lymphangioleiomyomatosis
A multifocal neoplasm with perivascular epithelioid cell differentiation affecting almost exclusively females of child-bearing age. It is characterized by the presence of smooth muscle and epithelioid cells and by the proliferation of lymphatic vessels. Sites of involvement include the lungs, mediastinum, and the retroperitoneum. It usually presents with chylous pleural effusion or ascites.
MONDO:0006359
neoplasm with perivascular epithelioid cell differentiation
is_a
LAM
related
lung lymphangioleiomyomatosis
narrow
lymphangio-myomatosis
related
lymphangioleiomyomatosis
exact
lymphangioleiomyomatosis, somatic
exact
lymphangiomyomatosis
exact
pulmonary lymphangioleiomyomatosis
narrow
SPAC22F3.13 SPAC18B11.11 SPAC630.13c