Spindle cell
hemangioma (hemangioendothelioma) is a distinct vascular lesion which was
initially considered to be a low grade angiosarcoma when
first described in 1986.
Spindle cell
hemangioendothelioma. A low-grade angiosarcoma resembling a cavernous
hemangioma and Kaposi's sarcoma.Am
J Surg Pathol.1986;10(8):521-30
In recent years it has been suggested that
spindle cell hemangioma is probably a vascular malformation or the lesion
results from abnormalities of local blood flow.
Spindle cell hemangioma
may be associated with anomalies such as Mafucci's syndrome,
Klippel-Trenaunay syndrome, lymphedema and early onset varicose vein.
Treatment consists of
wide local excision without adjuvant chemotherapy or radiotherapy.
Local recurrence of the lesion is common after incomplete excisions.
Age:
Spindle cell hemangiomas may occur at any age. About 50% patients are 25
years of age or younger.
Clinical presentation:
These lesions usually present as a single or multiple, often
painful, small circumscribed
reddish blue nodules.
Site:
This lesion is usually located in the dermis or subcutis of the distal extremities
(particularly the hands ). This lesion can also occur in the proximal
extremities, trunk, axilla, ear, vulva, penis, spleen, and pancreas.
Microscopic features:
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Microscopically this poorly circumscribed lesion consists of three
main components:
- Vascular component displaying gaping thin- walled vessels containing
organizing thrombi (features resemble Cavernous hemangioma).
- Solid area of spindle cells together with slit like vascular spaces
(features resemble Kaposi's Sarcoma).
- Plump endothelial cells. Some these cells contain vacuoles or
intracytoplasmic lumina.
Bundles of smooth muscle cells are present around the vascular spaces and
in the solid areas.
Immunohistochemistry:
Immunohistochemistry reveals that the cells lining the cavernous spaces
and the epithelioid cells in the solid area stain positively for
endothelial markers CD31 and factor VIII-related antigen.
The spindle cells are immunonegative for endothelial marker CD34 . Most of these spindle cells stain positively for Vimentin and
some cells are positive for actin and desmin.
Differential diagnosis:
The main differential diagnosis is with Kaposi's Sarcoma
in which cavernous spaces and epithelioid vacuolated cells are usually not
present. Unlike Spindle cell hemangioendothelioma the spindle cells in
Kaposi's sarcoma stain positively for CD34.
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