Lymphangioma circumscriptum D18.1

Fox and Fox, 1879

Benign, microcystic, cutaneous or cutaneous/subcutaneously localized, circumscribed lymphatic vasectasia (see also Vascular Malformation, see also Lymphangioma cavernosum) with dilated lymphatic vessels Lyphangioma circumscriptum is caused by a congenital (malformation) or acquired (usually postoperatively or after local radiotherapy) disturbance of the lymphatic flow, which leads to the clinically recognizable, mostly dense vesicles with water-clear (more rarely reddish or blue-reddish) content.

m:w=6:4;

• Primary lymphangioma circumscriptum: lymphatic malformation (dermal lymphectasia is often associated with subcutaneous cisterns which, as parts of the embryonic lymphatic vessel system, have no connection to the other lymphatic vessels). The increased intravascular pressure leads to an expansion of the papillary lymphatic vessels before birth or in the first years of life.
• Secondary (reactive) lymphangioma circumscriptum: Occurs after radiotherapy, postoperatively when lymph nodes have been cleared (up to decades of latency). Often associated with lymphedema.

• Primary lymphangioma circumscriptum: Mostly congenital or occurring in the first years of life; less frequently in adults.
• Secondary lymphangioma circumscriptum: Mostly in older adults.

Localizations in a larger collective (n=29): Anal/perianal region (25%), extremities (20%), tongue (15%), vulva (10%), scrotum (10%).

Densely standing, up to 1-3 mm large, thick-walled, sagocorn or frogspawn-like cysts that slowly increase in size (pseudovesicles). In longer populations wart-like aspect. Frequently bleeding, which is only very slowly absorbed, so that the impression of a haemangioma or haematolymphangioma can arise.

• Numerous lymph vessels filling the papillary body with optically empty lumen or filled with single erythrocytes. The vessel walls are free of pericytes. No mitoses. Immunohistology: Endothelia pos. for CD31.
• Deep lymphangiomas have muscular wall segments.

Occasionally the congenital forms of Lymphangioma circumscriptum are associated with genetically determined syndromal diseases. A gain-of-function mutation of genes coding for proteins of the RAS/RAF signalling cascade, as detected in Noonan syndrome, leads to a typical dysmorphic clinical picture and developmental disorders of the cardiovascular and lymphatic vascular system (Roberts AE et al. 2013).

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4. Fox T, Fox TC (1879) On a case of lymphangiectodes with an account of histology of the growth. Trans Path Soc Lond 30: 470
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