From the Guidelines
Embryonic Tumors
Embryonic tumors, also known as embryonal tumors, are a type of cancer that typically affects children and young adults, arising from embryonic cells that have failed to mature properly 1.
Characteristics and Types
These tumors can occur in various parts of the body, including the brain, where medulloblastoma is a common example, and the adrenal glands, where neuroblastoma is frequently found 1. Medulloblastoma is both the most common CNS embryonal tumor and the most common malignant pediatric brain tumor, with distinct molecular subgroups that influence prognosis and treatment 1.
Management and Treatment
The management of embryonic tumors usually involves a combination of surgery, chemotherapy, and radiation therapy. The specific treatment approach depends on the type and location of the tumor, as well as the patient's overall health 1. For example, in the case of medulloblastoma, treatment may involve surgery to remove as much of the tumor as possible, followed by craniospinal radiation therapy and chemotherapy with medications such as vincristine, cisplatin, and cyclophosphamide.
Importance of Multidisciplinary Care
It's essential to note that the treatment of embryonic tumors should be individualized and managed by a multidisciplinary team of healthcare professionals, including pediatric oncologists, neurosurgeons, and radiation oncologists 1. This approach ensures that patients receive comprehensive care tailored to their specific needs, improving outcomes and quality of life.
Key Considerations
- Early diagnosis and genetic testing can significantly impact treatment decisions and outcomes for patients with embryonic tumors 1.
- Family history and genetic predisposition play a crucial role in the risk assessment and management of these tumors 1.
- Ongoing research and advancements in genetic testing and targeted therapies offer hope for improved treatments and outcomes for patients with embryonic tumors 1.
From the Research
Definition of Embryonic Tumors
- Embryonal tumors are highly malignant cancers of the central nervous system, with a relatively high incidence in infants and young children 2.
- They are highly cellular tumors with brisk mitotic activity, and they share a propensity for dissemination throughout the neuroaxis 3.
- Embryonal tumors comprise a large heterogeneous group of highly malignant tumors that predominantly affect children and adolescents 4.
Types of Embryonic Tumors
- Medulloblastoma (MB) is a type of embryonal tumor that can be separated into four distinct subgroups with distinct clinicopathologic characteristics 2.
- Atypical teratoid rhabdoid tumor (ATRT) is a type of embryonal tumor that can be distinguished from histologically similar tumors by virtue of characteristic molecular findings 2, 4.
- Embryonal tumor with multilayered rosettes (ETMR) is a type of embryonal tumor that is characterized by the presence of ependymoblastic rosettes formed by undifferentiated neuroepithelial cells and neuropil 4.
- Other types of embryonal tumors include pineoblastoma, medulloepithelioma, CNS neuroblastoma, CNS ganglioneuroblastoma, and CNS embryonal tumors with rhabdoid features 4, 5.
Diagnosis and Treatment
- Embryonal tumors can be accurately diagnosed with pediatric-specific sequencing techniques 2.
- Methylation analysis can also allow further subgrouping of ATRT and pineoblastoma 2.
- The treatment of patients with embryonal tumors includes a combination of surgical resection, radiotherapy (focal or craniospinal), and chemotherapeutic agents 4, 6.
- Novel targeted therapies are expected in the treatment of patients with medulloblastoma due to the identification of the main driver genes 4.