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Soft Tissue Tumors Part 3
Muscle, Vascular, Nerve, Other
Cutaneous leiomyoma
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 9 August 2009, last major update - August 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Leiomyoma: classic, bizarre, cutaneous, epithelioid, genital
See also: leiomyoma of deep soft tissue
Definition
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● Arises from arrector pili muscles
● Superficial, small, multiple, painful
Terminology
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● Also called cutaneous piloleiomyoma
Clinical
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● May be due to hereditary leiomyomatosis and renal cell cancer syndrome (J Urol 2007;177:2074), associated with germline mutations in the fumarate hydratase gene (Nat Genet 2002;30:406, Cancer Genet Cytogenet 2008;183:83)
Case reports
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● 79 year old man with cutaneous leiomyomas and papillary renal cell carcinoma (Dermatology Online Journal 14(1):16)
Treatment
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● Avoidance of cold
● Possibly nifedipine, nitroglycerine, phenoxybenzamine (block smooth muscle contraction, Dermatol Online J 2005;11(4):21)
● Possibly botulinum toxin-type A (Dermatology 2009;218:44)
● Possibly excision with dermal skin template for multiple tumors (J Cutan Med Surg 2009;13:102)
Clinical images
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Multiple asymptomatic, Trunk lesions
hyperkeratotic nodules on leg
Micro images
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Nodular and diffuse tumors
Intradermal cellular nodular of spindle Whorled spindle cells
cells with elongated blunt-end nuclei
and fibrillar cytoplasm
Uniform cells without Trichrome stain highlights
mitotic activity smooth muscle cells
Molecular
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● Almost all patients who present with multiple skin leiomyomas have evidence of a germline fumarate hydratase mutation (Arch Dermatol 2005;141:199)
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Cutaneous leiomyoma
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