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Dermatology > DISEASES OF THE VESSELS
Acquired Progressive Lymphangioma
Article Last Updated: Oct 2, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Walter HC Burgdorf, MD, Clinical Lecturer, Department of Dermatology, Ludwig Maximilian University, Munich, Germany
Walter HC Burgdorf 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
Editors: Gregory J Raugi, MD, PhD, Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Rosalie Elenitsas, MD, Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
lymphangioendothelioma, APL, benign lymphangioendothelioma, Kaposi sarcoma, KS, angiosarcoma
Background
Acquired progressive lymphangioma (APL) is an uncommon vascular tumor that is of importance primarily because it can be confused histologically with Kaposi sarcoma (KS) or angiosarcoma. Wilson-Jones first described the tumor as APL and later as benign lymphangioendothelioma.
Pathophysiology
APL has long been suspected of being a lymphatic proliferation. Recently, stains for lymphatic endothelium (ie, podoplanin [D2-40], LYVE-1, and PORX-1) have become available. They will make it possible to unequivocally identify and categorize APL, although no studies have been published to date.
APL 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 APL is possibly triggered by hormonal changes. In contrast to KS, APL should not trigger a workup for HIV infection.
Frequency
United States
APL is rare; fewer than 30 cases have been reported.
Mortality/Morbidity
APL is a benign process with no mortality and minimal morbidity.
Race
No racial predisposition is reported.
Sex
Males and females are affected equally.
Age
APL affects adolescents and young adults.
History
A single, asymptomatic, slowly expanding patch or plaque usually manifests during adolescence or young adulthood.
Physical
- Flat to slightly elevated, slowly expanding red-brown lesions that resemble a bruise may be observed.
- Occasionally, a red-brown nodule may be observed, either within the patch or alone.
Causes
- In most instances, the cause is unknown.
- In one patient, APL appeared to be associated with radiation therapy for breast carcinoma.
- In another patient, femoral arteriography was a plausible trigger.
- Trauma also has been blamed, but a reliable connection has never been established.
Kaposi Sarcoma
Other Problems to be Considered
Angiosarcoma
Lab Studies
- No laboratory studies are necessary.
Imaging Studies
- Imaging studies usually are not helpful in diagnosing flat lesions.
- Obtain images of thicker plaques or tumors, to assess its 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 APL 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.
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.
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 KS.
The endothelial cells are not large or otherwise atypical. Extravasated red blood cells, hemosiderin, and plasma cells, which are 3 markers for KS, are not observed. Less differentiated accumulations of tumor cells are not found.
Special staining techniques reveal that the cells are variably positive for factor VIII, Ulex europaeus agglutinin I, CD31, and CD34. The staining patterns are too variable to be of diagnostic importance.
Medical Care
No medical care is required.
Surgical Care
- Once the diagnosis is established, no treatment is necessary.
- Solitary nodules can be excised; occasionally, local recurrence is observed.
- If more extensive patches and plaques are cosmetically disturbing but too large to excise, they can be treated with a laser.
- Because APL is relatively free of blood, the usual absorption characteristics that are importance in hemangiomas and vascular malformations are less important, and an individually tailored approach with test areas is recommended.
Consultations
Consultations with appropriate surgical specialists may be necessary, depending on the location of the APL.
No medical therapy is consistently effective; in one case, the patient's condition responded to oral corticosteroids. However, the administration of oral corticosteroids is not recommended.
Further Outpatient Care
- Annual follow-up care is recommended because of the possibility of local recurrence or misdiagnosis of angiosarcoma or KS.
Complications
- Only the usual complications of surgery and laser treatment, such as scarring and pigmentary change, exist.
Prognosis
- The prognosis is excellent.
Patient Education
- Inform patients that they have a rare vascular tumor that is poorly understood; thus, they should receive follow-up care.
Medical/Legal Pitfalls
- The problem with APL is the possible overdiagnosis of a serious, possibly fatal vascular malignancy when the entire clinical picture and histologic features are not considered.
- One should diagnose KS and angiosarcoma only in the appropriate clinical setting, not on the basis of histologic findings alone.
- Most dermatopathologists and surgical pathologists have little experience with APL; therefore, an expert should always be consulted.
| Media file 1:
Erythematous nodule with a hint of erythematous macular component at periphery. |
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| Media file 2:
Overview of a histologic section from the tumor in Image 1, with dilated vascular spaces interspersed between collagen fibers and a more central accumulation of many complex vascular spaces. |
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| Media file 3:
High-power view showing dilated vascular channel with innocent endothelial cells. |
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Acquired Progressive Lymphangioma excerpt Article Last Updated: Oct 2, 2006
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