ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (5)
Cutaneous leiomyoma - Anogenital in
See also in: Overview
Other Resources UpToDate PubMed

Cutaneous leiomyoma - Anogenital in

See also in: Overview
Contributors: David O'Connell MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Cutaneous leiomyomas (CLs) are benign tumors derived from smooth muscle. There are 3 subcategories based upon the proposed smooth muscle of origin:
  • Angioleiomyoma – This most common type of CL originates from the smooth muscle of vessel walls.
  • Piloleiomyoma – The next most common type of CL evolves from the arrector pili muscle associated with hair follicles.
  • Genital leiomyoma – The least common type of CL is derived from smooth muscle associated with the external genitalia and nipple-areolar complex.
CLs tend to be tumors of adulthood, usually developing after the second decade. There is no reported racial or gender predominance.

Genital CLs are very rare and tend to be solitary; they are more likely nonpainful compared to the other forms of CL. They evolve from dartoic, vulvar, or mammary smooth muscle and may be pedunculated.

Piloleiomyomas are the subtype most commonly associated with the syndrome of hereditary leiomyomatosis and renal cell cancer (HLRCC, also known as Reed syndrome). These patients have germline heterozygous mutation of the fumarate hydratase gene that is inherited in an autosomal dominant fashion. In this condition, CLs develop by the patients' 20s, uterine leiomyomas develop (in women) by their 30s, and type 2 papillary renal cell cancer develops in the patients' 40s.

Codes

ICD10CM:
D21.9 – Benign neoplasm of connective and other soft tissue, unspecified

SNOMEDCT:
254767008 – Cutaneous leiomyoma

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:06/08/2022
Last Updated:07/11/2022
Copyright © 2022 VisualDx®. All rights reserved.
Cutaneous leiomyoma - Anogenital in
See also in: Overview
Cutaneous leiomyoma (Trunk or Extremities) : Painful skin lesions, Smooth nodule, Trunk, Arms, Legs
Clinical image of Cutaneous leiomyoma
A close-up of smooth, pink papules.
Copyright © 2022 VisualDx®. All rights reserved.