serval:BIB_D07C5F1D118C
Effect of everolimus on multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis complex.
10.1016/j.rmcr.2020.101310
000600668000172
33312857
Daccord
C.
author
Nicolas
A.
author
Demicheli
R.
author
Chehade
H.
author
Hottinger
A.F.
author
Beigelman
C.
author
Lazor
R.
author
article
casereport
2020
Respiratory medicine case reports
2213-0071
2213-0071
journal
31
101310
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.
Alveolar epithelial cells
Everolimus
Lung
Mechanistic target of rapamycin
Multifocal micronodular pneumocyte hyperplasia
Tuberous sclerosis
eng
60_published
true
peer-reviewed
Publication types: Case Reports
Publication Status: epublish
University of Lausanne
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