• This record comes from PubMed

Retroperitoneálna lymfangioleiomyomatóza - kazuistiky
[Retroperitoneal lymphangioleiomyomatosis - a case reports]

. 2013 Apr ; 78 (2) : 206-10.

Language Czech Country Czech Republic Media print

Document type Case Reports, Journal Article

Links

PubMed 23710987
PII: 40562

Lymphangioleiomyomatosis (LAM) is a rare progressive disease affecting women of childbearing age. The disease is characterised by an abnormal proliferation of immature smooth muscle cells predominantly in the lung. It gradually leads to respiratory failure, and it frequently result in death. Extrapulmonary LAM typically presents with abdominal mass, abdominal pain and chylous ascites. In the case reports we describe two cases of premenopausal females with extrapulmonary LAM. In both cases they occur in pelvic location in the obturator fossa and around the external iliac artery. After surgical procedures patients were primary treated with progesterone. Sirolimus was second-line drugs.

Find record

Citation metrics

Loading data ...

Archiving options

Loading data ...