Childhood Germ Cell Tumours: Comprehensive Cancer Information

Childhood Germ Cell Tumours: A Comprehensive Guide

This information is intended to provide a general overview of Childhood Germ Cell Tumours. It is not a substitute for professional medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider for any health concerns or before making any decisions related to your health.

General Information

Germ cell tumours originate from germ cells, which are the fundamental cells intended to develop into sperm within the testicles or eggs within the ovaries. Occasionally, these cells do not complete their journey to the correct location in the body and instead form a tumour. These tumours can be either benign (non-cancerous) or malignant (cancerous, meaning they have the potential to spread to other parts of the body).

Classification and Location

Germ cell tumours are classified based on where they form and what they look like under a microscope.

Location-Based Classification:

Gonadal Tumours: Most malignant germ cell tumours are found in the ovaries or testes.
Extragonadal Tumours: They can also form in areas outside the reproductive organs, including:

The brain's pineal gland (near the pituitary gland).
The mediastinum (the area between the lungs).
The sacrum (a large bone in the lower spine that forms part of the pelvis).
The coccyx (tailbone).
The back.
The neck.

Microscopic/Hormone-Based Classification:

Teratomas: These are usually benign, though some can be malignant. Teratomas of the tailbone are the most common germ cell tumour found in children and are approximately four times more prevalent in girls than in boys.
Germinomas: These tumours produce the beta-human chorionic gonadotropin (β-hCG) hormone. They are named differently depending on where they form:
- Those in the ovaries are called dysgerminomas.
- Those in the testes are called seminomas.
- Those that appear outside the ovaries or testes are simply called germinomas.
Non-Germinomas: This group includes several types:
- Yolk Sac Tumours (Endodermal Sinus Tumours): These can develop in the ovaries, testes, or other body parts and are the most common type of testicular cancer in infants and children.
- Embryonal Carcinoma: These are malignant, most frequently found in the testicles, and have the potential to spread.
- Gonadoblastomas: Rare, almost always benign tumours linked to the abnormal development of reproductive organs.
- Polyembryomas: A very rare, aggressive type of germ cell tumour typically found in the ovaries.

Childhood Germ Cell Tumour Risk Factors

Several factors appear to increase the risk of a child developing a germ cell tumour:

Genetic Conditions:

Klinefelter syndrome may increase the chance of a tumour in the mediastinum.
Swyer syndrome may increase the chance of a germ cell tumour in the ovaries or testicles.
Turner syndrome may increase the risk of ovarian germ cell tumours.

Undescended Testicle

Having an undescended testicle increases the likelihood of a male developing a testicular germ cell tumour.

Family History

A family history of germ cell tumours also increases the chance of a person developing the condition.

Symptoms

The specific symptoms of a germ cell tumour depend on where the tumour forms.

In general, childhood germ cell tumour symptoms can include:

• A lump in the abdomen, lower back, or testicle.
• Pain in the abdomen.
• Breast growth (a symptom often associated with testicular germ cell tumours).
• In females, an absence of periods or unusual bleeding.
• Fever.
• Constipation.
• Headaches.

• Shortness of breath or chest pain.
• Excessive hair growth.
• Weakness in the legs.
• Early entry into puberty.
• Diabetes or hormonal abnormalities.
• Frequent urination.

It is important to understand that these symptoms do not definitively mean your child has a germ cell tumour. However, you must discuss any unusual symptoms with a doctor, as they could signal other health issues.

Diagnosis

Doctors use several methods to diagnose a childhood germ cell tumour. The diagnosis typically begins with a physical examination to check for symptoms such as swelling, lumps, and pain.

Diagnostic Tests

Blood Tests (Tumour Markers)

Blood tests are used to check for tumour markers, including high levels of hormones that are produced by specific types of germ cell tumours.

Imaging Exams

Tumours can be diagnosed and assessed through imaging exams, which include:

Bone scans
CT (Computed Tomography) scans
MRI (Magnetic Resonance Imaging)
Ultrasound
X-ray

Biopsy

Doctors can perform a biopsy, which involves surgically removing a small piece of the suspected tumour for detailed analysis under a microscope.

Childhood Germ Cell Tumour Staging

Cancer is staged based on how far it has progressed, which helps determine the patient's treatment plan and prognosis (outlook).

There is currently no universally agreed-upon staging system for childhood germ cell tumours in the brain.
Seminoma testicular cancers are staged using the main testicular cancer staging systems.
Staging systems for many other germ cell tumours are available (Source: National Cancer Institute).

Summary of Staging Systems

Tumour Type	Stage I	Stage II	Stage III	Stage IV
Childhood Nonseminoma Testicular Germ Cell Tumours	Cancer is in the testicle only and is completely removed by surgery.	Cancer is removed, but microscopic cancer cells remain in the scrotum or have spread to the scrotum or spermatic cord. Tumour marker levels do not return to normal or increase after surgery.	Cancer has spread to one or more lymph nodes in the abdomen and is not completely removed. Remaining cancer is visible without a microscope.	Cancer has spread to distant parts of the body such as the liver, brain, bone, or lung.
Childhood Ovarian Germ Cell Tumours (Children's Oncology Group)	Cancer is in the ovary, can be completely removed, and the capsule (outer covering) has not ruptured.	The cancer is not completely removed (remaining cancer is only visible with a microscope), OR it has spread to lymph nodes (only visible with a microscope), OR it has spread to the ovary's capsule.	The cancer is not completely removed (remaining cancer is visible without a microscope), OR it has spread to lymph nodes that are 2 centimetres or larger (visible without a microscope), OR cancer is found in fluid in the abdomen.	Cancer has spread to distant sites like the lung, liver, brain, or bone.
Childhood Extragonadal Extracranial Germ Cell Tumours	Cancer is in one place and completely removed by surgery. For tumours in the sacrum or coccyx, the sacrum and coccyx are completely removed. Tumour marker levels return to normal after surgery.	Cancer has spread to the capsule and/or lymph nodes, and the cancer remaining after surgery is only visible with a microscope. Tumour marker levels do not return to normal or increase after surgery.	The cancer is not completely removed (remaining cancer is visible without a microscope), OR it has spread to lymph nodes larger than 2 centimetres in diameter.	Cancer has spread to distant parts of the body, including the liver, brain, bone, or lung.

Prognostic Groups for Extragonadal Germ Cell Tumours

Instead of traditional staging, extragonadal germ cell tumours are often placed into prognostic groups:

Good Prognosis (Nonseminoma)

Tumour is in the back of the abdomen; has not spread to organs other than the lungs; and levels of tumour markers AFP and β-hCG are normal, with LDH only slightly above normal.

Good Prognosis (Seminoma)

Tumour has not spread to organs other than the lungs; and the level of AFP is normal (β-hCG and LDH may be at any level).

Intermediate Prognosis (Nonseminoma)

Tumour is in the back of the abdomen; has not spread to organs other than the lungs; and the level of any one tumour marker (AFP, β-hCG, or LDH) is more than slightly above normal.

Intermediate Prognosis (Seminoma)

Tumour has spread to organs other than the lungs; and the level of AFP is normal (β-hCG and LDH may be at any level).

Poor Prognosis (Nonseminoma)

Tumour is in the chest, OR has spread to organs other than the lungs, OR the level of any one tumour marker (AFP, β-hCG, or LDH) is high.

Note: No patients with a seminoma germ cell tumour are classified as having a poor prognosis.

Treatment

Treatment for germ cell tumours is highly personalised and depends upon several factors: the child's age and overall health, the specific type of tissues in the tumour, the tumour's location, the child's tolerance for specific procedures, and the overall prognosis.

Our Childhood Germ Cell Tumour Treatments

The treatment plan often involves a combination of therapies.

Surgery

Treatment for childhood germ cell tumours frequently includes surgery. In testicular cancer cases, this may involve removing the affected testicle or testicles. Ovarian cancer patients may have an ovary and fallopian tube removed.

Chemotherapy

Malignant germ cell tumours can spread before diagnosis. Chemotherapy travels throughout the body, making it an effective treatment for widespread cancer cells. SSCHRC provides the most current and advanced chemotherapy options for childhood germ cell tumours.

Radiation Therapy (Radiotherapy)

This uses high-energy beams to destroy cancer cells. New radiation therapy techniques and the remarkable skill of the doctors at SSCHRC allow them to target tumours more precisely, delivering the maximum amount of radiation while minimising damage to healthy cells. This precision is especially important for growing bodies.

Our Treatment Approach and Specialized Expertise

At SSCHRC, one of the world's foremost teams of experts customises a treatment plan for your child. This multidisciplinary group is among the few in the nation with specialised experience in treating these rare types of cancers.

SSCHRC is committed to providing the most advanced treatments for childhood germ cell tumours with the least possible impact on your child's body, both in the present and in the future.

The team that treats your child includes a number of highly trained doctors, such as oncologists, surgical oncologists, and radiation oncologists. They are supported by a group of dedicated professionals, including advanced practice nurses, physician assistants, therapists, and social workers.

SSCHRC offers a range of clinical trials for childhood germ cell tumours. We are at the forefront of discovering new and better ways to treat these tumours, which can translate to better chances for successful treatment for your child.