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Translational Medicine / 转化医学Rare Sarcoma Subtypes

Silvia Stacchiotti

西尔维娅·斯塔基奥蒂

MD

🏢Fondazione IRCCS Istituto Nazionale dei Tumori, Milan🌐Italy

Senior Medical Oncologist; Head, Rare Soft Tissue Tumors Unit

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h-index
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Key Papers
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Awards
4
Key Contributions

👥Biography 个人简介

Silvia Stacchiotti, MD is Senior Medical Oncologist and Head of the Rare Soft Tissue Tumors Unit at Fondazione IRCCS Istituto Nazionale dei Tumori in Milan, Italy. She is an internationally recognized leader in the clinical investigation of rare sarcoma subtypes, particularly chordoma, alveolar soft part sarcoma (ASPS), epithelioid hemangioendothelioma (EHE), and other ultra-rare mesenchymal tumors for which effective treatments have historically been lacking. Dr. Stacchiotti has pioneered the clinical development of imatinib and sunitinib in chordoma and other rare tumors, and has led global consortium trials within the Chordoma Global Consensus Group. She has collaborated extensively with international networks including CTOS and EORTC to conduct basket trials, natural history studies, and consensus-building activities for orphan sarcoma subtypes. With over 180 peer-reviewed publications, she is a defining expert voice for ultra-rare sarcomas.

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🧪Research Fields 研究领域

Chordoma
Alveolar Soft Part Sarcoma
Epithelioid Hemangioendothelioma
Rare Sarcoma Subtypes
PDGFRA/KIT Inhibitors in Sarcoma
Anti-angiogenic Therapy

🎓Key Contributions 主要贡献

Imatinib and PDGFR-Targeted Therapy in Chordoma

Led the first prospective clinical studies demonstrating activity of imatinib mesylate in advanced chordoma, establishing PDGFR targeting as a rational and clinically active approach in this otherwise treatment-refractory tumor and setting the foundation for chordoma-specific drug development.

Alveolar Soft Part Sarcoma: Antiangiogenic Therapy

Conducted seminal investigations of sunitinib and cediranib in alveolar soft part sarcoma (ASPS), demonstrating consistent high response rates to anti-VEGF/VEGFR therapy in this molecularly distinct sarcoma subtype driven by ASPSCR1-TFE3 fusion.

Chordoma Global Consensus Group

Founding member and scientific leader of the Chordoma Global Consensus Group, which produced international consensus recommendations for pathological diagnosis, clinical staging, treatment sequencing, and response assessment in chordoma, adopted globally.

Natural History and Characterization of Ultra-Rare Sarcoma Subtypes

Led large multi-institutional natural history studies of epithelioid hemangioendothelioma (EHE), PEComa, and other ultra-rare sarcoma entities, providing the epidemiologic and clinical data underpinning regulatory designations and rational trial design for these cancers.

Representative Works 代表性著作

[1]

Imatinib mesylate in the treatment of chordoma

Cancer (2012)

Prospective study demonstrating clinical benefit of imatinib in patients with advanced chordoma, establishing the first evidence-based treatment option for this rare axial skeleton tumor.

[2]

Sunitinib in alveolar soft-part sarcoma

Annals of Oncology (2017)

Phase II study demonstrating high rates of objective response and prolonged progression-free survival with sunitinib in alveolar soft part sarcoma, establishing antiangiogenic therapy as a preferred treatment approach.

[3]

Best practices for the management and treatment of chordoma

Expert Review of Anticancer Therapy (2015)

International consensus review from the Chordoma Global Consensus Group providing comprehensive treatment recommendations for primary, recurrent, and metastatic chordoma.

🏆Awards & Recognition 奖项与荣誉

🏆CTOS Young Investigator Award
🏆Italian Association of Medical Oncology (AIOM) Sarcoma Research Prize
🏆European Sarcoma Network Working Group Leadership Award

📄Data Sources 数据来源

Last updated: 2026-04-06 | All information from publicly available academic sources

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