Lajtman E, Mlynček M, Matejka M, Sládeček A, Langová I
Gynekologicko-Pôrodnická FN a Univerzita Knostantína Filozofa, Nitra, prednosta prof. MUDr. M. Mlyncek, CSc.
Ceska Gynekol. 2013 Apr;78(2):206-10.
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.
淋巴管平滑肌瘤病（LAM）是一种罕见的进行性疾病，影响育龄女性。该疾病的特征是主要在肺部出现未成熟平滑肌细胞的异常增殖。它逐渐导致呼吸衰竭，并常常导致死亡。肺外LAM通常表现为腹部肿块、腹痛和乳糜性腹水。在本病例报告中，我们描述了两例绝经前女性肺外LAM病例。两例均发生于盆腔闭孔窝及髂外动脉周围。手术后患者主要接受孕激素治疗。西罗莫司为二线药物。
