Therapeutic target
Cure or improvement of prognosis
Therapy recommendations
- Primary or neoadjuvant chemotherapy (NACT; before surgery) is given for cytoreduction (tumor size reduction) in initially inoperable tumors.
- Adjuvant (supportive) chemotherapy is used when the risk is intermediate (surgery with chemotherapy) or when the tumor could not be removed by surgery alone.
- Subsequent standard therapy consists of stem cell therapy (after myeloablative therapy) followed by treatment of minimal residual disease with isotretinoin:
- High-dose chemotherapy with autologous stem cell transplantation: used when malignant (malignant) cells remain in the body despite chemotherapy or when molecular genetic unfavorable markers can be detected. Before this therapy is started, stem cells are taken from the patient because bone marrow depression (bone marrow is damaged) occurs as a side effect of high-dose chemotherapy. After therapy, they are returned to the patient (= autologous stem cell transplantation).
No detailed information on active ingredients and dosages is given here, because the therapy regimens are constantly modified.
Further notes
- Patients with high-risk variant neuroblastoma (12 months and older) may be treated with the immune antibody dinutuximab (FDA, 2015). This antibody recognizes the antigen GD2, which is located on the surface of neuroblastoma cells.Indication: first-line therapy or in the absence of response to other forms of treatment, and recurrences despite intensive cycles of radiation and chemotherapy.
- In a clinical trial, it could be demonstrated that the antibody dinutuximab alone works just as well as the combination of antibodies and interleukin-2, which has pronounced side effects.
- High-dose therapy with busulfan/melphalan versus carboplatin/etoposide/melphalan improved 3-year event-free survival (50 vs 38%; p = 0.0005)) of patients with high-risk neuroblastoma (1-20 years old) after previously adequate response to induction therapy. The therapy also caused fewer serious adverse events than carboplatin/etoposide/melphalan. After induction therapy, patients received stem cell transplantation followed by local irradiation and maintenance therapy.
