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What Causes Kidney Cancer In Children?

What Causes Kidney Cancer In Children?

Kidney cancers are quite rare in children, it is approximately about 45-50 cases each year in the UK and 75% of these kidney cancers occur in children less than 5 years. There are many types of kidney cancers that affect children and 95% of cases of kidney cancers are due to Wilms’ tumor. The exact cause of kidney cancers is unknown but Wilms’ tumor is associated with several birth defects, congenital syndromes such as WAGR syndrome, Denys-Drash syndrome, Frasier syndrome and Beckwith-Wiedemann syndrome. Other risk factors for kidney cancers are a von-Hippel-Lindau disease, tuberous sclerosis, familial renal cell cancer, and hereditary leiomyomatosis. (1)

What Causes Kidney Cancer In Children?

Wilms’ Tumor (WT): WT is the commonest kidney cancer seen in children and 95% of kidney cancers in children is Wilms’ tumor.

Renal Cell Carcinoma: Renal cell carcinoma is very rare in children less than 15 years, it can be more common in adolescents age from 15 to 19 years.

Clear Cell Sarcoma: This is also a very rare type of kidney cancers and it commonly affects the infants and children aged from 1-4 years.

Rhabdoid Tumor: This occurs commonly in infants and in very young children. It rapidly grows and spread to other organs such as the lung and brain.

Ewing Sarcoma: This is a rare tumor and commonly occurs in adolescents.

Desmoplastic Small Round Cell Tumor: It is a rare type of soft tissue sarcoma that mainly affects boys.

Synovial Sarcoma: Commonly affects the adolescents

Anaplastic Sarcoma: It is more common in children less than 15 years

Cystic Partially Differentiated Nephroblastoma: It is a rare type of Wilms’ tumor that is made up of cysts.

These are the kidney cancers that affect children. The cause or pathogenesis of kidney cancers in children is not known but there are several identified risk factors/ conditions are associated with some of the kidney cancers.

Conditions And Risk Factors Associated With Kidney Cancer

Conditions Associated With Wilms’ Tumor

12-15% of cases or 1 in 10 with Wilms’ tumor is associated with congenital birth defects and most of these birth defects are associated with a syndrome.

Wilms’ tumor is linked up with some birth defects which does not include into a syndrome such as:

  • Aniridia
  • Hemihypertrophy
  • Cryptorchidism
  • Hypospadias

The common syndromes Wilms’ tumor is associated are:

  • WAGR Syndrome
  • Wilms’ tumor
  • Aniridia – complete or partial loss of the iris of the eye
  • Genitourinary tract abnormalities
  • Mental Retardation
  • 30-50% of children have an increased risk of getting Wilms’ tumor.

Denys-Drash Syndrome And Frasier Syndrome: In Denys-Drash syndrome due to a disease status of the kidneys, the normal kidney function stops at a young age. Then the Wilms’ tumor develops in these diseased kidneys. Frasier syndrome children are also at increased risk of developing Wilms’ tumor

Beckwith-Wiedemann Syndrome: Children with this syndrome have a 5% risk of getting Wilms’ tumor. The mutations are present in chromosome 11 which influences the WT2 gene.

  • Perlman syndrome
  • Bloom syndrome
  • Sotos syndrome
  • Trisomy 18
  • Li-Fraumeni syndrome
  • Simpson-Golabi-Behmel syndrome

Von-Hippel-Lindau Disease: It is an inherited disease that causes abnormal growth in the blood vessels. These children are prone to get different types of tumors and some of them are benign and some are malignant. These children are at an increased risk of getting renal cell carcinoma therefore, these children should be investigated yearly by an ultrasound scan of the abdomen or by an MRI scan of the abdomen to check for any kidney cancers starting from the age 8-11 years.

  • Tuberous sclerosis is an inherited disease that causes fatty cysts that are non-cancerous and can be associated with renal cell carcinoma.
  • A mutation in the INI1 gene can increase the risk of getting a rhabdoid tumor.
  • Familial renal cell cancer is an inherited gene mutation that is carried from one generation to another.
  • Hereditary leiomyomatosis is an inherited condition that increases the risk of cancer in the kidney, uterus, and skin. (2) (3) (4)


Also Read:

Pramod Kerkar, M.D., FFARCSI, DA
Pramod Kerkar, M.D., FFARCSI, DA
Written, Edited or Reviewed By: Pramod Kerkar, M.D., FFARCSI, DA Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:November 29, 2021

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