Germ cell tumours in children

This information was written by the Children’s Cancer and Leukaemia Group (CCLG).

Each year, fewer than 45 children in the UK develop malignant germ cell tumours. Most children who develop germ cell tumours will be cured.

More children than ever are surviving childhood cancer. There are new and better drugs and treatments, and we can now also work to reduce the after-effects of having had cancer in the past.

It is devastating to hear that your child has cancer, and at times it can feel overwhelming but there are many healthcare professionals and support organisations to help you through this difficult time.

Understanding more about the cancer your child has and the treatments that may be used can often help parents to cope. We hope you find the information here helpful. Your child’s specialist will give you more detailed information, and if you have any questions, it is important to ask the specialist doctor or nurse who knows your child’s individual situation.

This factsheet is published in conjunction with CCLG’s booklet entitled: ‘Children and Young People with Cancer: A Parent’s Guide’.

Germ cell tumours

Germ cell tumours can appear at any age. They develop from cells that produce eggs or sperm so germ cell tumours can affect the ovaries or testes. However, it is also possible for a germ cell tumour to develop in other parts of the body.

As a baby develops during pregnancy, the cells producing eggs or sperm normally move to the ovaries or testes. However, rarely they can settle in other parts of the body where they can develop into tumours. The most common places for this to happen are the bottom of the spine (sacrococcygeal), the brain, chest and abdomen.

Germ cell tumours are sometimes given different names based on what they look like under the microscope. These include yolk-sac tumours, germinomas, embryonal carcinomas, mature teratomas and immature teratomas.

They may be non-cancerous (benign) or cancerous (malignant). Malignant tumours have the ability to grow and spread to other parts of the body. Benign tumours do not spread but may cause problems by pressing on nearby tissue and organs. Immature teratomas fall between benign and malignant. They can occur at many different sites, most commonly in the abdomen and can spread locally, such as within the abdomen, but rarely beyond. They can usually be removed with an operation.


Causes of germ cell tumours

The cause of germ cell tumours is not well understood but research into the causes of different cancers is ongoing.


How germ cell tumours are diagnosed

Different tests will be needed to diagnose a germ cell tumour. Usually, the doctor will remove a sample of tissue from the lump (a biopsy) for examination under a microscope to find out if it is a cancer or not. If the main tumour can be removed at the same time, then this may happen as part of the same procedure. A CT or MRI scan may be used to see the exact position of a tumour within the body. CT scans may be done to see if the tumour has spread to the lungs.

Germ cell tumours often produce proteins called tumour markers that can be measured in the blood. The ones that are produced by germ cell tumours are alpha-fetoprotein (AFP) and human chorionic gonadotrophin (HCG). Your child will have blood tests to check these tumour markers when they are being diagnosed. The doctors will continue to check these during treatment and after treatment is over. If your child has a germ cell tumour in the brain then these may also be detected in the fluid around the brain and spine called cerebro-spinal fluid (CSF). This can be tested by doing a lumbar puncture.

Sometimes a germ cell tumour can be diagnosed with tumour marker and scan results so that a biopsy isn’t necessary. This is particularly so if surgery is not needed for treatment or a biopsy may be particularly difficult, for example, if the tumour is in the brain.


Staging of germ cell tumours

The stage of a cancer describes its size and whether it has spread from where it started. Knowing the stage helps doctors to decide on the most appropriate treatment.

Generally, cancer is divided into four stages:

Stage 1

The cancer is small, has not spread and has been completely removed by surgery.

Stages 2 and 3

The cancer is larger and may not have been completely removed, or may have spread to nearby organs.

Stage 4

The cancer has spread to other distant parts of the body.

Cancer that has spread to distant parts of the body and formed new tumours is known as secondary or metastatic cancer.


Treatment for germ cell tumours

Germ cell tumours outside the brain

The treatment your child will have usually depends on a number of factors, including the size, position and stage of the tumour. It usually includes either surgery or chemotherapy, or a combination of the two.

A benign tumour can be cured if it is removed by surgery. It may mean removing a testicle or an ovary if this is where the tumour started.

If the tumour is malignant and can be completely removed with surgery, chemotherapy is not always needed, especially if it began in the testis or ovary. If the tumour cannot be removed easily or has spread, your child will be given chemotherapy.

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. Germ cell tumours are very sensitive to chemotherapy. It's usually given as injections and drips (infusions) into a vein.

A combination of drugs is usually given every three weeks for between three to five months. The length of time chemotherapy is given for and the type of chemotherapy depends on a number of factors including your child’s age, the position of the tumour, sometimes the level of markers, and whether or not the tumour has spread.

Germ cell tumours in the brain

Treatment of malignant germ cell tumours in the brain is a little different. Although they are very sensitive to chemotherapy, treatment with radiotherapy is also needed.

Radiotherapy is the use of high-energy rays to destroy cancer cells.

Unlike germ cell tumours elsewhere in the body, it's not always necessary to remove germ cell tumours in the brain with surgery. Depending on the type and stage of the tumour, radiotherapy is given either to part of the brain or the whole brain and the spine. Radiotherapy side effects will depend on the area treated and will be discussed with you by your radiotherapy team.


Side effects of treatment

Treatment often causes side effects, and your child’s doctor will discuss these with you before treatment starts. The operation your child has will be individually planned to try to reduce any possible problems. The surgeon and specialist nurse will discuss this with you. The side effects of chemotherapy usually gradually improve when treatment is over and many of them can be well controlled.

The side effects of treatment depend on the drugs used but can include:

  • feeling sick (nausea) and being sick (vomiting)
  • hair loss
  • increased risk of infection
  • bruising and bleeding
  • tiredness
  • diarrhoea.


Late side effects

A small number of children may develop long-term side effects of treatment, sometimes many years later. These are not common but may include problems with how the kidneys or lungs work and some hearing loss. Your child’s specialist doctor or nurse will tell you more about any possible late side effects.

If your child has only one ovary or testis removed at surgery, they’ll usually still be able to have children in the future, although fertility may be affected by some types of chemotherapy. We suggest you discuss fertility with your doctor, including options such as semen cryopreservation (sperm banking) if your child is going through or has completed puberty.


Clinical trials

Many children have their treatment as part of a clinical research trial. Trials aim to improve our understanding of the best way to treat an illness, usually by comparing the standard treatment with a new or modified version. Specialist doctors carry out trials for children's cancers.

If appropriate, your child's medical team will talk to you about taking part in a clinical trial and will answer any questions you have. Written information is often provided to help explain things.

Taking part in a research trial is completely voluntary, and you'll be given plenty of time to decide if it's right for your child.


Treatment guidelines

Sometimes, clinical trials are not available for your child’s cancer. This may be because a recent trial has just finished, or because the cancer is very rare. In these cases, you can expect your doctors and nurses to offer treatment which is agreed to be the most appropriate, using guidelines which have been prepared by experts across the country. The Children’s Cancer and Leukaemia Group (CCLG) is an important organisation which helps to produce these guidelines.


Follow-up care for germ cell tumours

Your child will continue to have regular blood tests during and after treatment, to check their levels of tumour markers. If the levels rise, this indicates that the tumour might have come back and further treatment is needed. Your child may also have regular scans after finishing treatment.

If you have specific concerns about your child’s condition and treatment, it's best to discuss them with your child’s doctor, who knows the situation in detail.


Your feelings

For parents, having a child diagnosed with cancer can be devastating. It is normal to experience fear, guilt, sadness, anger and uncertainty.

The CCLG booklet ‘Children and Young People with Cancer: A Parent’s Guide’ talks about the emotional impact of caring for a child with cancer and suggests sources of help and support.

Your child may also experience a variety of powerful emotions through the cancer journey. The Parent’s Guide discusses these further and talks about how you can support your child.


Useful organisations and references

Useful organisations

Children's Cancer and Leukaemia Group (CCLG)

CCLG supports the 1,700 children who develop cancer each year in the UK. It gives support to healthcare professionals involved in caring for children with cancer and is key to the development of high standards of care.

CLIC Sargent

CLIC Sargent offers practical support to children and young people with cancer or leukaemia, and to their families.

References

This factsheet has been compiled using information from a number of reliable sources, including:

  • Gershenson D. Ovarian germ cell tumors: Pathology, clinical manifestations, and diagnosis.
  • Pinkerton, et al. Evidence-based paediatric oncology. 3rd edition. 2013. Wiley-Blackwell.
  • Pizzo P, et al. Principles and Practice of Paediatric Oncology. 7th edition. 2016. Lippincott Williams & Wilkins.
  • Frazier L, et al. Revised Risk Classification for Pediatric Extracranial Germ Cell Tumors based on 25 years of clinical trial data from the United Kingdom and United States. Journal of Clinical Oncology 2015.


Children's cancer information from CCLG

This information about children’s cancer was written by the Children’s Cancer and Leukaemia Group (CCLG). It has been reviewed and edited by their publications committee, which includes medical experts from all fields of children’s cancer and care.