Last revised by Joachim Feger on 13 Sep 2022

Angiomyofibroblastomas are benign mesenchymal neoplasms usually found in the pelvis or perineum, especially the vulva.

Angiomyofibroblastomas are uncommon tumors predominantly found in adult women usually between menarche and menopause. Approximately 10% of these tumors have been described in postmenopausal women. In men, they are rare 1.

The diagnosis of angiomyofibroblastoma is established histologically 1.

Diagnostic criteria according to the WHO classification of tumors: soft tissue and bone (5th edition) 1:

  • prominent well-defined stromal vessels
  • round to spindle-shaped frequently multinucleated perivascular tumor cells

The following criterion is desirable:

The usual complaint is a slowly progressive well-defined painless mass 1

Angiomyofibroblastomas are well-circumscribed myofibroblastic lesions characterized by prominent stromal vessels surrounded by round to ovoid or spindle-shaped tumor cells 1-4.

The etiology of angiomyofibroblastoma is unknown 1.

Most angiomyofibroblastomas are found in the perineal region with most being located in the vulva or perineum followed by the vagina and uterine cervix. They can occur in other regions for example in the mediastinum or nasal cavity but only on very rare occasions 1-4.

Macroscopically angiomyofibroblastomas are well-demarcated lesions, they are not encapsulated and some might be pedunculated. They usually have a soft consistency and are of pink-tan color 1.

The histological appearance of angiomyofibroblastomas is characterized by the following  features 1-4:

  • thin fibrous pseudocapsule
  • prominent thin-walled ectatic vessels in an edematous matrix
  • round to ovoid epithelioid or spindle-shaped tumor cells with eosinophilic cytoplasm
  • well-differentiated adipocytic components in about 10% of cases
  • degenerative nuclear atypia
  • rarely morphological overlap with aggressive angiomyxoma

Immunohistochemistry stains express vimentin and are usually positive for desmin, unless in postmenopausal women.   There is only a focal expression of smooth muscle actin (SMA) and muscle-specific actin (MSA). In addition tumor cells usually express estrogen receptors, progesterone receptors and occasionally CD34 1.

Angiomyofibroblastomas are usually solid well-demarcated vascularized lesions.

There is some variability concerning ultrasound findings. On one hand, angiomyofibroblastomas have been described as solid-cystic-appearing lesions with inhomogeneous echogenicity on the other hand homogeneous appearing lesions have also been described. Color Doppler imaging usually displays intralesional or peripheral vascularization 2-4.

Angiomyofibroblastomas appear as solid well-circumscribed variably heterogeneous soft tissue masses 2-4.  

  • T1: low to intermediate signal intensity
  • T2: heterogeneous mixed to high signal intensity
  • T1 C+ (Gd): intense enhancement with variable heterogenicity

The radiological report should include a description of the following:

  • location and size of the tumor
  • relation to other organs
  • relation to soft tissue structures structures

The main treatment is surgical excision.  Angiomyofibroblastomas are benign and have an excellent prognosis with local recurrences being reportedly rare even with marginal excision 1,2,6

Angiomyofibroblastoma was initially described by the British pathologist Christopher DM Fletcher and colleagues in 1992 2-5,8.

Conditions with similar clinical presentation or appearance of angiomyofibroblastomas include 1-4:

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