Retroperitoneal lymphangioleiomyomatosis – a case reports

Česká verzia

Authors: E. Lajtman ¹; M. Mlynček ¹; M. Matejka ¹; A. Sládeček ²; I. Langová ²
Authors place of work: Gynekologicko-pôrodnícka klinika FN a Univerzita Konštantína Filozofa, Nitra, prednosta prof. MUDr. M. Mlynček, CSc. ¹; Ústav patológie FN, Nitra, primár MUDr. A. Sládeček ²
Published in the journal: Ceska Gynekol 2013; 78(2): 206-210

Summary

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.

Keywords:
lymphangioleiomyomatosis – chylous ascites – sirolimus

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