Lymphangioma progressive D18.10

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Acquired benign lymphangioendothelioma; benign lymphangioendothelioma; Benign lymphangioendothelioma; lymphatic malformation, lymphangioma, low-flow lymphatic malformation; progressive lymphangioma; Progressive lymphangioma

Definition
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Acquired benign proliferation of superficial lymph vessels.

Occurrence/Epidemiology
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Rarely.

Manifestation
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Beginning in childhood or early adulthood.

Localization
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Mostly occurring on the trunk and/or the extremities; the genitals may also be involved.

Clinical features
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First changes are observed as a reddish-brown, painless spot. Gradual but permanent size growth to a slightly elevated plaque of up to 30 cm in size. Cases of excessively extensive acquired cases involving the abdomen, lower extremities and genitals are described (Larkin SC et al. 2018).

Histology
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Emphasizes horizontally oriented growth of differently dilated lymph vessels in the upper and middle dermis. Infiltrating growth pattern, whereby in places adnexa or vessels are circulated (promontorium sign!). Absence of endothelial nuclear atypia, mitoses or multilayering. Focal endothelial hobnail formation is possible.

Differential diagnosis
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Therapy
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Apparently, systematically applied Sirolimus is able to stop the progression of the lymphatic malformation or to achieve partial remissions (Wiegand S et al. 2017).

External therapy
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Salman A et al. (2017) reported a cessation of size growth after application of a 5% Imiquimod cream.

Literature
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  1. Larkin SC et al (2018) A case of extensive acquired progressive lymphangioma. Pediatric dermatol 35:486-489.
  2. Salman A et al (2017) Acquired progressive lymphangioma: Case report with partial response to imiquimod 5% cream. Pediatric Dermatol 34:e302-e304.
  3. Wiegand S et al (2017) Treatment of Lymphatic Malformations with the mTOR inhibitor Sirolimus: A Systematic Review. Lymphatic Res Biol 16:330-339.

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Last updated on: 29.10.2020