Difficult diagnosis and rare morphology of lymphangioleiomyomatosis with giant cysts

Autor: Stefan Welter, Philipp Thrull, Michael Westhoff, Peter Bach, Helmut Popper, Hans-Jörg Maschek
Jazyk: angličtina
Rok vydání: 2019
Předmět:
Zdroj: Respiratory Medicine Case Reports, Vol 28, Iss , Pp - (2019)
Druh dokumentu: article
ISSN: 2213-0071
DOI: 10.1016/j.rmcr.2019.100873
Popis: 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
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