ISSVA subsets

Under the ISSVA-2025 scheme the term lymphatic malformation denotes the common cystic lesion alone, and the complex anomalies are named separately. The catalogued variants map across the five subsets below.

LM Lymphatic malformation 15 variants

The common cystic lesion, macrocystic or microcystic, the isolated form of the disease.

GLA Generalized lymphatic anomaly 9 variants

A multifocal malformation involving several regions or organs at once.

KLA Kaposiform lymphangiomatosis 9 variants

An aggressive lymphatic anomaly with kaposiform spindle-cell foci and a consumptive coagulopathy.

GSD Gorham-Stout disease 2 variants

Progressive osteolysis, the vanishing-bone disease, with lymphatic overgrowth into bone.

CCLA Central conducting lymphatic anomaly 11 variants

A malformation of the central conducting channels, the thoracic duct and cisterna chyli.