- Department of Neurosurgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan
- Department of Radiology, Graduate School of Medicine and Faculty of Medicine, University of Tokyo, Tokyo, Japan
Correspondence Address:
Soichi Oya
Department of Neurosurgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan
DOI:10.4103/2152-7806.166799
Copyright: © 2015 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.How to cite this article: Tsuchiya T, Oya S, Mori H, Matsui T. Multiple hemorrhagic intraparenchymal tumors presenting with fatal intracranial hypertension: A rare manifestation of systemic epithelioid hemangioendothelioma. Surg Neurol Int 06-Oct-2015;6:156
How to cite this URL: Tsuchiya T, Oya S, Mori H, Matsui T. Multiple hemorrhagic intraparenchymal tumors presenting with fatal intracranial hypertension: A rare manifestation of systemic epithelioid hemangioendothelioma. Surg Neurol Int 06-Oct-2015;6:156. Available from: http://surgicalneurologyint.com/surgicalint_articles/multiple-hemorrhagic-intraparenchymal-tumors-presenting-with/
Abstract
Background:Epithelioid hemangioendotheliomas (EHE) is an extremely rare tumor that can arise not only intracranially but also systemically. Its radiological characteristics and the mechanism underlying the multiple organ involvement in EHE are poorly understood.
Case Description:A 24-year-old woman with a 7-month history of coughing and blood-stained sputum complained of visual disturbance in the right eye that had persisted for 1-month. Magnetic resonance (MR) imaging revealed multiple intraparenchymal masses with low-intensity on MR susceptibility-weighted images with minimal enhancement with gadolinium. Systemic computed tomography revealed multiple nodules in both lungs and the liver. Because her neurological status rapidly deteriorated, brain biopsy of the right frontal mass was performed. The pathological diagnosis was EHE. Over the following 3 months, the patient gradually developed disturbance of consciousness. She died at 4 months after admission because of significant intracranial hypertension.
Conclusion:Although intracranial EHEs are extremely rare, they should be included in the differential diagnoses of multiple small-sized masses with low-intensity on MR susceptibility-weighted images. We also emphasize that the systemic involvement of this tumor was more compatible with multicentric development than metastasis.
Keywords: Epithelioid hemangioendothelioma, hemorrhagic brain tumor, liver tumor, multiple intracranial mass, pulmonary tumor
INTRODUCTION
Epithelioid hemangioendothelioma (EHE) is a rare tumor of vascular endothelial origin with an epithelioid appearance.[
We describe a case of a 24-year-old female who presented with myriad intracranial small lesions resembling hematomas on computed tomography (CT) scans and magnetic resonance images (MRIs). She was also found to have similar small lesions in the lungs and liver. Although the histology of the biopsied specimen showed benign features with a low proliferation index, her clinical course was very rapid due to intracranial hypertension caused by multiple intracranial masses.
CASE REPORT
A 24-year-old woman with no remarkable previous medical history had a 7-month history of coughing and blood-stained sputum. She also presented with a visual disturbance in the right eye occurring over 1-month. She visited a local ophthalmologist and was referred to an ophthalmologist at our hospital. Detailed examinations revealed no ophthalmological abnormality. Brain MRI showed multiple intracranial lesions and she was referred to us. On examination, she was awake and alert with no disorientation except for visual deficits rated as counting fingers in the right eye, whereas the left visual acuity was 30/50. There were no other focal neurological deficits. A brain CT scan revealed multiple 5–15 mm high-density nodules with perifocal edema in the bilateral cerebrum and cerebellum [
Figure 1
(a) Brain computed tomography scan showed multiple small intra-axial nodules with slightly high density. (b) Magnetic resonance imaging demonstrated that the signal of the nodules was hypointense to isointense on the T1-weighted image. (c) The magnetic resonance T2-weighted image revealed significant edema around multiple nodular lesions showing hypointensity. (d) The magnetic resonance susceptibility-weighted image showed numerous low-intensity spots, indicating old hemorrhages. (e) Magnetic resonance T1-weighted image with gadolinium enhancement demonstrated little enhancement in most lesions but a weak enhancement in some nodules (arrow)
During the course of these examinations, the patient's condition significantly progressed. Her bilateral visual acuity rapidly declined for 3 days after admission. She also had frequent general clonic seizures. The steroid, osmotic diuretics, carbamazepine, and levetiracetam were administered to control her seizures. Although her seizures were controlled over the following 3 days, her bilateral visual acuity had further declined to light perception. Because of the necessity of determining pathological diagnosis, a biopsy of the right frontal brain lesion was performed 14 days after admission. Increased intracranial pressure was observed intraoperatively. The lesion with slight enhancement on MRIs in the right frontal lobe was removed using a navigation guide. The tumor was moderately hemorrhagic, presenting a reddish-brown color [
Histopathological examination showed a diffuse cellular proliferation upon hematoxylin and eosin stain. Fine vascular channels, hemorrhage, and hemosiderosis were observed in the tissue [
Figure 4
(a) Hematoxylin and eosin staining showing diffuse cellular proliferation, fine vascular channels, hemorrhage, and hemosiderosis. (b) Cells with a round and slightly coarse nucleus and a large volume of clear cytoplasm including large and small balloon-like lesions and erythrocytes were noted. (c) Immunostaining for CD31 revealed a significant staining of the cell membrane and cytoplasm. (d) The MIB-1 labeling index was <3% in the tumor cells
The patient's conditions were stable for 3 months after biopsy. After a discussion about the treatment strategy with the patient and her family, we opted for conservative therapy comprising only rehabilitation on the basis of the low proliferation rate indicated by the histopathological findings. However, after 3 months, the patient gradually developed moderate disturbance of consciousness, headache, and vomiting. MRIs demonstrated aggravation of peritumoral edema without apparent enlargement of each nodule [
The autopsy revealed the cause of death as brainstem necrosis, possibly induced by brain herniation. In addition to the multiple intracranial, pulmonary, and hepatic lesions confirmed on diagnostic imaging, multiple white nodules were observed, including two in the spleen, two in the left kidney, one in the right kidney, and one in the third lumbar vertebra. All were confirmed as EHE.
DISCUSSION
The term EHE was coined by Weiss and Enzinger in 1982 to designate a vascular tumor with an epithelioid appearance.[
On radiological examination, intracranial EHE can be extra- or intra-axial, with a variable size.[
The mechanism underlying the multiple organ involvement in EHE is poorly understood. Two hypotheses have been proposed; metastasis[
The clinical course of EHE is also variable. Some reports describe cases with no progression after a long follow-up period,[
Although the treatment strategy for EHE has not been well-established because of its rarity, most previous reports advocate surgical resection for intracranial lesions that are solitary and completely resectable.[
CONCLUSION
EHE is rare and can present as solitary or multiple intracranial masses. Although the diagnosis is not simple, radiological features indicating a mixture of old and new hemorrhages inside tumors are helpful. It should be included in the differential diagnoses, particularly when multiple intracranial, pulmonary, and hepatic lesions are observed. The systemic involvement of this tumor was more compatible with multicentric development than metastasis. The clinical manifestation of EHE can be rapid and aggressive regardless of a low proliferative potential.
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Conflicts of interest
There are no conflicts of interest.
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