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Prognosis of solid tumors outside the CNS

Neuroblastoma

The prognosis depends on the age of the patient, clinical stage, and biological characteristics, especially n-myc amplification. In advanced stage neuroblastoma (stage 4), the prognosis has historically been very poor (20% survival) (see figure). Improving this poor survival is a focus of current clinical research. Historically, stages 1, 2, and 4S have had a good prognosis of about 90% survival. 

 

 
Survival without recurrence of patients with neuroblastoma in Europe according to stage division.

Wilms' tumor

The prognosis depends on the stage and tumor anaplasia (unfavorable histology). Almost all stages with a favorable histology have > 90% probability of survival.  

Germ cell tumors

The prognosis is generally good when the tumor is radically removed (> 80% survival), but certain subgroups such as choriocarcinoma have a poorer prognosis. 

Malignant liver tumors

Metastatic liver tumors have a poorer prognosis than non-metastatic, (30% and 70%, respectively). Non-resectable tumors have a very poor prognosis.

Rhabomyosarcoma

The prognosis depends on the location of the tumor as well as tumor-size, histology, and the age of the patient. The poorest prognosis is for children > 10 years with a large alveolar tumor in an unfavorable localization.

Non-rhabdo soft tissue sarcoma

It is difficult to determine prognostic factors with such rare tumor types, but by comparing the results from multiple studies, the following factors are significant: 

  • disease extent
  • radicality of the tumor resection 
  • tumor location
  • tumor size
  • invasiveness
  • histology
  • age of the child

Osteosarcoma

The most important prognostic factors for survival are the presence of metastases and the tumor's response to chemotherapy.

Survival after relapse is very low: less than 20% are long term survivors. The result is particularly poor if the recurrence appears shortly after finishing treatment and 1-2 metastases are present.

Ewing's sarcoma and pPNET

The significant prognostic factors are:

  • presence of metastases
  • age of the patient
  • size of the tumor
  • tumor location

The specific factors significant for the choice of definitive surgery or radiation therapy are the size of the tumor at the time of diagnosis, metastases, tumor cells in the border of the resection, and the preoperative chemotherapy response. In terms of molecular changes, it is known that loss of the INK4 tumor suppressor gene and mutation of p53 are associated with a poorer prognosis. Recurrence during the first 2 years after finishing treatment is associated with a poor prognosis.

The prognosis for children with Ewing's sarcoma and pPNET depends primarily on tumor spread and the tumor size as well as the response to treatment. The tumor burden can be reduced by earlier diagnosis, but the treatment response will only improve with better treatment. Attempts to improve treatment response include new chemotherapy combinations, increased chemotherapy intensity combined with better supportive care, and more radical surgery. In addition, more specific determination of prognosis allows for stratification of patients to different treatment groups so that patients with more favorable prognosis may be spared long term effects of treatment.

 

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