Welcome to the new Gustave Roussy website
A new website designed to offer you a simpler, clearer, and more intuitive experience. Patients, caregivers, healthcare professionals, and donors: find information, news, and services more easily.
Germinal Tumors in Children
Germinal tumors develop from primitive cells that are destined to form oocytes or spermatozoa. They can occur in the ovaries or testicles, but also outside the gonads, particularly in the sacrococcygeal region, the mediastinum, or the brain. Some are benign, others are malignant, and many involve different types of tissue within a single tumor.
Pediatric germ cell tumors are rare and account for approximately 3% of childhood cancers. Their distribution varies with age. In infants and very young children, teratomas—which are often extragonadal and benign—are particularly common. Starting at puberty, gonadal, mediastinal, and intracranial tumors become more common.
One family of tumors, several diseases
The location, age, histology, markers, and extent of the tumor must be considered together. A mature teratoma, a dysgerminoma, and a yolk sac tumor are not treated in the same way.
Why can they appear outside the gonads?
During embryonic development, germ cells migrate toward the future gonads. In rare cases, some cells may remain along this path and give rise to an extragonadal tumor, often located along the body’s midline.
Gonadal locations, which are particularly common after the onset of puberty.
A common site in infants and young children, often associated with a teratoma.
Located between the lungs; more common in adolescents, particularly boys.
Intracranial tumors are most commonly diagnosed in older children and adolescents. They are managed according to specific protocols.
Deep location behind the abdominal organs
Rare locations, sometimes diagnosed as early as the neonatal period.
Two distinct paths
Extracranial and intracranial germ cell tumors have different classifications and treatments. A general page may present them together, but their treatment pathways must remain clearly separate.
The Main Types
A tumor composed of differentiated tissue. It is generally benign and is treated with complete surgical removal.
A tumor containing immature embryonic tissue. Its behavior and treatment depend on the site, age, grade, stage, and whether or not it has malignant components.
A malignant tumor that typically secretes AFP; it is common among malignant tumors in young children.
Names vary by website. These tumors are highly sensitive to chemotherapy and, in the case of intracranial forms, to radiation therapy.
Rare malignant forms, often found within mixed tumors and capable of secreting beta-hCG.
A combination of several factors. The strategy is guided by the malignant component, the markers, the stage, and the site.
Symptoms
Symptoms vary by location and may change gradually.
An increase in size, a hard lump, or asymmetry—usually not very painful.
Abdominal pain or a mass, a distended abdomen, digestive problems, or a medical emergency related to torsion.
External or pelvic mass, constipation, urinary problems, or neurological signs.
Cough, chest pain, shortness of breath, or swelling of the neck and face.
Headaches, vomiting, visual disturbances, thirst, and excessive urination or pubertal abnormalities, depending on the location.
Beta-hCG secretion can cause precocious puberty, especially in boys.
When to Seek Immediate Medical Attention
Sudden testicular pain, sudden abdominal pain accompanied by vomiting, difficulty breathing, swelling of the face or neck, neurological symptoms, or a rapid deterioration in overall health require urgent evaluation.
The Diagnosis
Tumor Markers
Alpha-fetoprotein (AFP) and beta-hCG aid in diagnosis, staging, and follow-up. AFP levels must be interpreted according to age, as they are physiologically elevated in infants. LDH can provide additional information, particularly in germ cell tumors, dysgerminomas, and seminomas. Normal marker levels do not rule out a germ cell tumor.
Imaging
Ultrasound is the initial test for an ovarian or testicular mass. MRI and CT scans determine the local extent of the tumor and look for metastases. If an intracranial lesion is suspected, brain MRI and, in some cases, spinal MRI are essential. The diagnostic workup is tailored to the site and the suspected histology.
Pathology
A surgical specimen or biopsy is used to identify the various components. In certain secretory tumors and specific locations, the combination of the site, imaging, and biomarkers can strongly guide the strategy prior to definitive surgery.
Treatments for extracranial forms
Surgery and chemotherapy play different roles depending on the type of tumor. For a mature or immature teratoma without a malignant component, complete resection may be sufficient. For a malignant germ cell tumor, surgery is combined with platinum-based chemotherapy depending on the location, stage, and risk group.
Ovarian Tumors
Surgery aims to preserve the uterus and the contralateral ovary. A unilateral adnexectomy may be necessary to remove a malignant tumor intact. Chemotherapy depends not only on whether the tumor has spread but also on the histology, stage, and changes in tumor markers.
Testicular Tumors
In young children, conservative surgery may be considered for a suspected benign lesion. A malignant tumor generally requires an inguinal orchiectomy, which may or may not be followed by chemotherapy depending on the stage, age, and tumor markers.
Sacrococcygeal and mediastinal locations
Treatment combines expert surgical intervention and, for malignant forms, appropriate chemotherapy. In the sacrococcygeal region, the coccyx must generally be removed along with the tumor to reduce the risk of recurrence. A large mediastinal tumor may require chemotherapy prior to surgery.
Radiation therapy is not a standard treatment for extracranial forms of the disease
High sensitivity to chemotherapy usually makes it possible to avoid radiation therapy. Intracranial tumors present a different situation, in which radiation therapy remains an important treatment option depending on the type of tumor and the risk involved.
Intracranial Germ Cell Tumors
They occur primarily in the pineal and suprasellar regions. Diagnosis is based on MRI, blood markers, and sometimes cerebrospinal fluid markers, as well as a biopsy when the markers alone are insufficient to determine the nature of the tumor.
Germinomas are treated with chemotherapy followed by appropriate radiation therapy. Non-germinomas are treated with a more intensive regimen combining chemotherapy, radiation therapy, and sometimes surgery to remove residual tumor. The doses and treatment volumes depend on the diagnosis, extent of the disease, and the patient’s response.
The term "seminoma" depends on the location
A seminoma is found in the testis, a dysgerminoma in the ovary, and a germinoma in the central nervous system. These tumors share biological characteristics, but their treatments are not interchangeable.
Prognosis and Risk Factors
The prognosis for pediatric germ cell tumors is generally favorable, but it varies depending on age, location, histology, stage, biomarkers, and tumor shrinkage in response to treatment. Localized forms are often curable. Certain mediastinal locations, very extensive disease, or relapses remain more difficult to treat.
The decline in marker levels is being monitored
After treatment, AFP and beta-hCG levels should decrease in accordance with their biological half-lives. An abnormal decline may warrant further evaluation to assess the response to treatment, the presence of active tumor tissue, and the appropriateness of the treatment.
In the event of a relapse
The strategy depends on the site, histology, time since diagnosis, biomarkers, and prior treatments. It may involve a combination of surgery, adjuvant chemotherapy, high-dose chemotherapy in certain situations, and participation in a clinical trial. A pathological review and a new multidisciplinary discussion are essential.
Preserving fertility and minimizing long-term effects
Most patients who have been cured have a long life expectancy. Treatment decisions therefore aim to minimize side effects while maintaining efficacy.
Sperm preservation, ovarian tissue preservation, or other options are discussed prior to gonadotoxic treatment when circumstances permit.
Cisplatin can cause hearing loss, which may require audiograms.
Renal function and electrolyte levels are monitored during and after platinum-based treatments.
Bleomycin may cause pulmonary toxicity in treatment regimens that include it.
Following an intracranial or gonadal tumor, growth, puberty, and hormones may require specialized monitoring.
Support is provided as needed for body image, sexuality, schooling, academic studies, and returning to activities.
Research and Development of Protocols
International research aims to maintain high cure rates while reducing cumulative doses and late effects. The MaGIC consortium combines data from pediatric and adult trials to improve risk stratification and our understanding of rare tumors.
The Méta-AFP study examined the prognostic value of declining AFP levels in extracerebral germ cell tumors and hepatoblastomas. For a page to be permanent, it must be presented as a research study and not as a trial that is necessarily still ongoing.
Patient Care at Gustave Roussy
The Department of Pediatric and Adolescent Oncology coordinates patient care with teams from pediatric surgery, urology, gynecology, neuro-oncology, imaging, pathology, clinical laboratory medicine, endocrinology, reproductive medicine, and supportive care. Each case is discussed during a multidisciplinary team meeting.
Fertility preservation, endocrine monitoring, and psychological support are integrated into the care pathway. Specialized procedures are arranged with partner institutions when necessary.
Key Points
-
Gonadal or extragonadal tumors
The ovaries, testicles, sacrococcyx, mediastinum, and brain are the primary sites.
-
Two Key Markers
AFP and beta-hCG guide diagnosis and monitoring, but their levels may be within the normal range.
-
A wide range of histological types
Teratomas, germinomas, yolk sac tumors, and mixed-type tumors require different treatments.
-
Appropriate Surgery and Chemotherapy
The role of each treatment depends on the site, stage, and malignant component.
-
A Unique Path for the Brain
Intracranial tumors require specific neuro-oncology protocols.
-
Preserving Fertility
Gonadal function and long-term effects are assessed prior to treatment.