eMedicine Specialties > Dermatology > Diseases of the Vessels
Benign Lymphangioendothelioma: Differential Diagnoses & Workup
Updated: Mar 10, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
Other Problems to Be Considered
Workup
Laboratory Studies
- No laboratory studies are necessary for diagnosis benign lymphangioendothelioma (BLAE).
Imaging Studies
- Imaging studies usually are not helpful in diagnosing flat lesions.
- Obtain images of thicker plaques or tumors in order to assess any connection to underlying vascular structures.
- Because the tumor is so uncommon, suggesting an appropriate imaging modality is difficult.
Other Tests
- No other tests are necessary.
Procedures
- Either an incisional or excisional skin biopsy is helpful in diagnosis, depending on the size of the lesion.
Histologic Findings
The striking features of benign lymphangioendothelioma are the thin-walled endothelial-lined spaces that are interspersed between strands of collagen. Flat lesions may exhibit only this feature, whereas more nodular lesions may have a central collection of multiple complex vascular spaces.
Overview of a histologic section from the tumor in Media File 1, with dilated vascular spaces interspersed between collagen fibers and a more central accumulation of many complex vascular spaces.
More superficial channels are dilated, whereas at the periphery and deeper, the channels are slitlike. The spaces are usually empty or they may contain proteinaceous material, but they usually do not have abundant red blood cells.
High-power view showing dilated vascular channel with innocent endothelial cells.
The endothelial cells are not large or otherwise atypical. Occasionally, a hobnail pattern is seen, suggesting some relationship to targetoid hemosiderotic hemangioma and related tumors. Extravasated red blood cells, hemosiderin, and plasma cells, which are 3 markers for Kaposi sarcoma, are not observed. Less differentiated accumulations of tumor cells are not found.
The endothelial cells stain positively for lymphatic markers such as podoplanin (D2-40), LYVE-1, and PROX-1. Staining for human herpesvirus type 8 is negative, excluding the diagnosis of Kaposi sarcoma. The cells are also variably positive for factor VIII, Ulex europaeus agglutinin I, CD31, and CD34. The staining patterns are too variable to be of diagnostic importance and one should rely primarily on the lymphatic stains.8,9
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.
More on Benign Lymphangioendothelioma |
| Overview: Benign Lymphangioendothelioma |
 Differential Diagnoses & Workup: Benign Lymphangioendothelioma |
| Treatment & Medication: Benign Lymphangioendothelioma |
| Follow-up: Benign Lymphangioendothelioma |
| Multimedia: Benign Lymphangioendothelioma |
| References |
| « Previous Page | Next Page » |
References
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. Aug 2000;24(8):1047-57. [Medline].
Requena L, Sangueza OP. Cutaneous vascular proliferation. Part II. Hyperplasias and benign neoplasms. J Am Acad Dermatol. Dec 1997;37(6):887-919; quiz 920-2. [Medline].
Jones EW, Winkelmann RK, Zachary CB, Reda AM. Benign lymphangioendothelioma. J Am Acad Dermatol. Aug 1990;23(2 Pt 1):229-35. [Medline].
Hwang LY, Guill CK, Page RN, Hsu S. Acquired progressive lymphangioma. J Am Acad Dermatol. Nov 2003;49(5 Suppl):S250-1. [Medline].
Kim HS, Kim JW, Yu DS. Acquired progressive lymphangioma. J Eur Acad Dermatol Venereol. Mar 2007;21(3):416-7. [Medline].
Kato H, Kadoya A. Acquired progressive lymphangioma occurring following femoral arteriography. Clin Exp Dermatol. Mar 1996;21(2):159-62. [Medline].
Paik AS, Lee PH, O'Grady TC. Acquired progressive lymphangioma in an HIV-positive patient. J Cutan Pathol. Nov 2007;34(11):882-5. [Medline].
Herron GS, Rouse RV, Kosek JC, Smoller BR, Egbert BM. Benign lymphangioendothelioma. J Am Acad Dermatol. Aug 1994;31(2 Pt 2):362-8. [Medline].
Watanabe M, Kishiyama K, Ohkawara A. Acquired progressive lymphangioma. J Am Acad Dermatol. May 1983;8(5):663-7. [Medline].
Grunwald MH, Amichai B, Avinoach I. Acquired progressive lymphangioma. J Am Acad Dermatol. Oct 1997;37(4):656-7. [Medline].
Further Reading
Keywords
benign lymphangioendothelioma, acquired progressive lymphangioma, BLAE, Kaposi sarcoma, KS, angiosarcoma, targetoid hemosiderotic hemangioma
