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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
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Enzinger FM.Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer 1970; 26: 1029-41
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cytokeratin
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Epithelioid sarcoma
classic-type proximal-type
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Proximal and deep-seated
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Urogenital & inguino-perineal
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Mod Pathol 2001;14:655
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Am J Clin Pathol 2009;131:222
70 pts
36 pts
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Patients
No. 31
M:F 14:17
Age 37 (19-71) yrs
Size 10 (1-27) cm
Metastatic d’emblée 2Site Urogenital & inguino-perineal 65% Limbs 23% (distal: 3 pts) Thoracic wall 6% Head & neck 6%
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Cancer Res 2005;65:4012
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Surgery
Wide excision 13 Amputation 3Marginal excision + re-excision 8Marginal excision relapse re-excision 3Intralesional excision 2No surgery 2
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Local recurrences
No. pts 13 Median time from surgery 4 mos (1-48)
Surgery 13 +RT 4 +CT 2
Further recurrences 5 (x 2-14)
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Metastases
Lymph nodes 9
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Metastases
Lymph nodes 9
Lungs 12 Bone 3Liver 2Soft tissues and peritoneum 1
median time to metastases = 4.5 mos
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Overall survival
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Disease-free survival
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urogenital & inguino-perineal 20 pts
limb, head&neck, thoracic wall11 pts
DFS
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RT (16 pts)
Adjuv CT (13 pts)
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0 EpiADM + IFX
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0 EI x 1
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EpiADM + IFX
8 pts 6 = PR
3 = surgically NED 3 = <3 mos
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0 EI x 4
W Alive @30 mos EI x 2 + RT
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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