Lymphangiomyomatosis: a report of three cases treated with tamoxifen.
Three cases are reported of lymphangiomyomatosis with pulmonary and abdominal manifestations. Two had a chylous pleura effusion, while the third presented a retroperitoneal manifestation, which was completely resectable. Antiestrogen therapy with tamoxifen was administered in all three cases. Two patients died of pulmonary progression after 4 months of therapy. The third is still alive, with stable disease for more than 6 years, and has been receiving tamoxifen for 66 months. These observations indicate that antiestrogen treatment may be as effective as oophorectomy when started at an early stage of the disease.
D000008:Abdominal Neoplasms / Q000188:drug therapy*; D000328:Adult; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary; D008203:Lymphangiomyoma / Q000188:drug therapy* / Q000556:secondary; D008232:Lymphoproliferative Disorders / Q000188:drug therapy*; D011379:Prognosis; D013629:Tamoxifen / Q000627:therapeutic use*
D013629:Tamoxifen