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Welter, S; Thrull, P; Westhoff, M; Bach, P; Popper, H; Maschek, HJ.
Difficult diagnosis and rare morphology of lymphangioleiomyomatosis with giant cysts.
Respir Med Case Rep. 2019; 28(3):100873-100873
Doi: 10.1016/j.rmcr.2019.100873
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- Co-Autor*innen der Med Uni Graz
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Popper Helmuth
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- 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.
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Lung cysts
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Lymphangioleiomyomatosis
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Pneumothorax