Nasopharyngeal
Angiofibroma
Epidemiology:
- Adolescent males
(almost exclusively)
Common sites:
- distinctive
location, arising from an area of erectile-like fibrovascular
stroma
- posterolateral wall of the roof of the nose
- where the sphenoidal process of the palatine bone meets the
horizontal ala of the vomer and the root of
the pterygoid process of the sphenoid bone
Gross features:
Histologic features:
- intricate mixture
of blood vessels and fibrous stroma
- vessels:
- range from
capillary to venous size
- “erectile tissue”
appearance
- plump endothelial
cells in small capillary-like vessels at “growing edge” of lesion
- large vessels have
irregular or incomplete smooth muscle coat and lack elastic fibers
- stroma:
- may be loose / edematous
with stellate fibroblasts and numerous mast
cells
- may be dense, acellular, highly collagenized
(diagnostic)
Immunophenotype:
Marker:
|
Sensitivity:
|
Specificity:
|
Androgen receptors (stromal and endothelial)
|
75%
|
|
Molecular features:
Other features:
- tendency to bleed profusely
on manipulation
- EM – distinctive electron-dense
granules composed of tightly bound RNA protein complexes in the nuclei of
the proliferating cells
- Some large angiofibromas regress after puberty, especially after
(incomplete) surgery or radiation