VEGFR Inhibitors for Uterine Metastatic Perivascular Epithelioid Tumors (PEComa) Resistant to mTOR Inhibitors. A Case Report and Review of Literature.

Details

Ressource 1Download: hastyr.pdf (605.81 [Ko])
State: Public
Version: Final published version
License: CC BY 4.0
Serval ID
serval:BIB_0F71532CFC6B
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
VEGFR Inhibitors for Uterine Metastatic Perivascular Epithelioid Tumors (PEComa) Resistant to mTOR Inhibitors. A Case Report and Review of Literature.
Journal
Frontiers in oncology
Author(s)
Liapi A., Mathevet P., Herrera F.G., Hastir D., Sarivalasis A.
ISSN
2234-943X (Print)
ISSN-L
2234-943X
Publication state
Published
Issued date
03/2021
Peer-reviewed
Oui
Volume
11
Pages
641376
Language
english
Notes
Publication types: Journal Article ; Review
Publication Status: epublish
Abstract
Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms. PI3K/AKT/mTOR pathway upregulation is critical for their pathogenesis and is often associated with TSC1/TSC2 inactivation. Although first line mTOR inhibitors are an effective treatment, metastatic PEComas eventually progress. A 53-year-old woman presented a 4-month history of post-menopausal vaginal bleeding. Clinical and radiological examination detected a uterine mass and a single S1 bone lesion. The patient underwent a radical hysterectomy and bone biopsy. The anatomopathological evaluation concluded to an oligo-metastatic uterine PEComa. The tumor harbored a heterozygous deletion of 9q34 that contains the TSC1 gene. Concerning the primary lesion, the resection was complete and the single bone metastasis was treated with radiotherapy. Three months later, the patient presented bone, lung and subcutaneous metastatic progression. An everolimus and denosumab treatment was initiated. After 2 years of treatment, a clinically significant bone, lung and subcutaneous progression was detected. Following a literature review of the possible therapeutic options, we initiated a second line treatment by pazopanib. This treatment resulted in regression of the subcutaneous lesions and stability of lung and bone metastases. In this challenging, rare setting, our report suggests single agent, anti-angiogenic, tyrosine kinase inhibitor to be effective as second line treatment of metastatic uterine PEComa progressing on mTOR inhibitors.
Keywords
PEComa, VEGFR, mTOR, second line, soft tissue sarcomas, uterine perivascular epithelioid cell tumor
Pubmed
Web of science
Open Access
Yes
Create date
13/04/2021 12:21
Last modification date
02/09/2021 5:40
Usage data