Lymphangiomatosis is a well-recognized congenital benign tumour, frequently seen in infancy and childhood, characterized by the presence of multiple lymphangiomas. The lymphangiomatosis spectrum encompasses a remarkable heterogeneity in its potential presentation, including micro- and macrocystic isolated lymphatic malformations, thoracic and intraabdominal diffuse lymphangiomatosis, and osseous and soft-tissue presentations known as Gorham disease. The disease can be considered a failure of normal lymphatic development, but the etiology remains poorly understood. Treatment has been limited to surgical resection or drainage procedures because there is no accepted effective systemic therapy.
Category
Neoplasm
Brite
Human diseases in ICD-11 classification [BR:br08403]
02 Neoplasms
Benign neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
Benign mesenchymal neoplasms
2E81 Benign vascular neoplasms
H01735 Lymphangiomatosis
