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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. Enzinger FM. Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer 1970; 26: 1029-41.
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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
Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma.
Cancer 1970; 26: 1029-41
classic-type proximal-type
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%
Wide excision 13
Amputation 3
Marginalexcision+ re-excision 8
Marginalexcision relapse
re-excision 3
Intralesionalexcision 2
No surgery 2
No. pts 13
Mediantimefromsurgery 4 mos (1-48)
Surgery13
+RT4
+CT2
Furtherrecurrences 5 (x 2-14)
Lymph nodes 9
Lymph nodes 9
Lungs 12
Bone 3
Liver 2
Soft tissues and peritoneum 1
median time to metastases = 4.5 mos
Adjuv CT (13 pts)
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…
