Pulmonary and extrapulmonary lymphangioleiomyomatosis. Report of a case with bilateral renal angiomyolipomas, multifocal lymphangioleiomyomatosis, and a glial polyp of the endocervix.

The authors report an extraordinary case of a 33-year-old female who had pulmonary and extrapulmonary lymphangioleiomyomatosis, bilateral renal angiomyolipomas, multifocal lymphangioleiomyomatosis involving the uterus, ovaries, periadrenal vessels, and liver. The patient also had a glial polyp of the endocervix which represented retained fetal parts from an abortion 15 years earlier. Both ovaries showed multiple follicle cysts which may have been a source of endogenous estrogen. The classical stigmata of the tuberous sclerosis complex were absent. The multifocal lymphangioleiomyomatosis of the various organs described presents an extremely rare manifestation of this disorder.