Palliative Chemotherapy in Epithelioid Sarcoma

Robin L Jones,Anastasia Constantinidou,Khin Thway, Cyril Fisher,

Omar Al-Muderis, Michelle Scurr,Ian R Judson

Sarcoma Unit, Royal Marsden Hospital

Epithelioid sarcoma

• Rare

• Prevalent young adults

• 20–40 years of age

• Extremities

• Multiple local recurrences

• Proximal-type

Methods

• Aim to document– Response– Progression-free survival

• Prospective database identify– Epithelioid sarcoma patients– Treated with chemotherapy– 1990–2009

Methods

• Histological diagnosis confirmed

• Response criteria used – RECIST– WHO

• Statistical analysis– Median and range were used for continuous variables and

proportions (%) for categorical variables– Kaplan-Meier method used to present survival data

Results

• 55 epithelioid sarcoma patients

• Epithelioid sarcoma comprise <1% of soft tissue sarcomas in database

• 20 treated with chemotherapy– 18 males– 2 females

Patient characteristics

Characteristic Number of patients

Age 33 (17-64) years

Site of primary

Upper limb

Lower limb

Head and neck

Other

8 (40%)

3 (15%)

2 (10%)

7 (35%)

Proximal variant

Yes

No

9 (45%)

11 (55%)

Primary tumour size

Median (range) 9 (2-20) cm

Treatment

• Surgery for local disease: 16 (80%)

• Surgery for metastatic disease: 9 (45%)

• Adjuvant radiotherapy: 6 (30%)

• Palliative radiotherapy: 8 (40%)

Radiotherapy post chemotherapy

• PD on chemotherapy, n=1• to pelvis

• Consolidation post chemotherapy– PR, n=2

• to chest wall

– SD, n=3• to tongue + left arm • to pelvis • to cervical spine

Results

• Adjuvant chemotherapy: 4 (20%)– One lost to follow-up

• First line chemotherapy– 2 locally advanced– 17 metastatic disease

• Metastatic sites– Pulmonary– Soft tissue– Lymph node– Hepatic– Pleura– Brain

First line chemotherapy

• One recently completed first-line therapy

• PR: 5 (28%)

• SD: 8 (44%)

• PD: 5 (28%)

First line chemotherapy

• 19 patients 19 patients

Type of treatment

No of patients

Response

PR SD PD

Recently finished

Doxorubicin 9 3 4 2

Anthracycline/ ifosfamide 8 2 3 2 1

IVAD 1 1

Trabectedin 1 1

Proximal-type

• Proximal-type– SD, n=5 (56%)– PD, n=4 (44%)

• Non-proximal type– PR, n=5 (56%)– SD, n=3 (33%)– PD, n=1 (11%)

First-line chemotherapy

• Median PFS: 7 (95%CI, 4-9) months

• Median OS: 11 (95%CI, 5-21) months

• From diagnosis

• Median OS: 33 (95%CI, 28-39) months

Progression free Survival

0

20

40

60

80

100

0 1 2Years since start of Chemotherapy

% P

rogr

essi

on f

ree

Overall Survival

0

20

40

60

80

100

0 1 2 3 4Years since start of Chemotherapy

% S

urv

ival

Second line chemotherapy

• 7 patients

Type of treatment

Number of patients

Response

PR SD PD

Ongoing

Duration of stable disease

(months)

Ifosfamide 3 1 2 11

Trabectedin 1 1

Doxorubicin 1 1

Phase I trial 2 1 1 5

Third line chemotherapy

3 patients

Type of treatment No of patients Response

PR SD PD

Ongoing

Duration of stable disease

(months)

Ifosfamide 1 1 5

Trabectedin 1 1 5

Phase I trial 1 1

Major toxicity

• Adjuvant– Ifosfamide-induced encephalopathy: 1 patient

• First-line– Neutropenic sepsis: 2 patients

• Second-line– Neutropenic sepsis: 1 patient

• Third-line – None

OS from first-line chemotherapy in all soft tissue sarcoma subtypes

Karavasilis et al, Cancer 2008: 112; 1585-1591

Conclusion

• Epithelioid sarcoma is chemosensitive

– Response rate

– Progression-free survival

Acknowledgments

• Sue Ashley

• Cerys Propert-Lewis• Alison Dunlop• Sam Hackett

• Elizabeth Barquin• Julie Dados

• Meirion Thomas• Andrew Hayes• Dirk Strauss• Frank Saran