Menstrual cycle variation of retroperitoneal lymphangioleiomyomas in lymphangioleiomyomatosis.

Abstract:

:Lymphangioleiomyomatosis (LAM) is a rare disease affecting women, classically involving the lungs. However, extrapulmonary manifestations also occur, including renal angiomyolipomas, retroperitoneal lymphangioleiomyomas and extrathoracic lymphadenopathy. The lung disease is hormone-responsive, but no information exists regarding sex hormone responsiveness of abdominal LAM. Here, we report two women with LAM whose abdominal lymphangioleiomyomas increased in size with hormonal changes of the menstrual cycle. This is the first description of abdominal lymphangioleiomyomas exhibiting hormone responsiveness in LAM. We describe these cases and summarize the literature on abdominal LAM. Menstrual cycle variation should be taken into account when assessing response to therapy, both clinically and in research studies.

journal_name

Intern Med J

authors

Sandrini A,Silverstone E,Yates DH

doi

10.1111/j.1445-5994.2011.02593.x

subject

Has Abstract

pub_date

2011-12-01 00:00:00

pages

832-5

issue

12

eissn

1444-0903

issn

1445-5994

journal_volume

41

pub_type

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