinent to the analysis were included. The tumor in case 1 originated from the right temporal bone and invaded the surrounding cranium, dura and temple muscles. The tumor in case 2 was located in the petroclival bone and had also invaded the surrounding cranium. Both tumors were well vascularized. The tumors were totally (case 1) or subtotally (case 2) removed with moderate blood loss. A total of 36 cases of intracranial epithelioid hemangioendothelioma were found in the literature. The tumor was typically diagnosed in young adults and infants. There was no sex predominance in adult patients, while in children, males were more frequently affected (M/F ratio, 3.5:1). Surgical removal was the main therapeutic protocol, and adjuvant therapy included radiotherapy or chemotherapy. Including the 2 patients presented here, a total of 38 patients were analyzed: 32 % showed local invasion, 24 % suffered a recurrence, 15 % had metastases, and the mortality rate was 15 %. Intracranial epithelioid hemangioendothelioma is a rare, low-proliferation tumor, but it exhibits some clinically malignant behaviors, such as local invasion, recurrence and metastasis. Total resection is mandatory where possible, and radiotherapy and/or chemotherapy are otherwise required. Preoperative feeding-artery embolization is recommended.

Computed tomography and magnetic resonance features of intracranial hemangioendothelioma: A study of 7 cases

“…The tumor is rare, and various clinical, radiological and pathological features have been sporadically reported (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16). To the best of our knowledge, the 7 intracranial HE cases enrolled in the current study represent the largest documented case series.…”

“…Histological examination revealed scattered small hemorrhages and thromboses in these tumors. Zheng et al (2) reported hemorrhage in approximately one-quarter of intracranial HE lesions. Moreover, Ibarra et al (15) proposed that the high signal in the T1-weighted images is due to the methemoglobin component of the intravascular thrombi.…”

“…As intracranial HE is seldom encountered in clinical practice, considerable confusion exists with regard to its correct diagnosis and management. To date, numerous studies have focused on the histogenetic, histopathological and clinical characteristics of HE (2)(3)(4)(5)(6)(7)(8)(9)(10)(11); by contrast, there have been only scattered case studies that have described the imaging findings (12,13). The purpose of the present study was to analyze the computed tomography (CT) and magnetic resonance imaging (MRI) characteristics of intracranial HE, and to describe the pathological features.…”

“…HE involving intracranial structures occasionally results in serious local compressive symptoms, including cranial nerve palsy or a potentially fatal increase in intracranial pressure. Despite the low proliferation indices, the clinical course of intracranial HE can be complicated (2). A total resection is essential where possible, otherwise radiotherapy and/or chemotherapy are required (2).…”

“…Despite the low proliferation indices, the clinical course of intracranial HE can be complicated (2). A total resection is essential where possible, otherwise radiotherapy and/or chemotherapy are required (2). Pre-operative embolization of the feeding-artery is recommended.…”