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A Case of Lymphangioleiomyomatosis (LAM) of the Lung in a Patient with a History of Breast Cancer

Challenging differential diagnosis, Unusual or unexpected effect of treatment, Rare disease

Sinem Koc-Günel, Nesrin Tekeli, Christina Smaczny, Thomas Vogl, Gernot Rohde

Medical Clinic 1, Department of Respiratory Medicine and Allergology, Goethe University Hospital, Frankfurt am Main, Germany

Am J Case Rep 2019; 20:390-393

DOI: 10.12659/AJCR.914355

Available online:

Published: 2019-03-25

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare progressive cystic and nodular disease of the lung characterized by smooth muscle cell proliferation. LAM predominantly affects young premenopausal women. This report is of a case of LAM presenting in a 47-year-old woman with a past history of breast cancer and discusses the possibility of an association between the two conditions.
CASE REPORT: A 47-year-old woman presented as an emergency with an exacerbation of a four-month history of shortness of breath and dry cough. Her symptoms began following the start of anti-hormonal treatment with letrozole and goserelin acetate for a moderately differentiated (grade 2) invasive ductal carcinoma of the breast (pT2, pN0, M0) which was positive for expression of estrogen receptor (ER+), progesterone receptor (PR+), and human epidermal growth factor receptor 2 (HER2+). Until the previous four months, she had breast-conserving treatment with radiotherapy and tamoxifen therapy. Following hospital admission, she was found to be in type I respiratory failure. Chest X-ray, lung computed tomography (CT), and positron-emission tomography (PET) showed diffuse cystic and nodular lung lesions, consistent with a diagnosis of LAM, and antihormonal therapy was discontinued. She developed pericarditis that was treated with the anti-inflammatory agent, colchicine. Treatment with letrozole and sirolimus improved her respiratory symptoms.
CONCLUSIONS: A rare case of LAM is presented in a woman with a recent history of breast cancer. Because both tumors were hormone-dependent, this may support common underlying gene associations and signaling pathways between the two types of tumor.

Keywords: Aromatase Inhibitors, Carcinoma, Ductal, Breast, Colchicum, Estrogen Receptor Modulators, Goserelin, lymphangioleiomyomatosis