title: [Retroperitoneal lymphangioleiomyomatosis - a case reports].
abstract: 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.
authors.affiliation: Gynekologicko-Pôrodnická FN a Univerzita Knostantína Filozofa, Nitra, prednosta prof. MUDr. M. Mlyncek, CSc. lajtmanerik@gmail.com
authors.forename: E
authors.initials: E
authors.lastname: Lajtman
authors.forename: M
authors.initials: M
authors.lastname: Mlynček
authors.forename: M
authors.initials: M
authors.lastname: Matejka
authors.forename: A
authors.initials: A
authors.lastname: Sládeček
authors.forename: I
authors.initials: I
authors.lastname: Langová
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D018192:Lymphangioleiomyomatosis; D011374:Progesterone / Q000627:therapeutic use; D012186:Retroperitoneal Neoplasms; D020123:Sirolimus / Q000627:therapeutic use
chemical_list: D000970:Antineoplastic Agents; D011374:Progesterone; D020123:Sirolimus