- Department of Neurosurgery, New York University School of Medicine, New York, USA
- Department of Pathology, New York University School of Medicine, New York, USA
Correspondence Address:
James Barger
Department of Neurosurgery, New York University School of Medicine, New York, USA
DOI:10.4103/2152-7806.189729
Copyright: © 2016 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: Barger J, Tanweer O, Liechty B, Snuderl M, Jafar JJ. Suprasellar epithelioid hemangioendothelioma: Case report and review of the literature. Surg Neurol Int 01-Sep-2016;7:
How to cite this URL: Barger J, Tanweer O, Liechty B, Snuderl M, Jafar JJ. Suprasellar epithelioid hemangioendothelioma: Case report and review of the literature. Surg Neurol Int 01-Sep-2016;7:. Available from: http://surgicalneurologyint.com/surgicalint_articles/suprasellar-epithelioid-hemangioendothelioma-case-report-review-literature/
Abstract
Background:Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin, which is clinically and histologically intermediate between benign hemangioma and angiosarcoma. It is most commonly found in the liver, lung, and bone, however, 46 intracranial cases have been reported in the literature, of which this is the fifth reported suprasellar tumor.
Case Description:A 45-year-old woman developed progressive lethargy, somnolence, and memory decline over the course of 6 months. On computed tomography (CT), she was found to have a large hypothalamic mass and underwent subtotal resection via a bifrontal craniotomy.
Conclusions:While primary intracranial EHE is an uncommon presentation of a rare tumor, the suprasellar region does not seem to be an unusual location when it does occur. Prognosis is generally good, and may be better for primary intracranial disease than that for EHE originating elsewhere. Surgery is the first line of therapy, with variable benefit from adjuvant chemotherapy or radiation when total resection is not possible. Chemotherapeutic approaches in current use are directed at preventing endothelial proliferation.
Keywords: Epithelioid hemangioendothelioma, intracranial, suprasellar, review, vascular tumor
INTRODUCTION
Epithelioid hemangioendothelioma (EHE) is an uncommon neoplasm of vascular origin which may arise in a number of locations; most frequently the liver, lungs, and bones but also intracranially.[
CASE DESCRIPTION
Over the course of six months, a 45-year-old Vietnamese woman with a history of type 2 diabetes mellitus and hyperlipidemia became progressively lethargic, somnolent, and forgetful. Originally thought by her physicians to have an endocrine issue, she was diagnosed with an intracranial mass on computed tomography (CT) scan when her husband found it difficult to arouse her at home and brought her to a local emergency department. She was referred to our institution for neurosurgical evaluation, at which time she was sleeping 16 hours a day, and was noted to ask the same questions repeatedly, forgetting the answers each time. She had gained 25 pounds during the months prior to the presentation. She did not complain of headaches or visual changes, and had no symptoms of diabetes insipidus. Her last menstrual period had been 3–4 years prior to presentation. Neurological exam was unremarkable [Extraocular movements were intact, visual fields were full, and there was no nystagmus. Muscle strength was 5/5 throughout, reflexes were brisk and symmetric, and gait was normal-based. There was no dysmetria or pronator drift.]
Magnetic resonance imaging (MRI) with contrast was obtained, revealing a 3.6 × 3.7 × 3.2 cm lobulated, heterogeneous, fluid-attenuated inversion recovery hyperintense, and avidly enhancing hypothalamic mass extending into the anterior third ventricle [
Pathology
Histologic examination demonstrated a predominantly epithelioid neoplasm with areas of spindled cytology with a dense inflammatory infiltrate [
DISCUSSION
Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin which is clinically and histologically intermediate between benign hemangioma and angiosarcoma. It can present at any age but most commonly presents in the fourth and fifth decades.[
Radiologically, EHEs typically demonstrate uniform contrast enhancement on CT, which in at least one case led to misdiagnosis as a meningioma.[
The clinical course of EHE is usually somewhat indolent compared to other sarcomas; overall, 5-year survival is 73% (Lau 2011) vs 35% for angiosarcoma.[
Despite the generally slow disease progression, some EHEs are quite aggressive and efforts have been made to determine prognosis based on tumor characteristics; an analysis of 49 patients with EHEs arising in soft tissue found 5-year disease-specific survival to be 59% among patients with tumor size >3 cm and >3 mitotic figures/50 HPFs and 100% for other patients.[
Treatment
The cornerstone of EHE treatment is surgical resection of the tumor. Recurrence is rare after total resection; a recurrence rate of 13% has been published.[
Given the higher long-term morbidity of radiotherapy and the lack of strong data supporting one over the other, chemotherapy is likely a better first option for adjuvant treatment than radiation. No chemotherapeutic agent has been shown to consistently effect tumor shrinkage but a number do seem to prevent further growth, albeit inconsistently. Traditionally, interferon alpha has been used; the rationale being that it inhibits endothelial growth and thus may be effective against a tumor derived from endothelial tissue.[
Radiotherapy seems to have similar rates of success; out of seven patients in the literature with intracranial EHE who received adjuvant radiotherapy, one had tumor shrinkage, three had a stable tumor, and three experienced recurrent tumor growth and symptoms.[
CONCLUSION
While primary intracranial EHE is an uncommon presentation of a rare tumor, the suprasellar region does not seem to be an unusual location when it does occur. Prognosis is generally good, and may be better for primary intracranial disease than for EHE originating elsewhere. Surgery is the first line of therapy, with variable benefit from adjuvant chemotherapy or radiation when total resection is not possible.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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