EPITHELIOID SARCOMAS: A RETROSPECTIVE ANALYSIS OF 31 PATIENTS

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Presentation transcript:

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

cytokeratin

Epithelioid sarcoma classic-type proximal-type

Proximal and deep-seated

Urogenital & inguino-perineal

Mod Pathol 2001;14:655

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

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%

Cancer Res 2005;65:4012

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

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

Metastases Lymph nodes 9

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

Overall survival

Disease-free survival

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

RT (16 pts) Adjuv CT (13 pts)

0 EpiADM + IFX

0 EI x 1

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

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

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

paolo.casali@istitutotumori.mi.it