Acquired Progressive Lymphangioma

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:

• In the US: 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.

Treatment
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.