Germ cell tumors are typical childhood neoplasms that belong to a large class of embryonal tumors. A special feature of germ cell tumors is their origin from germinal (embryonic) germ cells.
During embryonic development, the process of germinal cells migration into genital ridge zone occurs. Violation of this process leads to retention of these germinal cells in a certain part of the body, and in future germ cell tumors may develop from them. Thus, this type of tumor neoplasms can be localized not only in genital area, but also in other parts of the body (from which the process of embryonic germ cells migration to genital ridge area occurred).
Taking into account the peculiarities of germ cell tumors location, they are all divided into 2 large groups:
- Brain germ cell tumors.
- Extracerebral germ cell tumors.
You can also find a division of these tumors into following groups: gonadal localization (damage to gonads) and extragonadal localization (everything else, including brain).
The most common localization of germ cell tumors is brain, gonads, and mediastinum. All germ cell tumors are malignant and are capable of rapid invasive growth and metastasis.
Germ cell tumors are characterized by a hereditary predisposition. In these cases, there is a high incidence of bilateral genital lesions and tumors in early childhood.
These neoplasms account for about 3% of all malignant tumors in children. Girls get sick 2 times more often. Age peaks of incidence: extragonadal localization – children under three years of age; ovarian tumors – girls aged 10-15 years; testicular tumors – boys under 1 year of age and in postpubertal period. About 40% of germ cell tumors are neoplasms of sacrococcygeal region, 28% are ovarian tumors, 9% are testicular tumors. In 7%, tumor is localized in mediastinum (mainly in anterior), in 4% – in retroperitoneal space, in 4% – in head and neck area.
If your child is suspected of having this cancer or has already been diagnosed, do not waste time! Seek help from qualified specialists in the field of pediatric oncology. These are the doctors who work in Pediatric Oncology Department of Tel Aviv Medical Clinic. All our specialists have extensive experience and high qualifications; most of the doctors completed internships at the best oncology centers in the world, where they gained invaluable experience in communicating with sick children, so they will be able to find an approach to each child.
In their practical work, our oncologists are guided only by modern recommendations and treatment protocols based on evidence-based medicine principles. At the same time, an individual approach to each child is practiced. That is, doctor does not use one examination template and treatment regimen for everyone, but selects treatment and diagnostic procedures depending on baby’s age, his exact diagnosis (type of tumor, its location), stage of the disease, general health and concomitant diseases presence, wishes of parents or custodians must be taken into account obligatorily, as well as the young patient.
Causes of germ cell tumors in children
Germ cell tumors are most common in young children and teenagers.
Germ cell tumors causes are generally unknown. Some inherited syndromes may increase the risk of germ cell tumors development – these include Klinefelter syndrome, Swyer syndrome and Turner syndrome.
In boys, having an undescended testicle may increase the risk of developing a gonadal germ cell tumor in that testicle.
Symptoms of germ cell tumors
Symptoms of a tumor primarily depend on its location. For example, the first symptoms of a testicles germ cell tumor in boys are usually painless enlargement, hardening and unevenness of testicle surface, as well as a feeling of heaviness in scrotum. Sometimes the first symptoms are pain and swelling of scrotum, which makes it possible to suspect testicle inflammation (orchitis), but after further examination it turns out that the matter is completely different.
It happens that clinical symptoms of metastases first appear, for example, pain in lumbar region, abdominal pain, cough, shortness of breath, hemoptysis, various groups of peripheral lymph nodes enlargement.
With brain germ cell tumors, a child may be concerned about:
- Fluid accumulation in brain (hydrocephalus) and increased intracranial pressure.
- Regular headache, especially in the morning.
- Nausea and vomiting for no apparent reason.
- Decreased physical activity, increased fatigue.
- Visual impairment: double vision, limitation or pain when moving the eyeballs upward, difficulty focusing on nearby objects.
- Irritability.
- Memory impairment.
- A variety of neurological symptoms, depending on tumor location in brain.
- Changes in endocrine functions leading to such symptoms – growth retardation, early or late puberty, increased thirst and frequent urination in large quantities (diabetes insipidus), etc.
Most often, children encounter the following types of germ cell tumors:
- hepatoblastoma,
- gonadoblastoma,
- dysgerminomas in children,
- yolk sac tumor,
- pancreaticoblastoma (PBL),
- pneumoblastoma,
- polyembryoma,
- seminoma,
- sialoblastoma,
- chorionic carcinoma in children,
- embryonal carcinoma in children.
Modern diagnostic methods
Doctors of Pediatric Oncology Department at TAMC are fully proficient in all modern diagnostic methods, and thanks to innovative expert-class equipment, diagnostic process becomes completely safe and extremely effective.
Important! A feature of diagnostics in our department is speed and accuracy, because the earlier the diagnosis is established, the faster specific treatment is started, which significantly improves the prognosis. Also, the entire future success and life of the little patient depends on diagnosis accuracy, so all our efforts are aimed at observing these two basic diagnostic rules.
Diagnostic process begins with an initial consultation with a pediatric oncologist, collecting complaints from child and parents, studying medical history, a thorough examination and blood tests to obtain information about symptoms, general health, previous diseases and possible risk factors for disease developing.
The next stage is specific laboratory diagnostics. Using a blood test, special blood tumor markers are determined – β-hCG, AFP and LDH concentration. They are elevated in 80% of cases at invasive germ cell tumors.
Next, medical imaging is performed, that is, searching for a tumor, determining its size, spread to neighboring tissues and organs, metastases presence, etc. For this, modern diagnostic techniques are used – MRI, CT, MSCT with or without contrast, ultrasound of internal organs, lymph nodes, pelvic organs, testicles in boys. If it is necessary to evaluate metastases presence, whole body scanning techniques such as scintigraphy, PET-CT or PET-MRI are used.
The last stage, but very important, is a biopsy and histological examination of obtained tissue sample. This examination allows us to establish an accurate histological diagnosis and prescribe effective treatment. All biopsy materials are sent for revision to the best pathological laboratories in Israel. Thanks to such thorough checks, we are confident in diagnosis correctness and prescribed treatment regimen.
Another examination advantage at TAMC is the ability to perform innovative molecular genetic tests. All histological materials after biopsy are examined using such modern genetic panels as Foundation One and Caris Molecular Testing. These tests can determine specific types of mutations in malignant cells, as well as the presence of specific receptors on their surface. Then such data are used for individual immunobiological therapy drugs manufacture or for targeted drugs selection. In modern medicine this approach is called personalized oncology. And our patients have access to all the benefits of such innovative testing and treatment.
Depending on tumor location, doctor may prescribe additional laboratory and instrumental examination methods. This is decided separately for each child, based on his clinical situation.
Prognosis for germ cell tumors
The prognosis for germ cell tumors is favorable if it can be completely removed surgically. For children with stage 1 or 2 disease, the treatment success rate exceeds 90%. Survival rates for later stages (stage 3 or 4) are estimated to be approximately 80–85%. However, these indicators are considered conditional, since certain factors may affect the likelihood of a patient’s full recovery.
Modern treatment of childhood germ cell tumors
Germ cell tumors are serious malignant diseases with a serious prognosis. Therefore, in our clinic, not only the pediatric oncologist is involved in treatment plan development. An entire multidisciplinary team works on each individual case. Surgeons, neurosurgeons, radiation therapists, plastic surgeons, neurologists, chemotherapists, pediatricians, rehabilitation specialists, physical therapists, psychologists and other specialists provide assistance to a seriously ill child. Only together decisions are made about one or another diagnosis and treatment method, and all its pros and cons are assessed.
Surgery is the main treatment for germ cell tumors. The goal of treatment is complete, if possible, tumor removal. For early stage disease, if tumor can be completely removed surgically, another type of treatment may not be prescribed. Germ cell tumors require special care from a specialist performing a surgical intervention, since they can grow in close proximity to vital organs and large vessels or nerve trunks. All our surgeons have extensive experience performing such surgeries and masterfully possess the technique of such complicated surgical interventions.
Chemotherapy can be used alone or in combination with surgery to treat germ cell tumors. The type of chemotherapy depends on several factors, namely child’s age, the exact diagnosis and tumor location, and therapy response. Germ cell tumors usually respond well to chemotherapy drugs. Polychemotherapy regimens for children include drugs such as bleomycin, etoposide and cisplatin. Our department uses only high-quality drugs from well-known manufacturers. So we are confident in therapy effectiveness and its safety.
Chemotherapy may be used before surgery to shrink the tumor and make it easier to remove. Treatment may also be prescribed after surgery to destroy all remaining cancer cells in order to prevent tumor recurrence.
For recurrent germ cell tumors or for neoplasms that are primarily resistant to standard treatment protocols, high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation (own bone marrow transplantation) is used as an option to save the child’s life.
Radiation therapy is not usually used due to good effectiveness of chemotherapy and/or surgery. However, germ cell tumors are sensitive to radiation and it can be used in cases of disease recurrent or progressive.
All of our patients are also eligible to participate in clinical trials of experimental drugs or treatment methods. For some of them, this is a real chance for a full recovery and prolongation of life, this is especially true for those children for whom all known treatment methods have proven ineffective.
