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

Am J Clin Pathol 2009;131:222

70 pts

36 pts

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%

Cancer Res 2005;65:4012

Surgery

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

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)

Metastases

Lymph nodes 9

Metastases

Lymph nodes 9

Lungs 12 Bone 3Liver 2Soft tissues and peritoneum 1

median time to metastases = 4.5 mos

Overall survival

Disease-free survival

urogenital & inguino-perineal 20 pts

limb, head&neck, thoracic wall11 pts

DFS

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