Vulva, vagina & female urethra
Mesenchymal neoplasms
Cellular angiofibroma


Minor changes: 9 November 2020

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PubMed Search: Cellular angiofibroma vulva

David B. Chapel, M.D.
Jennifer A. Bennett, M.D.
Page views in 2019: 2,361
Page views in 2020 to date: 2,224
Cite this page: Chapel DB, Bennett J. Cellular angiofibroma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vulvacellularangiofibroma.html. Accessed December 5th, 2020.
Definition / general
  • Cellular angiofibroma is a benign, site specific soft tissue tumor of the lower genital tract
Essential features
  • Benign lower genital tract tumor, occurring equally in women and men
  • Bland spindle cell fascicles, abundant medium sized hyalinized vessels and wispy stromal collagen
  • Characterized by deletion of RB1 / FOXO1 locus on chr 13q
  • Local excision curative; recurrences exceptionally rare
ICD coding
  • ICD-10:
    • D21.9 - benign neoplasm of soft tissue
    • D23.9 - benign neoplasm of skin
    • D28.0 - benign neoplasm of vulva
Epidemiology
Sites
Pathophysiology
  • Histogenesis unknown
Clinical features
Diagnosis
  • Diagnosis typically follows complete local excision of a clinically benign appearing mass
Prognostic factors
Case reports
Treatment
Gross description
Gross images

Images hosted on other servers:
Inguinal cellular angiofibroma excision

Inguinal cellular angiofibroma

Scrotal cellular angiofibroma excision

Scrotal cellular angiofibroma

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by David B. Chapel, M.D.
Well circumscribed tumor

Well circumscribed tumor

Hyalinized vessels Hyalinized vessels

Hyalinized vessels

Virtual slides

Images hosted on other servers:
Cellular angiofibroma of the perineum

Cellular angiofibroma of the perineum

Cellular angiofibroma of the vulva

Cellular angiofibroma of the vulva

Positive stains
Negative stains
Molecular / cytogenetics description
Molecular / cytogenetics images

Images hosted on other servers:
Karyotype and <i>RB1</i> FISH

Karyotype and RB1 FISH

<i>RB1</i> FISH

RB1 FISH

Sample pathology report
  • Vulva, mass, excision:
    • Cellular angiofibroma (3.2 cm) (see comment)
    • Comment: Margins are negative for tumor.
Differential diagnosis
  • Angiomyofibroblastoma:
    • Alternating hypo and hypercellular foci
    • Spindled to epithelioid to plasmacytoid cells, clustering around vessels
    • Vascular hyalinization not prominent
  • Spindle cell lipoma:
    • Typically affects head and neck region in men
    • Thick ropy collagen bands
    • Hyalinized vessels not prominent
  • Mammary type myofibroblastoma:
    • More fascicular spindle cells
    • Less conspicuous hyalinized vessels
    • Thick ropy collagen bands
    • Desmin and CD34 consistently co-expressed
  • Solitary fibrous tumor:
    • Characteristic staghorn / hemangiopericytoma-like vessels
    • More marked variation in cellularity
    • Thick bands of hyalinized collagen
    • STAT6 positive
  • Leiomyoma:
    • Large cigar shaped nuclei
    • Prominent eosinophilic cytoplasm
    • Fewer and larger vessels, with less prominent hyalinization
    • SMA, desmin and caldesmon positive
  • Perineurioma:
    • Lacks vascular hyalinization
    • EMA positive
Board review style question #1

A 48 year old woman presented with a painless 3.5 cm vulvar mass, which had been slowly growing for 18 months. She underwent complete local excision of a rubbery, well circumscribed mass. A representative photomicrograph is shown. Which of the following is true about this lesion?

  1. Complete local excision is considered curative
  2. Immunohistochemistry for estrogen receptor and progesterone receptor is typically negative
  3. Nuclear pleomorphism is associated with an increased risk of distant metastasis
  4. Rb protein is typically overexpressed
  5. This lesion occurs exclusively in women
Board review answer #1
A. Complete local excision is considered curative. This is a cellular angiofibroma.

Comment Here

Reference: Cellular angiofibroma
Board review style question #2

Which of the following gene loci is characteristically deleted in cellular angiofibroma?

  1. CDKN2A / p16
  2. HMGA2
  3. NF2
  4. RB1
  5. SMARCB1 / INI1
Board review answer #2
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