Atypical Vascular Lesion

 Clinically presents as large, erythematous patch (median: 7 cm)


image Endothelial multilayering and nuclear atypia

image Mitotic figures often present

image Evidence of MYC amplification by FISH or immunohistochemistry


• Hobnail hemangioma

• Lymphangioma circumscriptum

• Progressive lymphangioma (benign lymphangioendothelioma)



image
Clinical Appearance as Small Papules and Patches
An atypical vascular lesion (AVL) typically presents as small papules image and patches image in radiated skin at a median time of 3 years post radiotherapy. The breast or chest wall are most common sites. The lesions can be solitary or multiple.


image
Atypical Vascular Lesion in Superficial Dermis
Low-magnification view shows a superficially located, lymphangioma-like vascular lesion composed of dilated vascular structures lined by cytologically bland endothelial cells.

image
Higher Magnification of Superficial Atypical Vascular Lesion
Higher magnification of this superficial dermal AVL shows a proliferation of dilated vascular channels lined by small, relatively bland-appearing endothelial cells.

image
Dissecting Growth Pattern Is Typically Present
AVLs often have a dissecting growth pattern consisting of jagged, anastomosing channels that infiltrate dermal collagen; a pattern that mimics well-differentiated angiosarcoma. Unlike angiosarcoma, however, there is no significant nuclear atypia, no multilayering of cells, and rarely any mitotic activity.


TERMINOLOGY


Abbreviations




• Atypical vascular lesion (AVL)


Synonyms




• Atypical vascular proliferation


Definitions




• Benign cutaneous vascular lesion presenting as small papule or patch in radiated skin, composed of thin-walled lymphatic vessels, usually limited to dermis


ETIOLOGY/PATHOGENESIS


Environmental Exposure




• Radiation (40-60 Gy)

• Median latency: 3 years post radiotherapy


CLINICAL ISSUES


Epidemiology




• Incidence
image Exact incidence unknown

• Age
image Wide age range; median in late 50s

image Usually 1 decade earlier than radiation-associated cutaneous angiosarcoma


Site




• Skin of breast or chest wall most common site, following radiotherapy for breast cancer

• Less frequent in radiation fields associated with gynecological and other malignancies


Presentation




• Small, flesh-colored papule or erythematous patch

• Solitary or multiple

• Rarely presents as large patch

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Atypical Vascular Lesion

Full access? Get Clinical Tree

Get Clinical Tree app for offline access