Soft tissue

Pericytic (perivascular)

Angioleiomyoma


Editor-in-Chief: Debra L. Zynger, M.D.
Omar P. Sangueza, M.D.

Minor changes: 29 July 2021

Copyright: 2003-2021, PathologyOutlines.com, Inc.

PubMed search: angioleiomyoma [title] vascular pathology

Omar P. Sangueza, M.D.
Page views in 2020: 13,855
Page views in 2021 to date: 13,578
Cite this page: Aljarbou O, Sangueza OP. Angioleiomyoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueangioleiomyoma.html. Accessed October 16th, 2021.
Definition / general
  • Cutaneous angioleiomyoma (ALM) is a rare benign smooth muscle tumor that originates from arterial smooth muscle cells or the venous tunica media (J Ultrasound Med 2016;35:1669)
Essential features
  • Angioleiomyoma is a benign smooth muscle tumor
  • Preoperative diagnosis is not certain, microscopic examination is needed for accurate diagnosis
  • Complete excision is curative
Terminology
ICD coding
Epidemiology
Sites
Pathophysiology
Etiology
Clinical features
  • Painful, solitary, slow growing nodule (J Ultrasound Med 2019;38:1201)
  • Well demarcated subcutaneous lesions classically found on the lower extremities but can also present on the head, trunk and upper extremities (J Cutan Pathol 2017;44:342)
  • Difficult to distinguish from other smooth muscle tumors and few are diagnosed pre-operatively (J Cutan Pathol 2017;44:342)
  • Presence of pain may be helpful in the diagnosis but is unreliable, as only 58% of tumors present with pain (J Cutan Pathol 2017;44:342)
  • Pain can be caused by local ischemia from contraction of the tumoral vessels and may be mediated by nerve fibers (J Ultrasound Med 2019;38:1201)
  • Multiple subcutaneous angioleiomyomas have been reported in a patient with AIDS (Br J Dermatol 2002;147:563)
  • Clinical differential diagnosis includes glomus tumors, spiradenomas, angiolipomas or neuromas
Diagnosis
Radiology description
  • On ultrasonography: subcutaneous masses with an oval shape, well defined margins, a homogeneous structure, hypervascularity and a small size (< 2 cm) (J Ultrasound Med 2016;35:1669)
  • On MRI: well circumscribed mass with slightly hyperintense signal on T1 weighted images, heterogeneous and hyperintense signal on T2 weighted and STIR images and heterogeneous enhancement on contrast enhanced T1 weighted images
Prognostic factors
  • No known prognostic factors
Case reports
Treatment
Clinical images

Images hosted on other servers:
Missing Image

Foot mass

Missing Image

Palate mass

Missing Image

Lip mass

Missing Image

Hand tumor

Gross description
  • Sharply demarcated, spherical, gray-white or brown tumor
  • Usually measuring less than 2 cm in diameter in approximately 80% of cases
  • Reference: Cancer 1984;54:126
Gross images

Images hosted on other servers:
Missing Image

Circumscribed lip mass

Missing Image

Homogenous cut surface

Microscopic (histologic) description
  • Encapsulated proliferation of eosinophilic smooth muscle cells with minimal nuclear pleomorphism
  • Rounded or slit-like veins with muscular walls present within the tumor (J Cutan Pathol 2017;44:342)
  • Tumors can further be classified into 3 subcategories, first described by Morimoto et al:
    • Solid type: smooth muscle bundles surround numerous small slit-like channels
    • Cavernous type: dilated vascular channels, the walls of which are difficult to distinguish from the intervascular smooth muscle
    • Venous type: thick walled vessels that are easily distinguished from the intervascular smooth muscle
  • Epithelioid and pleomorphic variants are reported in a few cases
  • Calcification and marked degeneration, including hyalinization and myxoid changes, may be present (J Cutan Pathol 2017;44:342, J Ultrasound Med 2019;38:1201)
  • Fat can be seen in a few cases; not to be mistaken for angiomyolipoma (Cancer 1984;54:126)
Microscopic (histologic) images

Contributed by Ohoud Aljarbou, M.D., Jijgee Munkhdelger, M.D., Ph.D. and Andrey Bychkov, M.D., Ph.D.

Smooth muscle fascicles and vascular channels

Lack atypia and mitosis

Well defined mass

Edema and hyalinization

Small fascicles of smooth muscles


Well defined mass with vascular component

Fascicles of smooth muscles

SMA

Desmin


Missing Image

Low power

Missing Image

Well defined nodule

Missing Image

Smooth muscle bundles, vessels

Missing Image

Organizing thrombus

Missing Image

Thin walled blood vessels

Missing Image

Thick walled blood vessels

Cytology description
  • Variable proportions of benign smooth muscle cells and uniform spindle cells
  • Dissociated or arranged in small fascicles
  • Small fragments of a collagenous matrix in the background (Monogr Clin Cytol 2017;22:68)
  • The limitations encountered are very small, hard or painful skin lesions that may be difficult to aspirate (Diagn Cytopathol 2002;27:161)
  • Abundant connective tissue and calcification can also cause problems in obtaining adequate material (Diagn Cytopathol 2002;27:161)
Positive stains
Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Skin, lower leg mass, excision:
    • Angioleiomyoma, completely excised (see comment)
    • Comment: The grossly noted tumor is 1 cm. The resection margins are negative. There is no evidence of atypia or malignancy.
Differential diagnosis
Board review style question #1

    The image shown above is from a 45 year old woman with a right leg mass. SMA and desmin are positive. Which of the following is the most appropriate diagnosis?

  1. Angioleiomyoma
  2. Myopericytoma
  3. Glomus tumor
  4. Angiomyolipoma
Board review style answer #1
A. Angioleiomyoma. A mixture of smooth muscle bundles arranged in small fascicles and intervening vascular channels is noted. The concentric perivascular spindle cell proliferation which is characteristic for myopericytoma is absent. There are no glomus cells seen. Adipocytes are not seen in this image, which makes angiomyolipoma less likely.

Comment Here

Reference: Angioleiomyoma
Board review style question #2
    Which of the following modalities is the gold standard for the diagnosis of angioleiomyoma?

  1. Ultrasonography findings
  2. Dermoscopic findings
  3. MRI findings
  4. Microscopic examination
Board review style answer #2
D. Microscopic examination. Angioleiomyoma has no specific findings preoperatively. Microscopic findings are the gold standard for accurate diagnosis.

Comment Here

Reference: Angioleiomyoma
Back to top
Image 01 Image 02