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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, PowerPoint PPT Presentation


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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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PROXIMAL-TYPE EPITHELIOID SARCOMAS: A RETROSPECTIVE ANALYSIS OF 31 PATIENTS Piovesan C, Dileo P, Puma E, Marrari A, Bertulli R, Fiore M,

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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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36 pts

70 pts

Am J Clin Pathol 2009;131:222


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Patients

No. 31

M:F14:17

Age37 (19-71) yrs

Size10 (1-27) cm

Metastatic d’emblée2

Site

Urogenital

& inguino-perineal65%

Limbs23% (distal: 3 pts)

Thoracicwall 6%

Head & neck 6%


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Cancer Res 2005;65:4012


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Surgery

Wide excision13

Amputation 3

Marginalexcision+ re-excision 8

Marginalexcision relapse 

re-excision 3

Intralesionalexcision 2

No surgery 2


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Local recurrences

No. pts13

Mediantimefromsurgery4 mos (1-48)

Surgery13

+RT4

+CT2

Furtherrecurrences 5 (x 2-14)


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Metastases

Lymph nodes 9


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Metastases

Lymph nodes 9

Lungs12

Bone 3

Liver 2

Soft 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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DFS

urogenital & inguino-perineal

20 pts

limb, head&neck, thoracic wall

11 pts


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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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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

In brief…


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