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Benign Lymphangioendothelioma

  • Author: Donald Shenenberger, MD, FAAD, FAAFP; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Oct 06, 2015
 

Background

Benign lymphangioendothelioma (BLAE) (also known as acquired progressive lymphangioma) is an uncommon vascular tumor that is of importance primarily because it can be confused histologically with Kaposi sarcoma (KS) or angiosarcoma.[1, 2] Jones et al first described the tumor as acquired progressive lymphangioma and later as benign lymphangioendothelioma.[3]

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Pathophysiology

Benign lymphangioendothelioma is a proliferation of lymphatic endothelial cells that stain positively for CD31, CD34, podoplanin (D2-40, a lymphatic marker), LYVE-1, and PORX-1.[4] Benign lymphangioendothelioma is not associated with preexisting vascular malformations or lymphedema. Although the lesion rarely is identified during infancy, some suggest it is a hamartoma that first becomes apparent during adolescence or young adult life; the development of benign lymphangioendothelioma is possibly triggered by hormonal changes.

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Epidemiology

US frequency

Benign lymphangioendothelioma is rare; fewer than 30 cases have been reported.

Race

No racial predisposition is reported.

Sex

Males and females are affected equally.

Age

Benign lymphangioendothelioma can affect patients ranging from age 17-90 years (median age 54 y).[1]

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Contributor Information and Disclosures
Author

Donald Shenenberger, MD, FAAD, FAAFP Virginia Dermatology and Skin Cancer Center; Assistant Professor of Dermatology, Eastern Virginia Medical School

Donald Shenenberger, MD, FAAD, FAAFP is a member of the following medical societies: American Academy of Dermatology, American Academy of Family Physicians, Association of Military Dermatologists, Uniformed Services Academy of Family Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Walter HC Burgdorf, MD Clinical Lecturer, Department of Dermatology, Ludwig Maximilian University, Munich, Germany

Walter HC Burgdorf, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Society of Dermatopathology, International Society of Dermatopathology, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

References
  1. Guillou L, Fletcher CD. Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series. Am J Surg Pathol. 2000 Aug. 24(8):1047-57. [Medline].

  2. Requena L, Sangueza OP. Cutaneous vascular proliferation. Part II. Hyperplasias and benign neoplasms. J Am Acad Dermatol. 1997 Dec. 37(6):887-919; quiz 920-2. [Medline].

  3. Jones EW, Winkelmann RK, Zachary CB, Reda AM. Benign lymphangioendothelioma. J Am Acad Dermatol. 1990 Aug. 23(2 Pt 1):229-35. [Medline].

  4. Messeguer F, Sanmartín O, Martorell-Calatayud A, Nagore E, Requena C, Guillen-Barona C. [Acquired progressive lymphangioma (benign lymphangioendothelioma)]. Actas Dermosifiliogr. 2010 Nov. 101(9):792-7. [Medline].

  5. Revelles JM, Díaz JL, Angulo J, Santonja C, Kutzner H, Requena L. Giant benign lymphangioendothelioma. J Cutan Pathol. 2012 Oct. 39 (10):950-6. [Medline].

  6. Hwang LY, Guill CK, Page RN, Hsu S. Acquired progressive lymphangioma. J Am Acad Dermatol. 2003 Nov. 49(5 Suppl):S250-1. [Medline].

  7. Kim HS, Kim JW, Yu DS. Acquired progressive lymphangioma. J Eur Acad Dermatol Venereol. 2007 Mar. 21(3):416-7. [Medline].

  8. Fernandez-Flores A. Benign lymphangioendothelioma. Dermatopathonline. Available at http://www.dermatopathonline.com/lymphangioendothelioma2.html. Accessed: February 24, 2013.

  9. Kato H, Kadoya A. Acquired progressive lymphangioma occurring following femoral arteriography. Clin Exp Dermatol. 1996 Mar. 21(2):159-62. [Medline].

  10. Paik AS, Lee PH, O'Grady TC. Acquired progressive lymphangioma in an HIV-positive patient. J Cutan Pathol. 2007 Nov. 34(11):882-5. [Medline].

  11. Herron GS, Rouse RV, Kosek JC, Smoller BR, Egbert BM. Benign lymphangioendothelioma. J Am Acad Dermatol. 1994 Aug. 31(2 Pt 2):362-8. [Medline].

  12. Watanabe M, Kishiyama K, Ohkawara A. Acquired progressive lymphangioma. J Am Acad Dermatol. 1983 May. 8(5):663-7. [Medline].

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Erythematous nodule with macular component at the periphery.
Overview of a histologic section from a tumor depicting dilated vascular spaces interspersed between collagen fibers and a more central accumulation of many complex vascular spaces.
High-power view showing dilated vascular channel with innocent endothelial cells.
High-power view showing lymphatic endothelial cells in a hematoxylin and eosin–stained section.
High-power view showing lymphatic endothelial cells stained positively with podoplanin.
 
 
 
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