Table 2 Overview of therapy according to stage

From: Rationale for the treatment of children with CCSK in the UMBRELLA SIOP–RTSG 2016 protocol

Stage

Preoperative chemotherapya

Postoperative chemotherapy

Abdominal radiotherapy

I

AV

Etoposide, carboplatin, ifosfamide alternating with cyclophosphamide, and doxorubicin

No

II

AV

Etoposide, carboplatin, ifosfamide alternating with cyclophosphamide, and doxorubicin

Yesb

III

AV

Etoposide, carboplatin, ifosfamide alternating with cyclophosphamide, and doxorubicin

Yesb

IV

AVD

Etoposide, carboplatin, ifosfamide alternating with cyclophosphamide, and doxorubicin or vincristinec

According to local stage and radiotherapy of metastatic sitesb,d

  1. AV, actinomycin and vincristine; AVD, actinomycin, vincristine, and doxorubicin; NA, not applicable.
  2. aIf clear cell sarcoma of the kidney has been confirmed by biopsy before the start of preoperative chemotherapy, starting treatment with five drugs (postoperative chemotherapy schedule) is advised instead of standard preoperative chemotherapy; evaluate the possibility of surgery after two cycles of chemotherapy (performing surgery within 6 weeks after the start of chemotherapy is recommended).
  3. bFor detailed radiotherapy recommendations, see the 'Radiotherapeutic Guidelines' chapter of the UMBRELLA protocol.
  4. cReplace doxorubicin with vincristine after reaching a total cumulative dose of 300 mg/m2.
  5. dAbdominal radiotherapy in instances of local stage II–III disease. Radiotherapy to metastases is indicated regardless of response to preoperative chemotherapy or surgical treatment (radiotherapy doses for metastases are described in the UMBRELLA protocol).
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