European Radiology

, 19:3015

Lymphangioleiomyomatosis: solitary abdominal manifestation (2009: 9b)

  • Anne-Kristin Possekel
  • Detlef Katenkamp
  • Hans-Jürgen Brambs
  • Sandra Pauls
Interpretation Corner

DOI: 10.1007/s00330-008-1275-4

Cite this article as:
Possekel, AK., Katenkamp, D., Brambs, HJ. et al. Eur Radiol (2009) 19: 3015. doi:10.1007/s00330-008-1275-4

Abstract

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.

Keywords

LymphangioleiomyomatosisComputed tomographySirolimus therapy

Copyright information

© European Society of Radiology 2009

Authors and Affiliations

  • Anne-Kristin Possekel
    • 1
  • Detlef Katenkamp
    • 2
  • Hans-Jürgen Brambs
    • 1
  • Sandra Pauls
    • 1
  1. 1.Department of Diagnostic and Interventional RadiologyUniversity Hospital of UlmUlmGermany
  2. 2.Department of PathologyUniversity Hospital JenaJenaGermany