Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

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
=========================================================================

● 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
=========================================================================

● Also called acquired progressive lymphangioma

Epidemiology
=========================================================================

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

Clinical description
=========================================================================

● 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
=========================================================================

● 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
=========================================================================

● Benign
● Occasional local recurrence

Clinical images
=========================================================================


Arm

Erythematous nodule

Hypogastrium (lower abdomen)

Thigh

Toe

Gross description
=========================================================================

● Median 1.5 cm, range 0.3 cm to 10 cm

Micro description
=========================================================================

● 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
=========================================================================


Hypogastrium

Thigh

Toe

Lymphatic endothelium is podoplanin+

Site unspecified

Positive stains
=========================================================================

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

Negative stains
=========================================================================

● HHV8

Differential diagnosis
=========================================================================

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 Kaposiís 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
=========================================================================

eMedicine

End of Soft tissue tumors > Lymphangioma > Lymphangioendothelioma


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).