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August 2009 – Case of the Month #5
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(1) We have a new shortcut URL - Dermout.net (dermout.com was already taken).
(2) We have updated Soft Tissue Tumors Part 3 - Smooth Muscle and Skeletal Muscle tumors
(3) We have reformatted all of our chapters so they are easier to update, including the Skin-Melanocytic Tumors chapter.
Dermatology Case of the Month #5
Clinical history
A 37 year old man had a skin lesion on the back, which was excised.
Micro images:
Immunostains:
CD31 - low power CD31 - high power
What is your diagnosis?
(scroll down to continue)
Diagnosis:
Retiform hemangioendothelioma
Discussion
Retiform hemangioendothelioma is a low grade variant of angiosarcoma that usually occurs in the distal extremities of young individuals. Weiss and Goldblum use the term hobnail hemangioendothelioma for retiform and Dabska-type tumors, which they believe to be closely related (Weiss: Enzinger and Weiss's Soft Tissue Tumors, 2007 (5th Ed)).
Microscopically, the reticular dermis and subcutaneous tissue have a retiform (net-like, similar to rete testis) pattern of blood vessels that disperse through the tissue, and is highlighted by endothelial markers. The vessels are lined by monomorphic hobnail endothelial cells with scant cytoplasm and rounded, naked-type nuclei. There often is a prominent lymphocytic infiltrate, as seen focally in this case. There are no epithelioid areas or cytoplasmic vacuoles (AJSP 1994;18:115). These tumors are rarely multiple (Am J Dermatopathol 1996;18:606)
The endothelial cells are immunoreactive for CD34 (strong), CD31 and vWF. They are usually negative for D2-40 (Am J Dermatopathol 2008;30:31) and keratin.
The differential diagnosis includes angiosarcoma, which may focally have low grade features, but also exhibits areas of marked atypia and pleomorphism. Angiosarcomas also dissect between individual collagen bundles and have mitotic activity. Hobnail hemangioma is a smaller, more superficial and more localized lesion, with papillary dermal vessels that disappear into the reticular dermis.
Treatment is wide local excision. These tumors are considered to have intermediate malignancy, with frequent recurrence (particularly if a wide excision was not done), but only rare metastases, and no tumor related deaths.
Nat Pernick, M.D., President
DermatologyOutlines.com
30100 Telegraph Road, Suite 408
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