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Soft Tissue Tumors Part 3
Muscle, Vascular, Nerve, Other
Cutaneous leiomyosarcoma
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 11 August 2009, last major update - August 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Leiomyosarcoma: general, cutaneous, epithelioid, myxoid, pleomorphic, rhabdoid features
Epidemiology
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● More common in men, almost always whites (Cancer 2008;113:616)
● Mean age 40-60 years; childhood tumors are rare
Clinical
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● Either dermal, arising from arrectores pilorum muscle, or subcutaneous, arising from wall of vessel
● Cutaneous tumors are indolent; subcutaneous tumors may metastasize (Cancer 2007;109:282)
Head and neck, extremities
Case reports
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● Family with cutaneous leiomyosarcoma (not leiomyoma) and renal cell carcinoma, but no fumarate hydratase mutation (Int J Dermatol 2008;47 Suppl 1:18)
● 59 year old woman with leg tumor with osteoclast-like giant cells (J Med Case Reports 2007 Dec 14;1:180)
● 65 year old man with diffuse tumor of head and neck with desmoplasia (Australas J Dermatol 2006;47:291)
● 67 year old woman with skin depression on hip (Univ Pittsburgh Case #107)
● 81 year old man with forehead mass (Am J Clin Dermatol 2008;9:185
● The Internet Journal of Dermatology 2007 Volume 5 Number 1)
Treatment
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● Complete excision with a narrow margin is acceptable for low-grade cutaneous tumors
● Wide excision with 2 cm lateral margin and one tissue barrier deep margin for low grade and early state subcutaneous and soft tissue tumors
● Wide excision for high grade tumors (Plast Reconstr Surg 2005;116:8)
● Mohs surgery offers tissue sparing with comparable results (Dermatol Surg 2004;30:108)
Micro description
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● Intersecting fascicles of brightly eosinophilic spindle cells with ovoid to cigar-shaped nuclei
● Pleomorphism and mitotic figures are common
Micro images
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Tumor arises from wall Spindle cells alternate with Spindle cells are positive
of subcutaneous artery osteoclast-like giant cells for smooth muscle actin
Various images Facial lesion: H&E and smooth muscle actin
Positive stains
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● Smooth muscle actin, muscle-specific actin, vimentin, calponin
● Variable desmin, caldesmon, myosin and keratin (Am J Surg Pathol 1997;21:979)
Molecular
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● No somatic mutations in fumarate hydratase gene in sporadic cases (Br J Cancer 2002;87:446)
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Cutaneous leiomyosarcoma
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