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Malignant Intracranial Epithelioid
Hemangioendothelioma Presumably Originating from the Lung: Case Report
Toshiki Endo, Ching-Chan Su, Yoshihiro Numagami, Reizo
Shirane
Department of Neurosurgery, Yamagata
Prefectural Shinjo Hospital, Shinjo (T.E., C.-C.S., Y.N.); Department of
Neurosurgery, Tohoku University, School of Medicine, Sendai, Japan (R.S.)
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.
Keywords: central nervous system neoplasm, epithelioid hemangioendothelioma,
metastasis
Copyright
©
2004 Kluwer Academic Publishers.
All rights reserved
Source:
http://journals.kluweronline.com/article.asp?PIPS=5266127
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