Lymphangioleiomyomatosis: solitary abdominal manifestation (2009: 9b).
Lymphangioleiomyomatosis (LAM) is a rare disease that usually affects premenopausal woman and is characterized by cystic lung lesions and lymphatic disorders. We report a case of a 23-year-old woman who presented with diffuse, but increasing abdominal pain. Transabdominal ultrasound showed multiple cystic formations. Due to the patient's uncharacteristic symptoms, an exploratory laparotomy with tissue sampling was performed, and the diagnosis of LAM was confirmed by two independent pathologists. With computed tomography a broad abdominal, but no pulmonary, manifestation could be established. During sirolimus therapy the patient showed clinical benefit, but only slight progress in computed tomography.
Possekel
Anne-Kristin
AK
Department of Diagnostic and Interventional Radiology, University Hospital of Ulm, Germany.
Katenkamp
Detlef
D
Brambs
Hans-Jürgen
HJ
Pauls
Sandra
S
D000008:Abdominal Neoplasms / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging*; D011860:Radiography, Abdominal; D014057:Tomography, X-Ray Computed; D055815:Young Adult