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Cutaneous leiomyosarcoma
Other Resources UpToDate PubMed

Cutaneous leiomyosarcoma

Contributors: Vivian Wong MD, PhD, Whitney A. High MD, JD, MEng, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed


Leiomyosarcoma is a rare, malignant smooth muscle tumor that can affect many body locations, including the uterus, the gastrointestinal system, the bladder, and the skin. This summary focuses on skin. Cutaneous leiomyosarcoma originates from arrector pilorum and it presents as a solitary nodule. The lower extremity is a common location, but lesions have also been reported upon the trunk, anogenital region, upper extremity, and the head / neck. Multiple lesions are unusual and should raise concern for metastatic events, particularly from disease of noncutaneous origin. Cutaneous leiomyosarcoma has a nonspecific clinical appearance and may be mistaken for numerous other diseases. Histopathologic analysis is necessary to establish the diagnosis.

Leiomyosarcoma of the skin is most common in middle-aged patients, with a predilection for men (male to female ratio of 2:1). Prior trauma, radiation exposure, and immunosuppression may predispose one to development of leiomyosarcoma. Cutaneous leiomyosarcoma has a favorable prognosis, particularly relative to visceral forms of disease. Some experts have proposed the term "atypical intradermal smooth muscle neoplasm," and disfavor use of the term "sarcoma," to prevent overtreatment of cutaneously limited disease.


C49.9 – Malignant neoplasm of connective and soft tissue, unspecified

254771006 – Cutaneous leiomyosarcoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Cutaneous squamous cell carcinoma
  • Amelanotic melanoma
  • Nodular basal cell carcinoma
  • Lobular capillary hemangioma (pyogenic granuloma)
  • Cutaneous metastasis of visceral malignancy (see Metastatic cutaneous carcinoma)
  • Vascular lesion
  • Histiocytoma
  • Cutaneous neuroma

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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Last Updated:10/29/2018
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Cutaneous leiomyosarcoma
A medical illustration showing key findings of Cutaneous leiomyosarcoma (Uterine Leiomyosarcoma) : Flank pain, Vaginal bleeding, Pelvic mass
Clinical image of Cutaneous leiomyosarcoma - imageId=6511732. Click to open in gallery.  caption: 'A smooth dull pink nodule with overlying telangiectasias on the scalp.'
A smooth dull pink nodule with overlying telangiectasias on the scalp.
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