What are the different types of retinoblastoma?
Types of Retinoblastoma
- Unilateral retinoblastoma affects one eye and accounts for 60% to 75% of cases.
- Bilateral retinoblastoma affects both eyes.
- PNET (primitive neuroectodermal tumors) retinoblastoma occurs when an associated tumor forms in primitive nerve cells in the brain.
- Learn more about retinoblastoma:
What is metastatic retinoblastoma?
Retinoblastoma is a highly invasive malignant tumor that often invades the brain and metastasizes to distal organs through the blood stream. Invasiveness and metastasis of retinoblastoma can occur at the early stage of tumor development.
What does endophytic mean in medical terms?
Medical Definition of endophytic : tending to grow inward into tissues in fingerlike projections from a superficial site of origin —used of tumors — compare exophytic.
What is endophytic tumor?
Exophytic tumors were defined as when growth of the cancer was principally above the surface epithelium of the cervix. Endophytic tumors were defined when the tumor growth appeared prin- cipally to be below the surface epithelium.
What is Pinealoblastoma?
Pineoblastoma is a rare, aggressive type of cancer that begins in the cells of the brain’s pineal gland. Your pineal gland, located in the center of your brain, produces a hormone (melatonin) that plays a role in your natural sleep-wake cycle.
What is primitive neuroectodermal tumor?
Primitive neuroectodermal tumors (PNETs) are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone.
How many stages are there in retinoblastoma?
There are 2 staging systems for intraocular retinoblastomas. It’s important to know that regardless of the stage, almost all children with intraocular retinoblastoma can be cured if they are properly treated. But the stage has a bigger impact on whether the affected eye (or the vision in the eye) can be saved.
What genes cause retinoblastoma?
The most important gene in retinoblastoma is the RB1 tumor suppressor gene. This gene makes a protein (pRb) that helps stop cells from growing too quickly. Each cell normally has two RB1 genes.
Can retinoblastoma spread to the brain?
Retinoblastoma can sometimes spread through the optic nerve to the brain and the spinal cord (called the central nervous system, or CNS).
What is the endophytic pattern of retinoblastoma?
The endophytic pattern, in which the tumor grows in the direction of the vitreous cavity and invades the inner retinal layers, is evidenced by several fluffy preretinal tumors with seeding into the vitreous ( Figure ). 1 – 3 Understanding these growth patterns is key to differentiating retinoblastoma from other simulating lesions in young children.
What are the classification systems for intraocular retinoblastoma (AR)?
There are two commonly use classification systems for intraocular retinoblastoma. In the 1950’s, the Reese-Ellsworth classification system (see Table 1) was developed to predict the prognosis after treatment with radiation: a. Solitary tumor less than 4 disc diameter (DD) in size, at or behind equator. b.
What is the relationship between germline disease and retinoblastoma?
Germline cases represent approximately 1/3 of retinoblastoma cases. Patients with germline disease tend to have bilateral and multifocal tumors. They also have a significantly increased risk for secondary tumors including primitive neuroendocrine tumors in the brain (so called “trilateral retinoblastoma”).
How is retinoblastoma passed from parent to child?
Heritable forms of retinoblastoma may be passed from parents to children through germline mutations. Germline cases represent just over 1/3 of retinoblastoma cases. Patients with germline disease tend to have bilateral and multifocal tumors but may more rarely have unilateral disease.