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Table 2 Treatment and chemotherapeutic regimens (n = 41)

From: Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature

Variables

No. of patients (%)

Treatment modality

 

Chemotherapy

38 (93%)

   - Neoadjuvant

4 (10%)

   - Adjuvant

2 (5%)

   - Palliative (including relapse after neoadjuvant/ adjuvant treatment)

35 (85%)

Radiotherapy

 

   - Radical

2 (5%)

   - Palliative

4 (10%)

Surgery and its indications

 

   - Diagnosis

14 (34%)

   - Resection/optimal debulking of primary tumour

8 (20%)

Chemotherapy

 

First-line

 

   - Vincristine + ifosfamide + doxorubicin + etoposide (VIDE)

13

   - Ifosfamide + vincristine + actinomycin D + doxorubicin (IVADo)

7

   - Cyclophosphamide or ifosfamide + etoposide

4

   - Vincristine + doxorubicin + cyclophosphamide (VAC) or vincristine + ifosfamide + doxorubicin

4

   - Bleomycin + etoposide + cisplatin

2

   - Ifosfamide + doxorubicin

2

   - Carboplatin + etoposide

1

   - Carboplatin + paclitaxel

1

   - Cisplatin + doxorubicin/paclitaxel + cisplatin + ifosfamide

1

   - Doxorubicin

1

   - Epirubicin + cisplatin + capecitabine

1

   - Ifosfamide + carboplatin + etoposide

1

   - Ifosfamide + vincristine + actinomycin D

1

Subsequent-line (with chemotherapeutic agents)

 

   - Platinum + etoposide

10

   - Etoposide

5

   - Ifosfamide + etoposide

5

   - Doxorubicin

2

   - Ifosfamide + doxorubicin

2

   - Cyclophosphamide + topotecan

2

   - VAC

2

   - Gemcitabine or irinotecan + temozolomide

2

   - Albumin-bound paclitaxel

1

   - Cisplatin + mitomycin C + irinotecan

1

   - Doxorubicin + etoposide

1

   - IVADo

1

   - Liposomal doxorubicin

1

   - VIDE

1

Subsequent-line (with non-standard therapies)

 

   - Figitumumab

2

   - Sirolimus + cyclophosphamide or liposomal doxorubicin

2

   - Pazopanib +/− etoposide

2

   - Sunitinib

2

   - Axitinib

1

   - Goserelin

1

   - Interferon

1

   - Semaxanib

1

   - Sorafenib

1