Germ cells are the cells that develop into the body's reproductive cells, and when these cells go to other parts of the body, they produce extragonadal germ cell tumours. These tumours are uncommon, accounting for about 1% to 4% of all germ cell tumours. The name 'extragonadal' indicates that the tumours are located outside of the gonads, or reproductive glands, in males and females, respectively. Extragonadal germ cell tumours can be benign or malignant; benign tumours, also known as teratomas, account for 80% of extragonadal germ cell tumours. Teratomas are more common and can be quite large. Nonseminomas and seminomas are the two forms of malignant EGCs.
Extragonadal germ cell cancer is a form of aggressive germ cell cancer that generally affects young adults.
CAUSES AND RISK FACTORS OF EGC
Extragonadal germ cell tumours are thought to be produced by aberrant germ cell migration during the foetal development.
- Genetic flaws:
Females with Klinefelter syndrome or Turner syndrome have genetic abnormalities in the number of X chromosomes. The chromosomal condition Klinefelter occurs when a boy is born with an extra X chromosome.
- Genetic mutations
These have been discovered in tumours, albeit only a small percentage of cases have shown specific genetic mutations.
- Family History
People who have a family history of extragonadal germ cell tumours are more likely to develop them than those who do not.
These tumours have only been seen in adults above the age of 20.
While benign tumours can occur in both men and women, males are nine times more likely than women to develop malignant extragonadal tumours.
SIGNS AND SYMPTOMS OF EGC
Extragonadal tumours can appear anywhere, however they are most commonly encountered in three places:
- In the brain, there is a gland called the pineal gland.
- The area between the lungs is known as the mediastinum.
- The back of the abdomen is known as the retroperitoneum.
The following are the symptoms of the condition, according to the tumor's location:
The pineal gland, a small pea-shaped endocrine gland that generates melanin, a hormone that governs sleep patterns, is located in the brain. The likelihood of tumours in the pineal gland is lower than in the other two locations, and symptoms include:
- The tumor's increased intracranial pressure might produce nausea, headaches, and vomiting.
- Seizures, mobility abnormalities, vision issues, and intellectual function impairment may occur depending on where the tumour is placed in the brain.]
- Puberty begins early in life.
- Having difficulty moving your eyes
- Diabetes insipidus is a kind of diabetes that affects the kidneys.
- Thyroid hormone and growth hormone deficiency
The following are examples of symptoms:
- Swelling or pain in the abdomen
- Bloating accompanied by nausea or vomiting
- Bowel Movements Have Changed
- Even if you eat tiny amounts, you will feel satisfied.
- Weight loss causes a decrease of appetite.
- Urination problems and frequent urination
The tumours arise 50% to 70% of the time in the mediastinum, with the following symptoms:
- Breathing or swallowing problems
- Weight loss (cough)
- Angioedema (swelling of the face)
- Voice that is hoarse
EGC CANCER DIAGNOSIS
- Physical examination and history: The patient's general health is examined, as well as signs and symptoms of disease such as lumps or swellings. The patient's medical history and behaviours are recorded in order to determine the disease's proclivity.
- Tumour marker tests : It involves drawing a blood sample and testing it for biomarkers that can be raised in the presence of cancer. The following are some of the tumour markers:
- Alpha fetoprotein (AFP)
- Lactate dehydrogenase (LDH)
- Beta human chorionic gonadotropin ( beta- hCP )
3. Imagining tests : Extragonadal tumours can be detected with imaging tests such as a CT scan, PET scan, or MRI of the chest, abdomen, or pelvis.
4. Biopsy: It is the surgical removal of tumour cells in order to study them under a microscope. Excisional biopsy (removal of a lump of tissue), core biopsy (removal of tissue with a wide needle), or fine needle aspiration (FNA) biopsy are performed depending on the location of the extragonadal germ tumour.
It varies based on where the tumour is located, how far it has gone, and the type of tumour. The following are some of the therapy options:
- Palliative care
If the tumour is benign, surgical removal is the best option; however, if the tumour is located in an inaccessible or unsafe area for surgery, non-invasive treatment options such as chemotherapy or radiotherapy are used. Debulking surgery is performed, followed by chemotherapy and radiotherapy, if the tumour is too big or likely to spread.