Lymphangiomatosis
Pronunciation: lim-fan-jee-oh-mah-TOH-sis
A diffuse, multifocal proliferation of abnormal lymphatic channels involving multiple organ systems, classified under the generalised lymphatic anomaly spectrum in pediatric pathology.
Full Definition
Lymphangiomatosis (now more precisely termed generalised lymphatic anomaly, GLA, in contemporary nosology) is characterised by the widespread abnormal proliferation of lymphatic channels across bones, lungs, spleen, liver, and soft tissues. In pediatric patients it typically presents in childhood or adolescence with chylous effusions, bone lytic lesions, and respiratory compromise. The International Society for the Study of Vascular Anomalies (ISSVA) 2018 classification introduced updated nomenclature; however, 'lymphangiomatosis' continues to appear in legacy literature and clinician correspondence. Editors must be aware that 'lymphangiomatosis' is a legacy term that many current guidelines replace with 'generalised lymphatic anomaly'; when editing contemporary documents, verify which term is preferred by the target journal or institution.
Usage
Usage note: Flag 'lymphangiomatosis' as a legacy term when editing current literature; 'generalised lymphatic anomaly (GLA)' is the ISSVA-preferred designation. Retain legacy term only in historical or quoted text.
In Context
- "The biopsy of the pulmonary lesion confirmed lymphangiomatosis, now reclassified as generalised lymphatic anomaly under ISSVA 2018 criteria." — Pediatric surgical pathology report
- "The author used 'lymphangiomatosis' throughout; the editor added a note that current ISSVA nomenclature prefers 'generalised lymphatic anomaly.'" — Manuscript editorial note