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Soft tissue tumors

Lymphangioma

Lymphangiosarcoma


Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 25 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Typically ~10 years post-axillary nodal dissection or radiation therapy for breast cancer with long-standing massive lymphedema
● Also after chronic lymphedema of lower leg
● 5 year survival < 10%

Case reports
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● 42 year old man with lymphangiosarcoma of pubic region arising in congenital non-hereditary lymphedema (Eur J Dermatol 1998;8:511)
● 74 year old woman with arm tumor 16 years after mastectomy (Cases J 2009 Sep 1;2:6887)
● 90 year old man with fatal lymphangiosarcoma on neck (Acta Derm Venereol 2009;89:318)

Clinical images
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Neck plaque

Arm

Arm

Gross description
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● Blue/purple papules in edematous skin, often multiple

Micro description
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● Angiosarcoma-like areas and endothelium-lined spaces without red blood cells
● Early: resembles benign collection of vessels, call "atypical vascular proliferation"
● Later: freely anastomosing vascular channels lined by atypical endothelial cells, often with solid areas resembling breast carcinoma
● Increased cellularity and pleomorphism with increased mitotic activity

Micro images
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Spindle cell proliferation with occasional intracytoplasmic vascular lumina

Atypical mitotic figures

CD31+

Various images


Thin walled vascular channels lined by endothelial cells are CD31+

Positive stains
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● D2-40

Differential diagnosis
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Angiosarcoma

End of Soft tissue tumors > Lymphangioma > Lymphangiosarcoma


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