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

Détails

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Version: Final published version
Licence: CC BY 4.0
ID Serval
serval:BIB_0F71532CFC6B
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Institution
Titre
VEGFR Inhibitors for Uterine Metastatic Perivascular Epithelioid Tumors (PEComa) Resistant to mTOR Inhibitors. A Case Report and Review of Literature.
Périodique
Frontiers in oncology
Auteur(s)
Liapi A., Mathevet P., Herrera F.G., Hastir D., Sarivalasis A.
ISSN
2234-943X (Print)
ISSN-L
2234-943X
Statut éditorial
Publié
Date de publication
03/2021
Peer-reviewed
Oui
Volume
11
Pages
641376
Langue
anglais
Notes
Publication types: Journal Article ; Review
Publication Status: epublish
Résumé
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.
Mots-clé
PEComa, VEGFR, mTOR, second line, soft tissue sarcomas, uterine perivascular epithelioid cell tumor
Pubmed
Web of science
Open Access
Oui
Création de la notice
13/04/2021 13:21
Dernière modification de la notice
02/09/2021 6:40
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