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EPITHELIOID HEMANGIOENDOTHELIOMA
Epithelioid
hemangioendothelioma is a malignant vascular tumour which
was described by Enzinger and Weiss in 1982.
The tumour occupies an
intermediate position in the spectrum of epithelioid vascular tumours
lying between the benign
epithelioid hemangioma and the more
aggressive
epithelioid angiosarcoma.
Site:
Soft tissue, lung ( formerly known as intravascular bronchioalveolar tumour), pleura, liver, peritoneum, bone, lymph
nodes and skin.
Age:
Middle-aged adults.
Microscopic features:
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Poorly defined and
infiltrative tumour, characterized by nests and cords of
spindle to epithelioid cells embedded in a hyaline, myxoid, chondroid
or collagenous stroma.
Diagnostic feature:
The cells show prominent cytoplasmic vacuoles. Red blood cells may be present within
some of these vacuoles (reminiscent of primitive vascular channels). These
vacuoles are mucin negative.
Other features that may be
present : Abundant osteoclast-like giant cells ;
dystrophic calcification; metaplastic ossification.
There is a low mitotic
activity and mild to moderate pleomorphism.
In
some cases there are solid nests of tumour cells displaying cytological atypia together with increased mitotic activity
(more than 1 per 10 HPF) and areas of necrosis. Cases with these
features are associated with bad prognosis.
Variant:
Epithelioid sarcoma-like
hemangioendothelioma. Am
J Surg Pathol.
2003;27(1):48-57
Immunohistochemistry: Tumour
cells express endothelial markers : CD31 is one of the most sensitive
immunohistochemical markers. von Willibrand factor is also
positive.
Some cases show positivity
for smooth muscle actin and cytokeratin. EMA is negative unlike other epithelial tumours.
S-100 is also negative.
The tumour has a good
overall prognosis but local recurrences and distant metastases can
develop.
Differential diagnosis :
Primary or metastatic
carcinoma ;
epithelioid sarcoma
(Positive for keratin, EMA and often CD34 ; Negative for CD31
or von Willibrand factor);
myxoid liposarcoma (branching
vascular pattern and small multivacuolated lipoblasts) ;
myxoid chondrosarcoma
(lobular architecture; absence of cytoplasmic vacuoles and
S-100 positive).
Characteristic morphological features
together with appropriate immuno- histochemical staining helps in
establishing the diagnosis.
Abstracts:
Primary pleural epithelioid
haemangioendothelioma with metastases to the skin. A case report and
literature review.J
Clin Pathol.
2005;58(1):107-9
Right upper quadrant pain and
fever in a 41-year-old man. Epithelioid hemangioendothelioma of the
liver with metastasis to porta hepatis lymph nodes and lung.Arch
Pathol Lab Med.
2005;129(5):e134-5.
Incidental in vivo detection
of an epithelioid hemangioendothelioma of the mitral valve.Pathol
Int. 2005;55(10):644-8
Cutaneous epithelioid
hemangioendothelioma.Ann
Dermatol Venereol.
2004 ;131 (8-9):818-21.
A clinicopathologic study on 9
cases of epithelioid hemangioendothelioma.
Zhonghua Bing Li Xue Za Zhi.
2001;30(3):177-9. |
