1. 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

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

3. cytokeratin

5. Epithelioid sarcoma
classic-type proximal-type

7. Proximal and deep-seated

8. Urogenital & inguino-perineal

9. Mod Pathol 2001;14:655

10. 36 pts
70 pts
Am J Clin Pathol 2009;131:222

11. Patients No. 31 M:F 14:17 Age 37 (19-71) yrs Size 10 (1-27) cm
Metastatic d’emblée 2
Site
Urogenital
& inguino-perineal 65%
Limbs 23% (distal: 3 pts)
Thoracic wall 6%
Head & neck 6%

13. Cancer Res 2005;65:4012

14. Surgery Wide excision 13 Amputation 3
Marginal excision + re-excision 8
Marginal excision  relapse 
re-excision 3
Intralesional excision 2
No surgery 2

15. Local recurrences No. pts 13 Median time from surgery 4 mos (1-48)
+RT 4
+CT 2
Further recurrences 5 (x 2-14)

16. Metastases
Lymph nodes 9

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

19. Overall survival

20. Disease-free survival

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

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

23. EpiADM + IFX

24. EI x 1

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

26. EI x 4
W mos
EI x 2 + RT

27. In brief… 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