Extrapulmonary lymphangioleiomyomatosis: a wolf in sheep's clothing

Thorax. 2013 Jan;68(1):111-3. doi: 10.1136/thoraxjnl-2012-201973. Epub 2012 Aug 3.

Abstract

We present a case of a 32-year-old woman who was diagnosed with lymphangioleiomyomatosis (LAM) after detecting a mass in the upper anterior mediastinum. Two years after presentation another metastatic localisation of LAM occurred in the cervical region. With this article we would like to highlight the fact that there are still a lot of unanswered questions, especially regarding the best management of extrapulmonary LAM.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy, Needle
  • Bronchoscopy / methods
  • Disease Progression
  • Dyspnea / diagnosis
  • Dyspnea / etiology
  • Female
  • Follow-Up Studies
  • Hemoptysis / diagnosis
  • Hemoptysis / etiology
  • Humans
  • Immunohistochemistry
  • Lymphangioleiomyomatosis / diagnosis*
  • Lymphangioleiomyomatosis / surgery
  • Magnetic Resonance Imaging / methods
  • Neoplasm Invasiveness / pathology
  • Neoplasm Recurrence, Local / parasitology*
  • Neoplasm Staging
  • Rare Diseases
  • Thymus Neoplasms / diagnosis*
  • Thymus Neoplasms / surgery
  • Tomography, X-Ray Computed / methods