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Discrepant Behaviors in Embryonal Tumors: Insights into Solitary Fibrous Tumors and Sarcomas

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This paper by Paolo G. Casali explores the complexities of solitary fibrous tumors (SFT) and their behaviors, particularly focusing on discrepancies observed after complete response (CR) or no evidence of disease (NED) following delayed surgery. It highlights the classification of SFT according to WHO 2013, the challenges in distinguishing between benign and malignant forms, and the implications for treatment strategies. Additionally, the discussions touch upon various sarcoma-related genetic translocations, enhancing the understanding of tumor biology and treatment responses in patients.

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Discrepant Behaviors in Embryonal Tumors: Insights into Solitary Fibrous Tumors and Sarcomas

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  1. Papers #041-044 Discussion Paolo G. Casali paolo.casali@istitutotumori.mi.it

  2. Potential conflicts of interest Empl Cons Stocks Honor Res Test Other Amgen Dompé l l ARIAD l Bayer ll Glaxo SK l l ImClone l Infinity l Janssen Cilag l l Lilly l Merck SD ll Molmed l Novartis llll Pfizer lll PharmaMar llll Sanofi Aventis ll Schering Plough l

  3. J Clin Oncol 2001;19:197

  4. J Clin Oncol 2001;19:197

  5. Discrepant behaviors? • embryonal • group III • favorable site • after CR or NED after delayed surgery

  6. Solitary fibrous tumor

  7. Solitary fibrous tumor benign malignant dedifferentiated

  8. WHO 2013 • SFT • SFT, malignant • SFT, dedifferentiated

  9. «Principle of excluded middle»… «A ≠ non-A» Aristotle (384-322 BC)

  10. WHO 2013… • Benign • Locally aggressive non metastazizing • Rarely metastatizing • Malignant < 2%!

  11. Surgery: main sites • Pleura • Peritoneum • Soft tissues • Viscera • Meninges (incl.: retrobulbar, paravertebral, …) • …..

  12. Re-challenge… +10 cycles +9 cycles (23 overall) PD after stopping T (TTP from the end of T = 14 mos)

  13. Mol Cancer Therap 2009;8:449

  14. Oncogene 2013; in press

  15. Synovial sa t(X;18)(p11;q11) SS18-SSX1 SS18-SSX2 SS18-SSX4 Myxoid liposa t(12;16)(q13;p11) FUS-CHOP t(12;22)(q13;q12) EWS-CHOP Esk. myx. chondrosa t(9;22)(q22;q12) EWS-NR4A3 t(9;17)(q22;q11) RBP56-NR4A3 t(9;15)(q22;q21) TCF12-NR4A3 Fibromyxoid sa t(7;16)(q33;p11) FUS-CREB3L2 t(11;16)(p11;p11) FUS-CREB3L1 Clear cell sa t(12;22)(q13;q12) EWS-ATF1 Dermatofibrosa t(17;22)(q22;q13) COL1A1-PDGFB EFT t(11;22)(q24;q12) EWS-FL1 t(21;22)(q22;q12) EWS-ERG t(7;22)(p22;q12) EWS-ETV1 t(17;22)(q12;q12) EWS-E1AF t(2;22)(q33;q12) EWS-FEV Desmopl. SRCT t(11;22)(p13;q12) EWS-WT1 Alv. rhabdomyosa t(2;13)(q35;q14) PAX3-FOXO1A t(1;13)(p36;q14) PAX7-FOXO1A Alv. soft part sa t(X;17)(p11;q25) TFE3-ASPL Angiomatoid f. histiocytoma t(12;16)(q13;p11) FUS-ATF1 Cong fibrosa t(12;15)(p13;q25) ETV6-NTRK3 Endometrial stromal sa t(7;17)(p15;q21) JAZF1-JJAZ1 Infl. myofibro. t. t(1;2)(q22;p23) TPM3-ALK t(2;19)(p23;p13) TPM4-ALK t(2;17)(p23;q23) CLTC-ALK t(2;2)(p23;q13) RANBP2-ALK …..

  16. L-STS! leiomyosarcoma liposarcoma

  17. Paolo G. Casali paolo.casali@istitutotumori.mi.it

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