Soft tissue tumors - Cutaneous leiomyosarcoma

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Soft tissue tumors

Smooth muscle

Cutaneous leiomyosarcoma

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 22 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

Leiomyosarcoma: see also general, epithelioid, myxoid, pleomorphic, rhabdoid features

Epidemiology
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● More common in men, almost always whites in US (Cancer 2008;113:616)
● Mean age 40-60 years; childhood tumors are rare

Clinical features
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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, scrotal

Case reports
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● 57 year old man with hard, painless erythematous tumor of presternum (An Bras Dermatol 2011;86:999)
● 59 year old woman with leg tumor with osteoclast-like giant cells (J Med Case Rep 2007;1:180)
● 65 year old man with diffuse tumor of head and neck with desmoplasia (Australas J Dermatol 2006;47:291)
● 66 year old man with tumor arising in smallpox scar on shoulder (World J Surg Oncol 2012;10:148)
● 66 year old man with desmoplastic leiomyosarcoma of thigh (Dermatol Online J 2010;16:4)
● 67 year old woman with hip lesion (Univ Pittsburgh Case #107)
● 81 year old man with forehead mass (Am J Clin Dermatol 2008;9:185)
● 98 year old woman with facial lesion (The Internet Journal of Dermatology 2007 Volume 5 Number 1)
● Family with cutaneous leiomyosarcoma (not leiomyoma) and renal cell carcinoma, but no fumarate hydratase mutation (Int J Dermatol 2008;47:18)

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 stage 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)
● Tumors may recur and occasionally metastasize

Clinical images
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Left to right: presternum, shoulder, thigh

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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Hip lesion

Leg tumor with osteoclast-like giant cells

Shoulder lesion (at smallpox scar)

Face #1	#2	#3-SMA
Dermal-based spindle cell tumor	Desmoplasia	Nuclear pleomorphism
Mitoses	Desmin+ tumor cells wrap around nerve	Presternum lesion

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 description
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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 > Smooth muscle > Cutaneous leiomyosarcoma

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