Intracranial epithelioid hemangioendothelioma.
ABSTRACT INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is an uncommon soft-tissue vascular neoplasm. Although a well-defined entity outside the neuraxis, its intracranial occurrence is rare. Literature review revealed 35 reported cases till date. CASE REPORT: The authors report a case of intracranial EHE in a 15-year-old girl, who presented with a short history and unusual radiology. Following radical removal, the lesion recurred within 1 month and progressed to terminal stages. This tumor is thought to have an indolent intermediate malignancy potential and such rapid progression has not been reported so far.
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ABSTRACT: The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival. A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols. Male sex, AS histology, tumor size >5 cm, and T2 invasiveness were independent predictors of inferior 5-year overall survival, while AS histology and T2 invasiveness were predictors of inferior 5-year event-free survival. AS histology was the most important negative prognostic factor for overall survival and event-free survival. Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis. Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses. Response to systemic treatment was poor (44%) and did not prevent local and distant relapses. The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS. RTX and delayed surgery should be performed more frequently and earlier in the disease course. An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy. The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.Annals of Surgical Oncology 03/2010; 17(7):1878-89. · 4.12 Impact Factor
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ABSTRACT: Spindle cell hemangioendothelioma is a rare benign vascular tumor that is not known to involve the sacrum. The authors describe the case of a 31-year-old woman presenting with low-back and radicular pain without weakness or bowel or bladder dysfunction. Admission CT and MRI studies revealed a large S1-3 lytic sacral lesion. The patient initially underwent a nondiagnostic percutaneous biopsy. She subsequently underwent an open biopsy, during which the lesion was found to be highly vascular. Histological investigation revealed a vasoformative lesion consistent with spindle cell hemangioendothelioma. Preoperative embolization followed by resection via intralesional currettage resulted in resolution of symptoms up to 9 months postintervention. Despite the authors' recommendation, the patient became pregnant at that time and requested no additional follow-up imaging. The authors present the first reported case of a spindle cell hemangioendothelioma of the sacrum and review the current literature.Journal of neurosurgery. Spine 05/2014; · 1.61 Impact Factor
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ABSTRACT: Epithelioid hemangioendothelioma is an extremely rare intracranial tumor and is regarded as a low-proliferation tumor. We present two cases of primary intracranial epithelioid hemangioendothelioma and give an overview of the English literature pertaining to this disease. We described two new cases of primary intracranial epithelioid hemangioendothelioma and performed a search of MEDLINE (PubMed) using the words "epithelioid hemangioendothelioma". Only cases in the English language that were intracranially located and contained clinical information pertinent to the analysis were included. The tumor in case 1 originated from the right temporal bone and invaded the surrounding cranium, dura and temple muscles. The tumor in case 2 was located in the petroclival bone and had also invaded the surrounding cranium. Both tumors were well vascularized. The tumors were totally (case 1) or subtotally (case 2) removed with moderate blood loss. A total of 36 cases of intracranial epithelioid hemangioendothelioma were found in the literature. The tumor was typically diagnosed in young adults and infants. There was no sex predominance in adult patients, while in children, males were more frequently affected (M/F ratio, 3.5:1). Surgical removal was the main therapeutic protocol, and adjuvant therapy included radiotherapy or chemotherapy. Including the 2 patients presented here, a total of 38 patients were analyzed: 32 % showed local invasion, 24 % suffered a recurrence, 15 % had metastases, and the mortality rate was 15 %. Intracranial epithelioid hemangioendothelioma is a rare, low-proliferation tumor, but it exhibits some clinically malignant behaviors, such as local invasion, recurrence and metastasis. Total resection is mandatory where possible, and radiotherapy and/or chemotherapy are otherwise required. Preoperative feeding-artery embolization is recommended.Journal of Neuro-Oncology 08/2012; 110(1):119-27. · 3.12 Impact Factor
The term epithelioid hemangioendothelioma (EHE) was proposed by Weiss and Enzinger
to nominate an unique vascular tumor with an epithelioid appearance20). The EHE was initially
believed to be a bronchiolo-alveolar tumor but was later confirmed to be of vascular origin3).
The EHE is well-differentiated endothelial tumor with variable presentation and behavior
and can occur at various sites, such as the lungs, liver, bones, head and neck, and vessels1,6,10).
Central nervous system involvement is extremely rare (?0.02% of all brain tumors) 5,11,13,16,19).
We report a case of intracranial
EHE in a 55-year-old man who
presented with dysarthria for
2 weeks. The brain computed
tomography (CT) scan and
magnetic resonance image (MRI)
showed fat containing masses
with enhancing component at
the left frontal convexity and
at left posterior parietal (Fig. 1).
Craniotomy and grossly total
resection of the left frontal mass
was performed. Removed mass
consisted of, a piece of pinkish
soft tissue, measured 3.5?3?
2 cm and the cut surface was
pinkish yellow and hemorrhagic.
Histologically, the atypical
cells forming solid nests were
scattered within the nodular
mass containing fatty tissue
and abnormal blood vessels.
The atypical cells were immuno-
positive for CD31, supporting
Intracranial epithelioid hemangioendothelioma is extremely rare. We report a case of intracranial epithelioid
hemangioendothelioma which developed in a 55-year-old man who presented with dysarthria for two weeks.
The brain computed tomography scan and magnetic resonance image showed masses which had fat component
at the left frontal convexity and at left posterior parietal area. Excisional biopsy at the left frontal convexity
confirmed epithelioid hemangioendothelioma which is immunopositive for CD31, supporting endothelial
differentiation, and negative for CD68, SMA and HMB-45.
KEY WORDS : Epithelioid hemangioendothelioma?CD31.
J Korean Neurosurg Soc 42 : ?????????????
Seok Kon Yeo, M.D.
Jeong Hoon Kim, M.D.
Chang Jin Kim, M.D.
Jung Kyo Lee, M.D.
Department of Neurological
Surgery, Asan Medical Center
College of Medicine
University of Ulsan
?Received?March 28, 2007
?Accepted?July 6, 2007
?Address for reprints?
Jeong Hoon Kim, M.D.
Department of Neurological Surgery
Asan Medical Center, College of
Medicine, University of Ulsan
Pungnap 2-dong, Songpa-gu
Seoul 138-736, Korea
Fig. 1. Brain computed tomography scan revealing a 3?2 cm sized mass
(A). Magnetic resonance image shows a fat containing extraaxial mass
with enhancing component at the left frontal convexity and another small
well-enhancing nodular lesion at the left posterior parietal (precuneus)
(B, C, D).
online ML Comm
endothelial differentiation, negative for CD68, representing
histiocytic marker, and negative for SMA and HMB-45,
supporting angiomyolipoma. Based on the histologic findings
and immunohistochemical staining, EHE was most likely
although this tumor could not be accurately classified under
the current classification of the vascular neoplasm (Fig. 2).
Central nervous system involvement of EHE is extremely
rare (?0.02% of all brain tumors) but may occur in several
different ways, as EHE can arise from the brain, dura matter,
crainum, and spine5,11,13,16,19).
The differential diagnosis of EHE includes a chordoid
meningioma, chordoma, myxoid chondrosarcoma, metastatic
carcinoma, hemanigoma, angiosarcoma, chordoid glioma,
yolk sac tumor, hemangiopericytoma and hemanigoblastoma.
Immunohistochemical profiling is useful because chordoma
is immunopositive for S100 and cytokeratin, chordoid glioma
stains for glial fibrillary acidic protein, and meningioma is
at least focally immunoreactive with epithelial membrane
antigen. Diffuse staining of epithelioid cells for endothelial
cell markers, such as CD31, factor VIII-related antigen, or
CD34, shows the endothelial nature of the neoplasm2,7,8,13,16).
EHE can occur at various sites, such as the lungs, liver,
bones, head and neck, and vessels1,6,10). Therefore, it is
important to survey not only brain but also the skeleton,
the visceral organs to determine the full extent of the disease.
Treatment options and prognosis are still controversial.
Also, unlike other aggressive vascular tumors (such as heman-
gioendothelial sarcoma or angiosarcoma), the histological
grading system is not useful for predicting prognosis1,10). If
EHE is completely removed, it is associated with a favorable
prognosis and adjuvant therapy is not needed9). Radiation
therapy, which might be used in an attempt to sclerose the
blood vessels, was attempted to restrain growth of incompletely
removed tumors8,9,15,17). The benefits of pre-and postoperative
irradiation of EHE are uncertain when total resection is
possible4,9,13,16). Treatment with ? -interferon became a
commonly used modality capable of controlling the growth
in most of the cases4,9,14,18). Therefore, radiation therapy
and/or ? -interferon have been recommended for partially
excised or surgically inaccessible lesion.
We report a case of epithelioid hemangioendothelioma
in the left frontal lobe. We performed grossly total resection
of the left frontal mass and will surgically remove mass at
left posterior parietal.
Intracranial epithelioid hemangioendothelioma extremely
rarely develops in central nervous system. Biopsy confirmed
epithelioid hemangioendothelioma which is immunopositive
for CD31, supporting endothelial differentiation, and
negative for CD68, SMA and HMB-45.
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intracranial hemangioendotheliomas : case report. Neurosurgery 30 :
J Korean Neurosurg Soc 42?August 2007
Fig. 2. The atypical cells forming solid nests are scattered within the
nodular mass, which contain abnormal blood vessels (A H&E?400).
The atypical cells are immnopositive for CD31, supporting endothelial
differentiation, and negative for CD68, SMA and HMB-45 (B CD31 staining).
Based on the histologic findings (epithelioid feature and intracytoplasmic
vacuoles containing intraluminal RBCs (arrow)) and immunohistochemical
staining (CD31 positivity), epithelioid hemangioendotheliom is most likely
although this tumor could not be accurately classified under the current
classification of the vascular neoplasm.
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of the brain. Am J Surg Pathol 16 : 619-625, 1992
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et al : Intravascular, bronchiolar, and alveolar tumor of the lung
(IVBAT) : an analysis of twenty cases of a peculiar sclerosing endothelial
tumor. Cancer 51 : 452-454, 1983
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elusive vacuole. Liver Transpl 9 : 310-312, 2003
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meningioma. Pathology 30 : 95-99, 1998
9. Hamlat A, Casallo-Quilliano C, Saikali S, Lesimple T, Brassier G :
Epithelioid hemangiendothelioma of the infundibular-hypothalamic
region : case report and literature review. J Neurooncol 67 : 361-366,
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Intracranial Epithelioid Hemangioendothelioma?SK Yeo, et al.