Home Chapter Home Jobs Conferences Fellowships Books
Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other
Lymphangioendothelioma
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 11 October 2009, last major update – October 2009
Copyright: (c) 2002-2009, 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 (AJSP 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)
Treatment and prognosis
=========================================================================
● Benign
● Occasional local recurrence
Clinical images
=========================================================================
Thigh lesion Erythematous nodule
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
=========================================================================
Various images Lymphatic endothelium is podoplanin+
Positive stains
=========================================================================
● CD31, CD34, Factor VIII, podoplanin, smooth muscle actin
Negative stains
=========================================================================
● HHV8
Differential Diagnosis
=========================================================================
● Atypical or benign vascular proliferations post-radiation of breast - clinical histiory of radiation therapy
● Kaposi sarcoma (patch stage) - usually 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 - arises in infancy
● Well differentiated angiosarcoma - 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
=========================================================================
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Lymphangioendothelioma
This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must also 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, (c) 2001-2009, PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions.