Angiomyofibroblastoma

 Cells characteristically cluster around thin-walled capillaries



• In postmenopausal patients, cells are more spindled, and overall cellularity is low

• Mature adipose tissue component in minority of cases

• Some cases show morphologic overlap with cellular angiofibroma




Ancillary Tests




• Desmin (+), ER(+), PR(+)

• Usually CD34(-) and SMA(-)


Top Differential Diagnoses




• Deep (aggressive) angiomyxoma

• Cellular angiofibroma

• Fibroepithelial stromal polyp

image
AMFB at Low Magnification
Angiomyofibroblastoma (AMFB) is a distinctive, benign neoplasm of the lower female genital tract. At low magnification, the classic morphologic pattern is that of irregular zones of cellularity within a myxoid or fibrous stroma.


image
AMFB With Fibrous Areas
Some areas of AMFB are less myxoid and more fibrous, as seen here. Note the sharp circumscription image, a feature seen in most examples.

image
AMFB With Alternating Zones of Cellularity
The neoplastic cells of AMFB are characteristically clustered around small, thin-walled capillary channels image, which can often be recognized by the presence of intraluminal erythrocytes. The intervening myxoid stroma is hypocellular.

image
Perivascular Organization in AMFB
In classic cases of AMFB, the tumor cells are epithelioid, ovoid, or plasmacytoid-appearing, and form small nests or clusters around capillary channels. This perivascular orientation is characteristic of this tumor.


TERMINOLOGY


Abbreviations




• Angiomyofibroblastoma (AMFB)


Definitions




• Benign genital stromal tumor composed of fibroblasts oriented around a prominent capillary vasculature


ETIOLOGY/PATHOGENESIS



Cell of Origin


 



CLINICAL ISSUES


Epidemiology




• Age
image Generally 35-60 years (median: 46 years)

• Sex
image Affects women almost exclusively


Site




• Vulva (most common) and vagina

• Rarely perineum or inguinal region


Presentation




• Slow-growing, painless mass
image Often mistaken clinically for Bartholin cyst


Treatment




• Simple local excision


Prognosis




• Benign

• Very rare recurrences reported


MACROSCOPIC


General Features




• Well-circumscribed solid mass

• Tan-white coloration

• Rubbery or mucoid cut surface


Size




• Usually < 5 cm


MICROSCOPIC


Histologic Features




• Nonencapsulated
• Alternating zones of cellularity

image Hypocellular zones often myxoid or edematous

• Prominent vascular component
image Numerous thin-walled capillaries

• Plump epithelioid, ovoid, or plasmacytoid tumor cells with eosinophilic cytoplasm
image Singly or in cords or small nests

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Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Angiomyofibroblastoma

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