Vol 17, No 1 (2021)
Review paper
Published online: 2021-02-26

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Diagnosis and treatment of lymphangioleiomyomatosis (LAM) from the PEComa group

Aleksandra Sobiborowicz12, Anna M. Czarnecka13, Anna Szumera-Ciećkiewicz45, Piotr Rutkowski1, Tomasz Świtaj1
Oncol Clin Pract 2021;17(1):28-41.


Lymphangioleiomyomatosis (LAM) is a rare, proliferative lung disease, leading to progressive damage of their structure and is a member of the PEComa neoplasm family (perivascular epithelioid cell tumors). In the patients, solid-cystic masses described as lymphangioleiomyoma or extrapulmonary LAM (E-LAM) can occur. E-LAM foci have been described in the mediastinum, supraclavicular lymph nodes, the liver, walls of the small and large intestine, the pancreas, mesentery. E-LAM masses can attain very large sizes — tumors 15–22 cm long have been described. On the basis of positive results of clinical trials sirolimus, a drug from the group of mTOR kinase inhibitors, was registered by the Food and Drug Administration (FDA) in May 2015 as the first and currently only drug for systemic LAM therapy. Sirolimus use is recommended in patients with LAM, accompanied by rapidly progressing deterioration of respiratory system function or FEV1 ≤ 70% predicted value and in patients with pleural lymph exudate before applying invasive methods of treatment.

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