Lymphangiomyomatosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Varun Kumar, M.B.B.S. [2]
Overview
Lymphangiomyocytosis is defined as a multifocal neoplasm with differentiation of the perivascular epithelioid cell and has a female prepondrance, especially females of child-bearing age.
Characteristic Features
Lymphangiomyocytosis is characterized by the presence of smooth muscle and epithelioid cells and by the proliferation of lymphatic vessels.
Sites of Involvement
- Lungs,
- Mediastinum, and
- Retroperitoneum.
Presentation
It usually presents with chylous pleural effusion or ascites.