Table 4.
Summary of Treatment of Relapsed Paediatric Sarcomas
| Disease Site and Extent | Resectability | Management |
|---|---|---|
| Rhabdomyosarcoma | ||
| Localized or few metastasis |
Resectable | Surgery → chemotherapy ± radiotherapy |
| Unresectable | Chemotherapy → surgery or/+ radiotherapy | |
| Multiple metastasis | Unresectable | Previously received 2 drug (VCR + Act D) → VAC regimen |
| Previously received 3 drug VAC regimen → ICE or VCR + Irinotecan ± Temozolomide | ||
| Osteosarcoma | ||
| Localized or few metastasis |
Resectable | Surgery → chemotherapy ± radiotherapy |
| Unresectable | Palliative chemotherapy (high dose Ifosfamide based) or regorafenib | |
| Lung metastasis | Resectable | Surgery → chemotherapy ± radiotherapy |
| Unresectable | Palliative chemotherapy (high dose Ifosfamide based) or regorafenib | |
| Painful bony metastasis | Unresectable | Sm 153 EDTMP or Ra 223 |
| Multiple metastasis | Unresectable | Palliative chemotherapy (high dose Ifosfamide based) or regorafenib |
| Ewing sarcoma | ||
| Localized or few metastasis |
Resectable | Surgery → chemotherapy ± radiotherapy |
| Unresectable | Chemotherapy → surgery or radiotherapy | |
| Multiple metastasis | Unresectable | Palliative chemotherapy (ICE or Irinotecan + Temozolomide ± VCR) |
| Infantile fibrosarcoma | ||
| Localized | Resectable | Surgery → chemotherapy ± radiotherapy |
| Multiple metastasis | Unresectable | Palliative NTRK inhibitors viz. Larotrectinib |
Abbreviations: Act D, actinomycin D; ICE, ifosfamide/carboplatin/etoposide; NTRK, neurotrophic tropomyosin receptor kinase; VAC, vincristine/actinomycin D/cyclophosphamide; VCR, vincristine.