Soft tissue

Fibroblastic / myofibroblastic

Angiomyofibroblastoma



Last author update: 30 March 2023
Last staff update: 30 March 2023

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PubMed Search: Angiomyofibroblastoma

Maria Tasneem Khattak, M.B.B.S.
Nasir Ud Din, M.B.B.S.
Page views in 2022: 7,076
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Cite this page: Khattak MT, Anjum S, Ud Din N. Angiomyofibroblastoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueangiomyofibroblastoma.html. Accessed May 29th, 2023.
Definition / general
Essential features
Terminology
ICD coding
  • ICD-10: D21.9 - benign neoplasm of connective and other soft tissue, unspecified
Epidemiology
Sites
Pathophysiology
Etiology
Clinical features
Diagnosis
Radiology description
Radiology images

Images hosted on other servers:

Ultrasound scan

Transvaginal / Doppler ultrasound

Sagittal section of MRI

MRI with gadolinium contrast

Case reports
Treatment
Clinical images

Images hosted on other servers:

Left vulvar mass

Gross description
Gross images

Images hosted on other servers:

Pendunculated,
well circumscribed,
nonencapsulated
tumor

Well demarcated, yellow-white mass

Frozen section description
  • Spindle cells or cords of epithelioid cells around abundant blood vessels with thin to thick vascular walls embedded in loose stroma (Heliyon 2020;6:e04123)
Frozen section images

Images hosted on other servers:

Cords of epithelioid to spindle cells

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Nasir Ud Din, M.B.B.S.

Numerous blood vessels

Variable cellularity

Myxoedematous stroma

Stromal cells around blood vessels


Oval to spindled cells

Bland cytology

Collagenous stroma

Desmin+

ER+

Negative stains
Electron microscopy description
Molecular / cytogenetics description
Videos

Histopathology

Sample pathology report
  • Vulva, wide local excision:
    • Angiomyofibroblastoma (see comment)
    • Comment: Histological examination reveals a well circumscribed, unencapsulated tumor with alternating hypercellular and hypocellular areas set in edematous to collagenous stroma. Numerous thin walled blood vessels and bland looking spindled to epithelioid cells, arranged singly or in small nests or cords concentrating around blood vessels are seen. No mitotic activity, nuclear atypia or necrosis is noted. It is a benign condition and has been completely excised.
Differential diagnosis
Board review style question #1

A 38 year old woman presented with a gradually growing nodule in left labium majus. On physical examination, there is a well demarcated, firm, subcutaneous mass protruding from the left labium majus. Radiology shows well demarcated subcutaneous mass. Histology is shown in the image above. Which of the following is the most likely diagnosis in this case?

  1. Aggressive angiomyxoma
  2. Angiomyofibroblastoma
  3. Cellular angiofibroma
  4. Epithelioid leiomyoma
  5. Superficial myofibroblastoma
Board review style answer #1
B. Angiomyofibroblastoma. The history of slow growing subcutaneous nodule in vulvovaginal areas in this middle aged woman suggests a soft tissue lesion. In this area, the most common mesenchymal tumors include aggressive angiomyxoma, angiomyofibroblastoma and cellular angiofibroma. The alternating hypocellular and hypercellular areas with prominent blood vessels and tumor cells concentrating around these vessels seen on the photomicrograph are characteristic features of angiomyofibroblastoma. In contrast, the aggressive angiomyxoma have low cellularity with abundant hyalinized blood vessels, extravasated red blood cells and plump stromal cell scattered in mucin rich stroma. Cellular angiofibroma and superficial myofibroblastoma also lack this characteristic, alternating cellularity pattern.

Comment Here

Reference: Angiomyofibroblastoma
Board review style question #2
A 27 year old woman noticed a small gradually increasing nodule in her vulvar region. Physical examination revealed a firm, well demarcated nodule in right labium majus. Reactivity of which of the following markers would help you to reach final diagnosis?

  1. Desmin
  2. Keratin
  3. NSE
  4. SMA
  5. S100
Board review style answer #2
A. Desmin. The differential diagnosis of gradually increasing subcutaneous nodules in vulvovaginal areas in young women includes site specific mesenchymal tumors (i.e., aggressive angiomyxoma, angiomyofibroblastoma and cellular angiofibroma). The alternating areas of cellularity with prominent blood vessels and tumor cells concentrating around these vessels as seen on photomicrographs are characteristic features of angiomyofibroblastoma. This tumor commonly shows reactivity for desmin and ER / PR receptors. They are negative for keratin, NSE and S100, while SMA can be rarely positive.

Comment Here

Reference: Angiomyofibroblastoma
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