Last revised by Rohit Sharma on 2 Sep 2023

Angioleiomyomas, also known as angiomyomas or vascular leiomyomas, are benign pericytic or perivascular soft tissue tumors.  

Angioleimyomas are rare and have been observed in a wide age range with a peak in the 4th to 6th decades of life. Generally, women are more commonly affected 1-4. They account for up to 5% of all benign soft tissue neoplasms 3.

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

  • essential: well-circumscribed tumor composed of cytologically bland smooth muscle cells showing concentric growth around vascular channels of varying caliber

The usual presentation is a firm slow-growing nodule, more than half of the tumors are associated with pain 1-3.  

Angioleiomyomas typically originate in the skin and subcutaneous tissues and mainly constitute well-differentiated perivascular smooth muscle cells aligned around multiple variably-sized vascular channels 1. There are descriptions of a fibrous pseudocapsule 2,5.

There is no clear established etiology for angioleimyomas. Venous stasis and minor trauma have been proposed as potential causative factors 1.

Angioleiomyomas are most frequently found in the subcutaneous tissues and dermis and rarely in the deep soft tissues of the following locations 1-5:

  • lower and upper extremities

  • head and neck region

  • trunk

Macroscopically angioleiomyomas are usually circumscribed solitary relatively small masses <3 cm at the time of their presentation and feature a grayish-white to brown appearance 1,4.  

There are three different histological subtypes of angioleiomyomas subject to their vascular morphology (solid, venous and cavernous) with the following features 1:

  • fascicles of bland, well-differentiated smooth muscle cells

  • interposed vascular channels of a variable calliber

  • possible myopericytoma-like elements

  • might show adipocytic metaplasia, prominent hyalinization or uncommonly calcification

The subtypes do not seem to have any clinical significance.

Immunohistochemistry stains are usually positive for smooth muscle actin, calponin and h-caldesmon as well as variably positive for desmin 1.

Imaging findings are generally non-specific 2,3:

  • usually well-defined, oval, lobulated or round masses

  • expansile growth

  • sometimes with cortical scalloping if adjacent to bony structures

On ultrasound angioleiomyomas have been described to display the following characteristics 3,4:

  • echotexture: homogeneous in the majority of cases, heterogeneous in about a fourth

  • usually hypoechoic or hypoechoic and isoechoic

  • might show hypoechoic protrusions at the ends of the mass

  • posterior acoustic enhancement

  • vascular flow signal on color Doppler

  • no septations

CT will probably show a well-defined and otherwise unspecific cutaneous or subcutaneous soft tissue density mass. There might be calcifications.

In addition to the general imaging features a peripheral low-signal intensity rim corresponding to a fibrous pseudocapsule with adjacent vascular structures have been described on MRI in addition to the following signal characteristics 2-5:

  • T1: isointense or hyperintense to muscle

  • T2: heterogeneous and slightly hyperintense to muscle

  • T1 C+ (Gd): homogenous to heterogeneous enhancement, rarely only peripheral

The radiological report should include a description of the following:

  • form, location and size

  • tumor margins and transition zone

  • distance from the muscular fascia

  • contact to bones or neurovascular structures

Management is primarily surgical excision. Angioleiomyomas are benign and recurrence is rare 1-5.

Angioleiomyomas were first described in 1937 by the American surgical pathologist Arthur Purdy Stout (1885-1967) 6,7.

Conditions that can mimic the presentation and/or appearance of angioleiomyomas include 2-4:

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