ArticleJournal of clinical medicine2021
Efficacy of Sirolimus Treatment in PEComa-10 Years of Practice Perspective.
Article in Journal of clinical medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
20 citing papers in PubMed, 22 citations in OpenAlex.
- TFE3-rearranged PEComa with hepatic and pulmonary involvement: diagnostic challenges and resistance to mTOR-targeted therapy.Journal of cancer research and clinical oncology · 2026Article
- PEComas: current concepts in diagnosis, molecular pathways, and emerging treatments.The oncologist · 2026Review
- How I treat soft tissue sarcomas.Future oncology (London, England) · 2026Review
- Renal malignant perivascular epithelioid cell tumor: a case report and literature review.Frontiers in oncology · 2026Article
- Clinicopathological and molecular features of gynecologic perivascular epithelioid cell tumors: a single-center study.Frontiers in oncology · 2026Article
- Characteristics and Treatment of Primary Hepatic Perivascular Epithelioid Cell Tumor (PEComa) in Adults: A Systematic Review.Cancers · 2025Review
- Malignant perivascular epithelioid cell tumor of the colorectum: Clinicopathological characterization, diagnosis and treatment process of 7 cases.World journal of gastrointestinal oncology · 2025Article
- Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis.Frontiers in oncology · 2025Article
- Hepatic perivascular epithelioid cell tumor: a retrospective analysis of 36 cases.Frontiers in oncology · 2024Article
- Case report: Malignant epithelioid angiosarcoma in a Chinese female patient.Frontiers in oncology · 2024Article
- Inhibitors of phosphoinositide 3-kinase (PI3K) and phosphoinositide 3-kinase-related protein kinase family (PIKK).Journal of enzyme inhibition and medicinal chemistry · 2023Review
- Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: a Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study.Journal of cancer research and clinical oncology · 2023Article
- Malignant Perivascular Epithelioid Cell Neoplasm of Left Kidney Treated With Targeted Therapy: A Rare Case Report.Cureus · 2023Article
- A rare metastatic mesenteric malignant PEComa with TSC2 mutation treated with palliative surgical resection and nab-sirolimus: a case report.Diagnostic pathology · 2023Article
- Case report: Responses to the combination of gemcitabine with sirolimus in two patients withFrontiers in oncology · 2023Article
- Insights into clinical diagnosis and treatment of malignant hepatic perivascular epithelioid cell tumor.Intractable & rare diseases research · 2022Article
- Dermatologic Disease-Directed Targeted Therapy (DDermatology and therapy · 2022Review
- The Molecular Biology of Soft Tissue Sarcomas: Current Knowledge and Future Perspectives.Cancers · 2022Review
- Rapamycin golden jubilee and still the miraculous drug: a potent immunosuppressant, antitumor, rejuvenative agent, and potential contributor in COVID-19 treatment.Bioresources and bioprocessing · 2022Review
- Spectrum of Presentations and Management Strategies in Renal Angiomyolipoma.Journal of kidney cancer and VHL · 2022Article
Corrections and comments
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Perivascular epithelioid cell tumors (PEComa) represent a family of rare mesenchymal tumors resultant from deregulation in mTOR pathway activity. The aim of this study is to evaluate the long-term efficacy of targeted PEComa treatment. We reviewed all consecutive patients with PEComa who started systemic treatment with sirolimus in our reference sarcoma center between January 2011 and August 2020. Histopathology of PEComa was reviewed and confirmed in all cases by a designated sarcoma pathologist. Any surviving progression-free patients were censored at the last follow-up (31 March 2021). Survival curves were calculated according to Kaplan-Meier method and compared with the log-rank test or a Cox proportional hazard model. Fifteen (12 females and 3 males) consecutive PEComa patients were treated. The median age of patients treated systemically was 50 years. Median progression-free survival (PFS) was 4.9 months (95% CI: 3.8-NA) for first-line chemotherapy and was not reached (95% CI: 42.0-NA) for sirolimus as first-line therapy. There was one objective response (OR) in the chemotherapy group. The OR rate reached 73% (11/15 cases) for sirolimus regardless of the treatment line. All patients archived disease control. Three patients died due to disease progression after 55, 32, and 32 months since metastatic disease diagnosis. After a median follow-up of 55.7 (range: 3.2-220) months, the 5 yr OS was 65% (CI 95% 39-100). Our study is the largest single-institution report on PEComa systemic targeted therapy and fills the gap in the field of advanced PEComa care since the FDA/EMEA approval of sirolimus.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.