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How is neuroblastoma treated?

 
, medical expert
Last reviewed: 04.07.2025
 
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Currently, neuroblastoma program treatment is carried out according to the risk group. Independent risk factors include the patient's age over one year and the presence of N MYC gene amplification. Many research groups introduce various additional risk factors.

The effectiveness of treatment is assessed according to the criteria of response to treatment:

  • complete remission (CR) - the tumor is not detected;
  • very good partial remission (VGPR) - reduction in tumor volume by 90-99%;
  • partial remission (PR) - reduction in tumor volume by more than 50%;
  • mixed remission (MR) - no new lesions, reduction of old lesions by more than 50%, increase of some lesions by no more than 25%;
  • no remission (NR) - decrease in foci by less than 50%, increase in some foci by no more than 25%;
  • progression (PROG) - new lesions or an increase in old ones by more than 25% or de novo bone marrow damage.

Treatment of neuroblastoma should be comprehensive. Surgical removal of the tumor is based on the principle of the most complete excision possible within healthy tissues. The location of the tumor in hard-to-reach areas may be an obstacle to following this principle. The results of most studies show that complete removal of the primary tumor improves survival.

Treatment tactics depend on the stage of the process and the risk group.

At stages I-II, an "observation" group is distinguished, for which chemotherapy is not provided. This group includes patients under one year of age without amplification of the N MYC gene and without life-threatening symptoms (severe general condition, severe respiratory and renal failure, etc.). Some researchers also include in this group children over one year of age with stage 1-IIa neuroblastoma without amplification of the N MYC gene and without life-threatening symptoms.

The cure rate for low-risk patients exceeds 90%. Most researchers include stage I-II of the disease in the absence of N MYC amplification and stage IVS in the presence of favorable biological factors (favorable histological type, hyperploidy, and absence of N MYC gene amplification ) in this group. In stage I, treatment is limited to surgical excision of the tumor and observation. If the residual tumor remains, chemotherapy is administered. The presence of severe life-threatening complications is an indication for chemotherapy. The most widely used drugs are carboplatin, cyclophosphamide, doxorubicin, and etoposide. If there is no effect, radiation therapy can be used. In some cases (absence of severe complications and tumor type), management of stage IVS is limited to observation only. In a study that included 80 children with stage IVS neuroblastoma, the survival rate using this tactic was 100%; When symptoms developed, low-dose chemotherapy resulted in 81% survival. According to a number of studies, tumor resection in these cases does not lead to increased survival.

The average risk group includes patients under one year old with stage III-IV neuroblastoma and no NMyC amplification, as well as patients over one year old with stage III neuroblastoma, no NMYC amplification and a favorable histological tumor variant. Cure of patients in the average risk group is possible in 70% of cases. Moreover, the highest cure rates are observed in children under one year old. Chemotherapy includes the same drugs as for the low-risk group, but its duration and cumulative doses of cytostatics are increased.

The most difficult task is to treat patients in the high-risk group, which includes cases with NM US amplification and/or an unfavorable histological variant of the tumor and stage IV in children over one year old. Survival in this group is low and amounts to 10-40%. Even with aggressive treatment tactics, relapses are often observed.

The standard approach is to use high-dose chemotherapy regimens that include cyclophosphamide, ifosfamide, cisplatin, carboplatin, vincristine, doxorubicin, dacarbazine, and etoposide. The primary tumor site is then irradiated.

Autologous hematopoietic stem cell transplantation plays a certain role in improving treatment results. In a large randomized study in a group of children who received high-dose chemotherapy with autologous transplantation of purified hematopoietic stem cells, 3-year event-free survival was 34% (in the group of children who received only consolidation chemotherapy - only 18%). The same study showed the advantage of using isotretinoin (13-cis-retinoic acid) for 6 months after the end of chemotherapy. Event-free survival for 3 years with the use of differentiating therapy with this drug was significantly higher.

New therapeutic approaches to the treatment of high-risk neuroblastoma are currently being studied. Certain successes have been achieved using monoclonal antibodies to neuroblastoma cell antigens. Experience has been accumulated using chimeric immunoglobulins to ganglioside 2 expressed on neuroblastoma cells. After binding of the antibody to the tumor cell, its lysis occurs as a result of complement activation or antibody-dependent cytotoxicity. The method is used in high-risk patients as adjuvant therapy in the presence of a minimal-volume tumor. Targeted radiotherapy with iobenguane (I 131 ) has proven successful in a number of patients with residual tumor. New methods of hematopoietic stem cell transplantation (myeloablative regimens with iobenguane-1131, tandem transplantation, etc.) are at the clinical trial stage.

Radiation therapy

The results of the conducted studies did not show any survival benefits for patients with neuroblastoma who received radiotherapy. Currently, radiation is used in the presence of residual tumor after chemotherapy or for palliative purposes. The radiation dose is 36-40 Gy. In young children, the maximum permissible radiation load on various organs and tissues and possible negative effects on the growing organism should be carefully calculated.

Neuroblastoma is one of the most unique human tumors, capable of both spontaneous regression and rapid growth. The prognosis for this disease depends on the patient's age and a number of biological characteristics. The following problems are currently the most pressing in relation to neuroblastoma:

  • the feasibility of conducting mass screening;
  • determination of a group of children who do not require therapy (observation group);
  • treatment of relapses and refractory forms of tumors;
  • search for drugs with a targeted effect on neuroblastoma cells;
  • the possibility of antitumor vaccination.

Resolving these issues could radically change the prognosis of one of the most common malignant diseases in children.

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