Malignant Intracranial Epithelioid Hemangioendothelioma Presumably Originating from the Lung: Case Report

OBJECTIVE AND IMPORTANCE: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that presents histological features and biological behavior of low-grade malignancy. The authors report a case of malignant intracranial EHE, in which surgical excisions and additional immuno-chemotherapy were ineffective. Emphasis is placed on the histological features of this rare tumor and its potential for malignancy. CLINICAL PRESENTATION: A 69-year old male presented with paresis of the right arm. Magnetic resonance imaging revealed intracranial multiple lesions in the right temporal and parietal and left frontal lobes. Chest radiography revealed a mass lesion occupying the lower lobe of the left lung that had been followed as old tuberculosis since it had not changed in size for as long as 5 years. INTERVENTION: We performed craniotomy and excised tumors. Histological examination demonstrated that the excised tumors had the features of EHE. Further intracranial recurrence after the excision of the tumors necessitated adjuvant treatment with interferon alpha-2b. However, the patient continued to deteriorate and died 3 months later. Postmortem study disclosed the tumor cells existing in the left lung, pleura, ribs, intercostal tissues and diaphragm. Other organs including liver, bone, and skin were free from tumor invasion, which indicated that the malignant EHE originated from the left lung and metastasized to the brain. CONCLUSION: To the authors knowledge, no case of malignant EHE that simultaneously involves the central nervous system and the lung has been previously reported. An extensive inspection for involvement of other organs is recommended after recognizing an intracranial EHE.