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PROXIMAL-TYPE EPITHELIOID SARCOMAS: A RETROSPECTIVE ANALYSIS OF 31 PATIENTS

PROXIMAL-TYPE EPITHELIOID SARCOMAS: A RETROSPECTIVE ANALYSIS OF 31 PATIENTS Piovesan C, Dileo P, Puma E, Marrari A, Bertulli R, Fiore M, Olmi P, Gronchi A, Casali PG. Enzinger FM. Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer 1970; 26: 1029-41.

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PROXIMAL-TYPE EPITHELIOID SARCOMAS: A RETROSPECTIVE ANALYSIS OF 31 PATIENTS

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  1. PROXIMAL-TYPE EPITHELIOID SARCOMAS:A RETROSPECTIVE ANALYSIS OF 31 PATIENTS Piovesan C, Dileo P, Puma E, Marrari A, Bertulli R, Fiore M, Olmi P, Gronchi A, Casali PG

  2. Enzinger FM. Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer 1970; 26: 1029-41

  3. cytokeratin

  4. Epithelioid sarcoma classic-type proximal-type

  5. Proximal and deep-seated

  6. Urogenital & inguino-perineal

  7. Mod Pathol 2001;14:655

  8. 36 pts 70 pts Am J Clin Pathol 2009;131:222

  9. Patients No. 31 M:F 14:17 Age 37 (19-71) yrs Size 10 (1-27) cm Metastatic d’emblée2 Site Urogenital & inguino-perineal 65% Limbs 23% (distal: 3 pts) Thoracicwall 6% Head & neck 6%

  10. Cancer Res 2005;65:4012

  11. Surgery Wide excision 13 Amputation 3 Marginalexcision+ re-excision 8 Marginalexcision relapse  re-excision 3 Intralesionalexcision 2 No surgery 2

  12. Local recurrences No. pts 13 Mediantimefromsurgery 4 mos (1-48) Surgery13 +RT4 +CT2 Furtherrecurrences 5 (x 2-14)

  13. Metastases Lymph nodes 9

  14. Metastases Lymph nodes 9 Lungs 12 Bone 3 Liver 2 Soft tissues and peritoneum 1 median time to metastases = 4.5 mos

  15. Overall survival

  16. Disease-free survival

  17. DFS urogenital & inguino-perineal 20 pts limb, head&neck, thoracic wall 11 pts

  18. RT (16 pts) Adjuv CT (13 pts)

  19. 0 EpiADM + IFX

  20. 0 EI x 1

  21. EpiADM + IFX 8 pts  6 = PR  3 = surgically NED  3 = <3 mos

  22. 0 EI x 4 W Alive @30 mos EI x 2 + RT

  23. Rare subgroup within a rare subtype Young adults Urogenital & inguino-perineal region affected, followed by limbs and other sites High local recurrence rate Regional lymph nodes affected High metastatic potential & poor prognosis Possibly frequent, but often transient responses to chemotherapy Role of adjuvant treatments uncertain, but multimodal approaches may be rewarding in selected cases In brief…

  24. paolo.casali@istitutotumori.mi.it

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