Transcript GIST - CTOS
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 Site Urogenital & inguino-perineal Limbs Thoracic wall Head & neck 2 65% 23% (distal: 3 pts) 6% 6% Cancer Res 2005;65:4012 Surgery Wide excision Amputation Marginal excision + re-excision Marginal excision relapse re-excision Intralesional excision No surgery 13 3 8 3 2 2 Local recurrences No. pts 13 Median time from surgery 4 mos (1-48) Surgery +RT +CT 13 4 2 Further recurrences 5 (x 2-14) Metastases Lymph nodes 9 Metastases Lymph nodes Lungs Bone Liver Soft tissues and peritoneum 9 12 3 2 1 median time to metastases = 4.5 mos Overall survival Disease-free survival DFS urogenital & inguino-perineal 20 pts limb, head&neck, thoracic wall 11 pts RT Adjuv CT (13 pts) (16 pts) 0 EpiADM + IFX 0 EI x 1 EpiADM + IFX 8 pts 6 = PR 3 = surgically NED 3 = <3 mos 0 W Alive @30 mos EI x 4 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 [email protected]