Primary Epithelioid Hemangioendothelioma in the Cerebellum: Case Report with Reference to Drastic Change in the WHO Classification

Abstract

SUMMARY – Epithelioid hemangioendothelioma is a rare vascular brain tumor. It develops from endothelial cells, usually in the liver, lung, bone and soft tissue. Primary localization of this tumor in the intracranial space is very uncommon; only 47 cases have been described in the literature. This tumor was initially classified as grade I (benign) in the World Health Organization ( WHO) 2007 classification. In 2016, this tumor was re-classified as grade III (malignant). Herein, the first case report of epithelioid hemangioendothelioma in the cerebellum of a male patient is presented. Complete surgical excision was done. No adjuvant therapy was administered. Magnetic resonance imaging performed 2 years after the surgery continued to show no recurrence of the tumor. To our knowledge, this is the first report of cerebellar location of this rare tumor. In addition, the authors report drastic re-classification of the epithelioid hemangioendothelioma from the benign tumor (WHO 2007) to a malignant one (2016), which significantly changes postoperative management and follow up of this brain neoplasm.

Dural composite hemangioendothelioma: The first intracranial case

“…[96] A review of the literature revealed 61 cases of primary intracranial EHE [Table 2]. [2,3,6,7,9,10,16,18,23,26,27,29,30,33,35,38,40,43,45,47,53,54,57,59,[62][63][64][65][66][67][68][70][71][72][73]76,79,[82][83][84][85][86]89,92,94,95,98,99,101,104,105] ere is a wide age range (31 weeks gestation-74 years ...…”

Long-Term Observation and Treatment of Epithelioid Haemangioendothelioma of the Mediastinum: A Case Report

“…Epithelioid haemangioendothelioma was initially believed to be an aggressive form of bronchoalveolar cell carcinoma invading adjacent blood vessels and small airways, hence the name intravascular bronchioloalveolar tumour (9,10). However, the development of pathology proved that EHE could occur anywhere in the body, such as the liver, lungs, skin, bone, spleen, pleura, lymph nodes, thyroid, parotid gland, and cerebellum (11)(12)(13). In the 2020 WHO classification of sarcomas, EHE is distinguished from other vascular tumours and defined as an independent disease (5).…”

“…The preoperative misdiagnosis of EHE is usually due to its low incidence, lack of specific oncological markers, and unclear pathogenesis (1,13). However, the improvement of medical imaging equipment and diagnostic technology has made it possible for medical imaging, including CT, MRI, and F-FDG-PET to show certain pathological characteristics of EHE (1,(6)(7)(8).…”

Intradural Extramedullary Epithelioid Hemangioendothelioma of the Thoracic Spinal Cord: A Case Report

“…Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor, accounting for less than 1% of all vascular tumors. 1 , 2) EHE is most often found in the lung, liver, and bones, and is rarely located in the skull base, dura, or brain parenchyma. 1 , 2) To date, no previous reports have described EHE of the spinal cord, although there was a report of EHE of the filum terminale.…”

“… 1 , 2) EHE is most often found in the lung, liver, and bones, and is rarely located in the skull base, dura, or brain parenchyma. 1 , 2) To date, no previous reports have described EHE of the spinal cord, although there was a report of EHE of the filum terminale. 3) The authors here report the first case of EHE of the thoracic spinal cord.…”