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Rare Cancers

Wilms’ tumour (Nephroblastoma)

  • Wilm's tumour (nephroblastoma) is an embryonal malignancy of the kidney, the most common renal tumor of childhood.
  • It usually presents as an abdominal mass in an otherwise apparently healthy child. 
  • It occurs most commonly among children under 5 years old, and has a very low incidence in the 10-14 and 15-19 year old populations. 
  • The incidence is 7/1,000,000 for children under 15 years old.
  • It represents 5% of all paediatric cancers.

Childhood rhabdomyosarcoma 

  • Rhabdomyosarcoma is the most common soft tissue tumor found in children and adolescents. 
  • It is a cancer made up of cells that normally develop into skeletal muscles. 
  • The incidence is 4.5/1,000,000 in children under 19 years old.
  • It accounts for 3% of childhood cancers.

Ewing’s sarcoma 

  • Ewing's sarcoma is a malignant small round cell bone tumor with strong metastatic potential. 
  • It is the second most frequent primary malignant bone cancer found in young people after osteosarcoma. 
  • The first symptom is usually pain at the tumor site, sometimes alongside a mass or swelling. 
  • It occurs between the ages of 5 and 30 with a peak of incidence between ages 12 and 18. The median age of patients with Ewing’s sarcoma is 15 years old.
  • The incidence is 3/1000 000 in children under 15 years old.

Gestational trophoblastic neoplasia 

  • Gestational trophoblastic tumors (GTT) or gestational trophoblastic neoplasia is a group of tumours that grow from the tissue that forms in the womb during pregnancy. 
  • It develops from the cells that would normally develop into the placenta during pregnancy. 
  • GTT include invasive mole, choriocarcinoma, placenta site trophoblastic tumor and epithelioid trophoblastic tumor (very rare). 
  • Choriocarcinoma is the most aggressive form of GTT and can quickly spread to the lungs, brain, liver, and other organs. The incidence is 1 per 20,000- 40,000 pregnancies

 

The prognosis of these cancers has been dramatically improved in the recent decades (80% to 90% of survival in early stages) thanks to a better understanding of the disease leading to fine tuning the therapeutic strategy which includes a dactinomycin based polychemotherapy.