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Soft tissue tumors

Lymphangioma

Lymphangioendothelioma

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 26 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

Definition
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● Proliferation of D2-40+ endothelial cells
● Uncommon benign vascular lesion that may mimic well differentiated angiosarcoma or patch-stage Kaposi's sarcoma (Am J Surg Pathol 2000;24:1047)

Terminology
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● Also called acquired progressive lymphangioma

Epidemiology
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● Rare
● No gender preference, median age 54 years, range 17-90 years
● Usually not associated with other vascular anomalies or HIV infection

Clinical description
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● Solitary red or bruise-like slow growing plaque present for median 5.5 years
● Often in head and neck, but variable sites
● May resemble actinic keratosis (Cutis 2001;67:29)

Case reports
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● 26 year old woman with two year history of thigh lesion (Univ Pittsburgh Case #134)
● 32 year old man with a lesion in the hypogastric lesion (Actas Dermosifiliogr 2010;101:792)
● 75 year old man with giant lesion (J Cutan Pathol 2012;39:950)

Treatment and prognosis
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● Benign
● Occasional local recurrence

Clinical images
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Arm

Erythematous nodule

Hypogastrium (lower abdomen)

Thigh

Toe

Gross description
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● Median 1.5 cm, range 0.3 cm to 10 cm

Micro description
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● Delicate, thin-walled, endothelium-lined dilated vascular spaces involving the superficial dermis
● Intravascular papillary stromal projections resembles papillary endothelial hyperplasia
● Deeper portion of lesions have vascular space collapse and dissect collagen bundles, mimicking patch-stage Kaposi's sarcoma
● Preexisting vessels and adnexal structures of the dermis also appear dissected by newly formed vascular channels
● Smooth muscle often focally present around vascular spaces
● Endothelial cells may hobnail, may form morula resembling giant cells
● Crowding of endothelial cells present, but no endothelial atypia
● Vascular spaces lack erythrocytes and hemosiderin deposits
● No mitotic figures

Micro images
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Hypogastrium	Thigh
Toe	Lymphatic endothelium is podoplanin+
Site unspecified

Positive stains
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● CD31, CD34, Factor VIII, podoplanin, smooth muscle actin

Negative stains
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● HHV8

Differential diagnosis
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● Atypical or benign vascular proliferations of breast: history of radiation therapy
● Kaposi sarcoma: patch stage usually has widespread multiple lesions in HIV+ patients or extensive lesion of lower extremities in elderly patients of Jewish or Mediterranean origin; usually lymphoplasmacytic infiltrate, with inflammatory cells aggregating around vessels, commonly extravasated red blood cells, often other forms of Kaposi’s sarcoma present
● Lymphangioma circumscriptum
● Angiosarcoma-well differentiated: elderly patients, red-blue plaques or nodules, more endothelial atypia, multilayering and micropapillary tufting, often epithelioid or spindle cell component, inflammatory response common (Am J Dermatopathol 2000;22:151)

Additional references
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● eMedicine

End of Soft tissue tumors > Lymphangioma > Lymphangioendothelioma

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