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
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