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.
- SourceAvailable from: Prof. Dr. Giovanni Tuccari[Show abstract] [Hide abstract]
ABSTRACT: A number of meningeal neoplastic lesions may radiologically and clinically simulate meningioma. In the present paper, we review meningeal non-meningothelial tumors which may also mimic different histotypes of meningioma at the histological examination. Awareness that these lesions exist may facilitate their recognition and correct diagnosis, which is of fundamental importance for prognosis and an appropriate therapeutic approach. Histological and immunohistochemical clues for the differential diagnosis are discussed.Pathology - Research and Practice 08/2012; 208(10):567-77. · 1.56 Impact Factor
- [Show abstract] [Hide abstract]
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
- [Show abstract] [Hide abstract]
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
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.
1. Adler B, Naheedy J, Yeager N, Nicol K, Klamar J : Multifocal epithelioid
hemangioendothelioma in a 16-year-old boy. Pediatr Radiol 35 :
2. Baehring JM, Dickey PS, Bannykh SI : Epitheloid Hemanigoendo-
thelioma of the Suprasellar Area : A Case Report and Review of the
Literature. Arch Pathol Lab Med 128 : 1289-1293, 2004
3. Bhagavan BS, Dorfman HD, Murthy MS, Eggleston JC : Intravascular
bonchiolo-alveolar tumor (IVBAT) : a low-grade sclerosing epithelioid
angiosarcoma of lung. Am J Surg Pathol 6 : 41-52, 1982
4. Chen TC, Gonzalez-Gomez I, Gilles FH, McComb JG : Pediatric
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.
5. Chow L, Chow W, Fong DT : Epithelioid hemangioendothelioma
of the brain. Am J Surg Pathol 16 : 619-625, 1992
6. Dial DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W,
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
7. Elias KR, Ryan CK : Epithelioid hemangioendothelioma and the
elusive vacuole. Liver Transpl 9 : 310-312, 2003
8. Fryer JA, Biggs MT, Katz IA, Brazier DH, Shakespeare TP : Intracranial
epithelioid hemangioendothelioma arising at site of previously atypical
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,
10. Kleer CG, Unni KK, Mcleod RA : Epithelioid hemangioendothelioma
of bone. Am J Surg Pathol 20 : 1301-1311, 1996
11. Kubota T, Sato K, Takeuchi H, Handa Y : Successful removal after
radiotheraphy and vascular embolization in a huge tentorial epithelioid
hemangiendothelioma : a case report. J Neurooncol 68: 177-183, 2004
12. Miettinen M, Lindenmayer AE, Chaubal A : Endothelial cell markers
CD31, CD34, and BNH9 antibody to H-and Y-antigens-evaluation of
their specificity and sensitivity in the diagnosis of vascular tumors and
comparison with von Willebrand factor. Mod Pathol 7: 82-90, 1994
13. Nora FE, Schelthauer BW : primary epithelioid hemangioendothelioma
of the brain. Am J Surg Pathol 20 : 707-714, 1996
14. Palmieri G, Montella L, Martignetti A, Bianco AR : Interferon alpha-
2b at low dose as long-term antiangiogenic treatment of a metastatic
intracranial hemangioendothelioma : a case report. Oncol Rep 7 :
15. Puca A, Meglio M, Rolle M, Zannoni GF : Intracranial epithelioid
hemangioendothelioma : case report. Neurosurgery 38 : 399-401,
16. Rushing EJ, White JA, D’Alise, Chason DP, White CL III, Bigio
EH : Primary epithelioid hemangioendothelioma of the clivus. Clin
Neuropathol 17 : 110-114, 1998
17. Tammam AG, Lewis PD, Crockard HA : Cerebello-pontine angle
epithelioid hemangioendothelioma in a 4-year-old boy. Childs Nerv
Syst 13 : 648-650, 1997
18. Tancredi A, Puca A, Carbone A : Multifocal cerebral hemanigo-
endothelioma : case report and review of the literature. Acta Neurochir
(Wien) 142 : 1157-1164, 2000
19. Taratuto AL, Sevlever ZG, Saccoliti SM : Epithelioid hemangioendo-
thelioma of the central nervous system. Pediatr Neurosci 14 : 11-14,
20. Weiss SW, Enzinger FM : Epithelioid hemangioendothelioma : a
vascular tumor often mistaken for a carcinoma. Cancer 50 : 970-981,
Intracranial Epithelioid Hemangioendothelioma?SK Yeo, et al.