Respiratory Medicine Case Reports (Jan 2019)

Difficult diagnosis and rare morphology of lymphangioleiomyomatosis with giant cysts

  • Stefan Welter,
  • Philipp Thrull,
  • Michael Westhoff,
  • Peter Bach,
  • Helmut Popper,
  • Hans-Jörg Maschek

Journal volume & issue
Vol. 28

Abstract

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We report on a 32-year-old woman with a second manifestation of a tension pneumothorax two weeks after drainage therapy. The chest CT-scan revealed multiple large bilateral pulmonary cysts. She underwent minimally invasive wedge resection and pleurectomy for treatment. The extensive histologic evaluation revealed the diagnosis of a lymphangioleiomyomatosis (LAM) with an uncommon pattern of lung cysts. Initial staining for HMB-45 was negative. Repeated evaluation of other sections and reference pathology examination detected minimal expression of HMB-45.This case illustrates that immunohistochemistry for HMB-45 may be negative, although LAM is present and repeated immunohistochemistry may be necessary to establish the correct diagnosis. Keywords: Lung cysts, Lymphangioleiomyomatosis, Pneumothorax