Effect of everolimus on multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis complex.

Details

Serval ID
serval:BIB_D07C5F1D118C
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Effect of everolimus on multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis complex.
Journal
Respiratory medicine case reports
Author(s)
Daccord C., Nicolas A., Demicheli R., Chehade H., Hottinger A.F., Beigelman C., Lazor R.
ISSN
2213-0071 (Print)
ISSN-L
2213-0071
Publication state
Published
Issued date
2020
Peer-reviewed
Oui
Volume
31
Pages
101310
Language
english
Notes
Publication types: Case Reports
Publication Status: epublish
Abstract
Multifocal micronodular pneumocyte hyperplasia (MMPH) is a benign proliferation of alveolar type II cells presenting as multiple pulmonary nodules at chest imaging, which is frequently seen in patients with tuberous sclerosis complex (TSC). We report a case of a woman with TSC and MMPH who received everolimus, a mechanistic target of rapamycin (mTOR) inhibitor, for the treatment of a subependymal giant cell astrocytoma (SEGA). After 3 months of therapy, a remarkable decrease in density of all pulmonary MMPH lesions was observed, without any change in size. This shows that everolimus is active on MMPH similarly to its effects on SEGA, renal angiomyolipomas, and pulmonary lymphangioleiomyomatosis in TSC, and suggests that the dysregulated activation of mTOR which characterizes TSC also plays a role in the pathogenesis of MMPH.
Keywords
Alveolar epithelial cells, Everolimus, Lung, Mechanistic target of rapamycin, Multifocal micronodular pneumocyte hyperplasia, Tuberous sclerosis
Pubmed
Web of science
Open Access
Yes
Create date
22/12/2020 10:33
Last modification date
14/01/2021 7:22
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