Nasal cavity, paranasal sinuses, nasopharynx
Benign tumors
Nasopharyngeal angiofibroma

Editor-in-Chief: Debra Zynger, M.D.
Bin Xu, M.D., Ph.D.

Minor changes: 31 July 2020

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PubMed Search: Nasopharyngeal angiofibroma[TI] free full text[sb]

Bin Xu, M.D., Ph.D.
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Cite this page: Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed August 7th, 2020.
Definition / general
  • Locally aggressive fibrovascular neoplasm of the nasopharynx developed almost exclusively in adolescent and young male patients
Essential features
  • Nasopharyngeal location
  • Affecting adolescent or young male patients
  • Histologically composed of vasculature of various sizes and cellular fibrotic stroma with fibroblasts
Terminology
  • Juvenile nasopharyngeal angiofibroma, juvenile fibroma, angiofibroma
ICD coding
  • ICD-O: 9160/0 - angiofibroma, NOS
  • ICD-10: D10.6 - benign neoplasm of nasopharynx
Epidemiology
Sites
Etiology
  • Etiology remains unclear
  • May be hormonally affected given the predilection for adolescent male
Clinical features
Diagnosis
  • Diagnosis is typically rendered using angiography, which allows visualization of the feeding vessels and preoperative embolization
  • Given the risk of massive hemorrhage, preoperative biopsy and fine needle aspiration is strongly discouraged
Radiology description
  • Angiography allows identification of a highly vascularized mass with a central feeding vessel
Radiology images

Images hosted on other servers:
Angiography

Angiography

Prognostic factors
Case reports
Treatment
  • Complete surgical resection is the treatment of choice
  • Preoperative embolization through angiography may be performed
Clinical images

Images hosted on other servers:
Facial swelling

Facial swelling

Gross description
  • Polypoid firm mass
  • Color varies from yellow to dark red or black, depending on the extent of intraoperative hemorrhage
Gross images

Images hosted on other servers:
Polypoid, beige to brown, firm, fibrotic mass

Polypoid, beige to brown, firm, fibrotic mass

Microscopic (histologic) description
  • Benign fibrovascular lesion composed of 2 components
    • Vascular space of various sizes, ranging from dilated branching vessel of various thickness to slit-like capillaries
    • Fibrous or collagenous stroma with fibroblasts
  • Central area of the tumor is typically cellular, composed of fibroblasts or myofibroblasts with spindle, round or stellate morphology
  • Stroma can be fibrous, edematous or collagenized
  • Fibrinous thrombi may be seen in dilated vessels
  • Frequently contain (abundant) mast cells
  • Mitotic figures are usually absent
  • Reference: Chan: WHO Classification of Head and Neck Tumours, 4th Edition, 2017
Microscopic (histologic) images

Contributed by Bin Xu, M.D., Ph.D.
Polypoid mass Polypoid mass

Polypoid mass

Large caliber vessels

Large caliber vessels

Slit-like vasculature

Slit-like vasculature

Cellular stroma

Cellular stroma


Bland fibroblasts Bland fibroblasts

Bland fibroblasts

Edematous or collagenized stroma

Edematous or collagenized stroma

Beta catenin immunohistochemistry

Beta catenin

AR immunohistochemistry

AR

Cytology description
  • Given the nasopharyngeal location and the highly vascular nature of the lesion, fine needle aspiration should be avoided
Positive stains
Molecular / cytogenetics description
Sample pathology report
  • Nasopharynx, resection:
    • Nasopharyngeal angiofibroma, 1.8 cm, margin negative for tumor (see comment)
    • Comment: Immunostain shows that the tumor is focally positive for AR. There is abnormal nuclear accumulation of beta catenin. The immunoprofile supports the diagnosis.
Differential diagnosis
  • Hemangioma:
    • May affect both genders, any age and is not limited to nasopharynx
    • Lacks cellular stroma enriched with fibroblasts
    • Vessels are typically uniform in size, whereas angiofibroma usually contains central large caliber vessels and peripheral slit-like vascular space
    • Does not show AR immunopositivity or nuclear beta catenin
  • Inflammatory sinonasal polyp:
    • May contain fibrous to edematous stroma but usually hypocellular
    • Lack rich vasculature seen in angiofibroma
    • Does not show AR immunopositivity or nuclear beta catenin
    • Occurs in nasal cavity or paranasal sinuses, rather than nasopharynx
  • Nasal turbinate:
    • Normal nasal turbinate is vascular rich, containing large caliber blood vessels with muscle wall
    • Lacks hypercellular stroma and slit-like vasculatures of angiofibroma
Board review style question #1

    A 15 year old boy underwent resection of nasopharyngeal mass. Which of the following statements is true?

  1. It is commonly S100+ and desmin+
  2. It is locally aggressive with 20% risk of local recurrence
  3. It is typically diagnosed preoperatively using endoscopic biopsy
  4. It often harbors PAX3 translocation
Board review answer #1
B. It is locally aggressive with 20% risk of local recurrence

Reference: Nasopharyngeal angiofibroma

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Board review style question #2
    Which of the following statements of nasopharyngeal angiofibroma is true?

  1. It generally affects adolescent women
  2. It has a hormonal profile of ER+, PR+, AR+
  3. It is a benign lesion that can be safely managed by long term observation even when large
  4. The most frequent molecular alteration in this lesion is CTNNB1 somatic mutation
Board review answer #2
D. The most frequent molecular alteration in this lesion is CTNNB1 somatic mutation

Reference: Nasopharyngeal angiofibroma

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