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.
D000328:Adult; D005260:Female; D006391:Hemangioma / Q000473:pathology*; D006801:Humans; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000473:pathology*; D008067:Lipoma / Q000473:pathology*; D008168:Lung / Q000473:pathology; D008175:Lung Neoplasms / Q000473:pathology*; D008203:Lymphangiomyoma / Q000473:pathology*; D008232:Lymphoproliferative Disorders / Q000473:pathology*; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D011127:Polyps / Q000473:pathology*; D002583:Uterine Cervical Neoplasms / Q000473:pathology*; D014599:Uterus / Q000473:pathology