A tragical paediatric case history of intraorbital and intracranial epithelioid hemangioendothelioma

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare tumor of intermediate malignancy. We report a case of intracranial and intraorbitar EHE. A 3-year-old girl presented with a 3-month history of progressive left exophthalmia. Neuroradiologic imaging (CT scan and MRI) showed an intraorbitar process with an intense enhancement extending to temporal fossa, ethmoidal bone, nasal fossa, maxillary sinus, and cavernous sinus. The angiogram was normal. The tumor was operated through subfrontal approach but only a partial resection was performed. The histological diagnosis was epithelioid hemangioendothelioma. The patient was neurologically intact 2 months after surgery without exophtalmia. However 4 months after surgery he displayed a fall of the right eye vision with intense headache. Control CT scan showed persistence of important tumoral residue. Epithelioid hemangioendothelioma is a hemorrhagic tumor. Total removal must be possible. Otherwise, we recommend a complementary chemoradiotherapy and close followup. We propose this interesting case history of a tragical evolution of EHE in contradiction with what has already been reported.

Figure 1

Photograph taken in the second hospitalisation shows exophtalmia.

Figure 2

CT scan on axial (a) and coronal (b) views with contrast administration shows an endoorbital tumor involving the optical nerve spreading to the cavernous sinus and widening the pterygopalatine fissure.

Figure 3

Magnetic resonance imaging in the axial (a, b, and e) and sagittal (c) planes showing the tumoral process in low signal in T1 (b) and high signal in T2 weighted images (e) as well as intense and homogeneous enhancement after contrast administration (a, c). The angio RMI sequence (d) shows the vascular character of the tumor (d).

Figure 4

(a) Histopathologic examination revealed pathognomonic features of a hemangioendothelioma, showing that the tumour was mainly composed of epithelioid cells which form primitive vascular channels. Some cells even showed evocating intracytoplasmic vacuoles. (b, c) Immunohistochemical stains for factor CD 34 and vimentin displayed positive reaction.

Figure 5

Photograph taken in the second hospitalisation shows the recurrence of the tumor.