Rozpoznanie i leczenie limfangioleiomiomatozy (LAM) z grupy PEComa

نویسندگان

چکیده

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

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ژورنال

عنوان ژورنال: Oncology in Clinical Practice

سال: 2021

ISSN: ['2450-1654', '2450-6478']

DOI: https://doi.org/10.5603/ocp.2020.0034