Immediately after delivery a 17-year-old Turkish primipara developed e
dema, supraclavicular lymphoma and pleural effusion. CT-scans showed m
assive abdominal and mediastinal lymphoma. Lymphangioleiomyomatosis (L
AM) was diagnosed by supraclavicular and retroperitoneal biopsy and pr
ogesterone receptors were documented in the tumor. There was no eviden
ce of pulmonary involvement. 5 months' treatment with the LHRH-analogu
e goserelin showed neither clinical improvement nor regression of LAM.
Irradiation of the ovaries and the abdomen with 30.2 Gy was followed
by amenorrhea without immediate tumor remission. A follow-up examinati
on 1 year later revealed clinical improvement and CT scans showed 50%
abdominal and mediastinal regression of LAM. 51/2 years after diagnosi
s the asymptomatic patient is still in partial remission. Sucessful tr
eatment of LAM is rare. We describe a case in which a major tumour red
uction was documented by CT scanning.