What are the other Names for this Condition? (Also known as/Synonyms)

• CNS Neuroblastoma
• Primary Cerebral Neuroblastoma
• Primary Intracranial Neuroblastoma

What is Central Nervous System Neuroblastoma? (Definition/Background Information)

• A Central Nervous System Neuroblastoma is a tumor that may form anywhere within the central nervous system (CNS). The brain along-with the spinal cord constitute the central nervous system (CNS) of the body. A CNS/brain tumor may be described as a mass of abnormally growing cells arising from the brain tissue (brain parenchyma and meninges) or the spinal cord (central spine)
• Broadly, these tumors are classified as benign (non-cancerous) and malignant. Malignant tumors are cancerous and can spread or metastasize to other regions of the body. They generally exhibit rapid growth and, in some cases, may present symptoms during the early stages of development
• The World Health Organization (WHO) classifies central nervous system tumors according to their grade and histological subtypes. A tumor subtype is determined by a pathologist after examining a tissue biopsy of the tumor under the microscope. This WHO classification system is used by healthcare professionals all over the world in diagnosing and treating these tumors
• Neuroblastoma is a rare malignant tumor that is mostly diagnosed in infants and young children. It develops from immature nerve cells of the sympathetic nervous system. The name is derived as “neuro” meaning nerves, and “blastoma” meaning cancer affecting immature (developing) cells. A majority of cases (about 70% of them) are diagnosed in the advanced stages among the pediatric population
• Neuroblastomas most commonly originate in the adrenal glands, which are located on top of the kidneys. These tumors can also arise in other areas of the body such as the abdominal cavity, neck, chest, or pelvis. Generally, neuroblastomas are not known to involve the central nervous system; although, occasionally, they can metastasize to involve the CNS (called secondary neuroblastomas)
• Primary Central Nervous System Neuroblastoma, with origin in the brain and/or spinal cord, is a highly uncommon and aggressive malignancy. It is a high-grade tumor that may arise anywhere in the brain but is mostly observed at supratentorial sites such as the frontal and temporal lobes or the ventricles. A large proportion of these tumors are found in childhood, with a majority of cases occurring in children less than 10 years old
• According to WHO, Central Nervous System Neuroblastoma is part of a group designated “embryonal tumors”, which are all high-grade malignancies. It is a histologic subtype of a primitive neuroectodermal tumor (PNET). High-grade tumors are known to exhibit aggressive behavior and grow rapidly. These tumors are known to infiltrate widely and destroy tissues, causing necrosis. They are also known to recur sooner following treatment
• The cause of formation of Central Nervous System Neuroblastoma is not well-established, although several genetic and chromosomal abnormalities are implicated. A large majority of tumors occur sporadically. The risk factors for tumor development are not well-established but may include certain genetic disorders (such as Beckwith-Wiedemann and DiGeorge syndromes) and a positive family history
• The signs and symptoms of Central Nervous System Neuroblastoma depend on the location of the tumor. Individuals may experience headaches, weakness in different parts of the body, convulsions, vision and speech disturbances. If the ventricular system is affected, it may result in obstruction of cerebrospinal fluid flow leading to hydrocephalus and other complications. Large-sized tumors may compress adjacent brain tissue resulting in complications. Metastatic tumors may spread outside the brain and spinal cord
• The treatment modalities for Central Nervous System Neuroblastoma may include a combination of surgery, radiation therapy, and chemotherapy. The prognosis is determined by a wide variety of factors, such as age of the individual, tumor size, and overall health status. Nevertheless, the prognosis is extremely poor, since it is a high-grade malignant tumor

Who gets Central Nervous System Neuroblastoma? (Age and Sex Distribution)

• Central Nervous System Neuroblastoma is a rare type of embryonal tumor
• Most of the tumors are diagnosed in children below age 10; sometimes, it may be present in infants too
• Some cases are reported in older children and adults
• Both males and females are affected, with a slight preference noted for males/boys
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Central Nervous System Neuroblastoma? (Predisposing Factors)

Infants and young children report a higher incidence of Central Nervous System Neuroblastoma formation. The risk factors for neuroblastoma, in general, include:

• Rarely, children with a family history of neuroblastoma may have a higher risk for developing this malignant tumor. A vast majority of children who develop the tumor, do not have a family history of the same
• Birth defects: Presence of congenital anomalies during fetal development may increase the risk for neuroblastoma in children
• Association with certain genetic disorders are noted including:
  • Neurofibromatosis type 1 (NF1)
  • Beckwith-Wiedemann syndrome
  • DiGeorge syndrome
  • Hirschsprung disease
  • Hypoventilation syndrome

Overall, the following factors may increase one’s risk for brain tumors:

• Advancing age is an important risk factor; although, some tumors are common among children
• In general, males are at a higher risk for brain tumors than females
• Positive family history: It is reported that certain genetic (hereditary) factors are responsible for the formation of certain brain tumors. Such hereditary conditions include:
  • Li-Fraumeni syndrome
  • Neurofibromatosis type 2 (NF2)
  • Tuberous sclerosis
  • Turcot syndrome
  • Von Hippel-Lindau disease
• Environmental factors such as ionized radiation exposure (both high-dose and low-dose)
• Individuals who have undergone radiation therapy for other cancers involving the head and neck region are at an increased risk
• Viral infections involving pathogens, such as Epstein-Barr virus (EBV) and cytomegalovirus (CMV), have been implicated
• Exposure to certain chemicals, pesticides, or products, either at work or at home
• Some reports indicate that the use of mobile phones may increase one’s risk due to exposure to electromagnetic waves. However, the scientific research community is presently divided on this factor
• Head injuries
• History of seizures
• Hormonal factors: Studies have shown a correlation between hormones and some types of brain tumors
• Intake of N-nitroso compounds, either in one’s diet (such as cured meat) or via vitamin supplements may increase one’s risk

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Central Nervous System Neuroblastoma? (Etiology)

The exact cause of development of Central Nervous System Neuroblastoma is not well-understood. A vast majority of neuroblastomas (about 98% of them) occur sporadically. Research scientists believe that the cause of brain tumors may involve a combination of several factors including genetic, environmental, and occupational factors.

• As with other forms of cancer, neuroblastoma is observed to develop because of the transformation of normal body cells to abnormal cells, as a result of genetic mutations
• These cancer cells divide uncontrollably, as they lack the mechanism of regulating their growth and division. Ultimately, the accumulation of cells leads to the formation of tumors

Several genetic abnormalities have been identified as being causative for the formation of sporadic and/or inherited forms of neuroblastoma. Some of these include:

• Involvement of the ALK gene is observed in some sporadic cases, and in nearly 75% of the familial cases (inherited forms)
• Occasionally, PHOX2B gene mutations are noted
• Deletion of genetic material on chromosomes 1 and 11
• Amplification of MYCN gene

Neuroblastoma starts to form at the embryonic stage of immature nerve cells, called neuroblasts, which are found in certain tissues.

• Normally, during the developmental process, neuroblasts mature into functioning nerve cells and are typically fully-developed by birth
• However, in the case of neuroblastoma, the immature neuroblasts do not develop into nerve cells. Instead, they divide and multiply uncontrollably, leading to the formation of a tumor mass (or neuroblastoma)

What are the Signs and Symptoms of Central Nervous System Neuroblastoma?

The signs and symptoms of brain tumors may be significantly different from one individual to another. It may be mild or severe, depending on several factors. The onset of symptoms and speed of progression of the tumor can vary. In many individuals, tumors that grow slowly and remain asymptomatic, are only diagnosed during certain radiological imaging studies of the head and neck region, which are performed for unrelated health conditions. However, Central Nervous System Neuroblastoma is an aggressive and rapidly-growing pediatric tumor that may present signs and symptoms early in its formation.

The signs and symptoms of CNS Neuroblastoma primarily depend on the location and size of the tumor. The tumor may develop in any part of brain or spinal cord but is usually noted in the cerebral hemispheres (supratentorium), including the frontal and parietal lobes (higher number of cases), followed by other sites, such as the temporal and occipital lobes, the third, fourth, and lateral ventricles.

The signs and symptoms may include:

• Headaches that may increase in intensity and be persistent
• Nausea and vomiting
• Irritability
• Numbness and tingling sensation
• Muscle weakness; loss of strength in the arms of legs
• Neck pain
• Visual impairment such as blurred vision, double vision or poor eyesight
• Ringing in the ears (tinnitus)
• Hearing impairment or hearing loss
• Speech problems
• Insomnia or loss of sleep, or excessive sleepiness (usually during daytime)
• Increased intracranial pressure due to obstruction of CSF drainage, resulting in nausea, vomiting, headaches, and seizures
• Obstruction of the CSF also leads to increased size of the head and associated complications, a condition termed as obstructive hydrocephalus
• Damage to peripheral nerves
• Tremors
• Seizures or convulsions
• Lack of coordination; unsteadiness and loss of balance (vertigo)
• Dizziness and fainting
• Confusion
• Changes in one’s behavior; personality changes
• Mental impairment
• Memory loss

When the spinal cord is involved, the following may be noted (based on tumor location):

• Back pain and/or chest pain
• Numbness and tingling sensation
• Weakness of the body or lower extremities; loss of strength in the arms of legs
• Pain in the arms and legs, depending on the nerves that are compressed by the tumor
• Severe cases may result in loss of bowel and bladder control; especially observed in lower spine tumors

Large tumors can cause significant signs and symptoms. Most of the tumors are single; but occasionally, multiple tumors can be detected growing simultaneously in the brain or spinal cord region, particularly in individuals with genetic disorders. The associated symptoms of the underlying condition, if any present, may be noted.

How is Central Nervous System Neuroblastoma Diagnosed?

The tumors may be detected when there is a sudden worsening of the symptoms prompting the healthcare provider to perform radiological studies of the brain and/or spinal cord. 

Central Nervous System Neuroblastoma reportedly resembles a medulloblastoma in its behavior and may be misdiagnosed for the same.

The diagnosis of CNS Neuroblastoma may involve the following tests and examinations:

• Complete physical examination with thorough evaluation of the individual’s medical history (including family history of any genetic disorder)
• Assessment of the presenting signs and symptoms
• Visual field tests; tests to assess visual acuity
• Neurological, motor skills, and cognitive assessment:
  • Checking intellectual ability, memory, mental health and function, language skills, judgment and reasoning, coordination and balance, reflexes, sensory perceptions (space, sight, hearing, touch)
  • The healthcare provider/neurologist may use the Karnofsky Performance Scale in order to assess the neurological functioning of the individual’s central nervous system (CNS)
• Catecholamines urine test: It is a test to assess the levels of catecholamine hormones in urine. It is used to diagnose hormone-releasing tumors (or neuroendocrine tumors). An elevated level of catecholamines may indicate the presence of a neuroblastoma
• Electroencephalography (EEG)
• Electromyography with nerve conductivity tests
• Imaging studies that may be performed include:
  • X-ray of head and neck
  • Computerized tomography (CT) scan of the head and neck region; CT with contrast - the scans may reveal calcifications
  • Magnetic resonance imaging (MRI) scan of the central nervous system (brain and spine); MRI with contrast agents such as gadolinium
  • Cerebral angiographic studies or MR angiography: An angiogram involves injecting dye into the bloodstream, which makes the blood vessels to appear visually on X-rays. The X-ray may show a tumor in the brain or the blood vessels leading into the tumor
  • Magnetic resonance spectroscopy: This radiological technique is used to study the chemical profile of the tumor. It is often performed with and compared to corresponding MRI scan images of the affected region
  • Positron emission tomography (PET scan): A PET scan is a nuclear medicine imaging technique that uses three-dimensional images to show how tissue and organs are functioning. It may be performed to detect any malignancy, and if there is a metastasis (spread) of the tumor to other regions
• Iodine-123 metaiodobenzylguanidine scan (123-I-MIBG): In a MIBG scan, a radioactive substance is injected into blood, which will then bind to the neuroblastoma cells. This test helps in determining whether the tumor has spread to other regions of the body or not
• Cerebrospinal fluid analysis, where a spinal tap or lumbar puncture procedure may be performed: This diagnostic test is used to remove a sample of cerebrospinal fluid (CSF) from the spaces in and around the brain and spinal cord. The sample is removed from the lower spinal cord using a thin needle, and it is then checked for the presence of cancer cells
• Bone marrow aspiration and biopsy: It is an invasive procedure that involves the removal and examination of a sample of the bone marrow. The procedure helps in detecting the presence of cancer cells in bone marrow
• Molecular testing to determine genetic mutations, if any

Tissue biopsy: A biopsy of the affected region is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy sample under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy sample under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis.

There are two ways to perform a biopsy:

• First, a biopsy can be performed as part of a surgical procedure to remove the brain tumor
• Second, a stereotactic needle biopsy can be performed when the tumor is deep within the brain or located in a sensitive area. In this procedure, the surgeon drills a small hole (called a burr hole) into the skull. A thin needle, guided by CT or MRI scanning, is then inserted through the hole to help in the removal of tissue

A differential diagnosis to eliminate other conditions or tumor types may be considered, before arriving at a definitive diagnosis. These may include:

Radiological differential diagnosis may include:

• Astrocytoma
• Ependymoma
• Medulloblastoma
• Medulloepithelioma
• Meningioma
• Oligodendroglioma
• Pineoblastoma

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Central Nervous System Neuroblastoma?

The possible complications associated with Central Nervous System Neuroblastoma include:

• Emotional and mental stress for both the patients and the caretakers, due to the diagnosis and treatment of a malignant brain tumor
• Large undetected tumors can severely affect brain function and be disabling or even life-threatening, due to mass effect/compression of brain tissue
• Large tumors may also press against the skull (or other) bones causing it to expand, causing severe complications
• Spinal cord compression is another potential complication associated with neuroblastoma located on the spine. As the tumor grows, it can press upon the spinal cord leading to paralysis of the arms/legs and pain
• Some tumors can secrete vasoactive intestinal peptide (VIP) hormone causing severe watery diarrhea
• Tumors in the neck can cause Horner’s syndrome that consists of contracted pupil (myosis), drooping eyelid (ptosis), decreased sweating (anhidrosis), depression of eye ball (or enophthalmos, which occurs due to compression of nerves)
• Trouble with concentration
• Severe loss of memory
• Dementia including personality changes causing a reduced quality of life
• Bleeding in the brain (brain hemorrhage)
• Paralysis and coma
• Peritumoral brain edema (PTBE): Presence of fluid around the tumor region in the brain resulting in severe symptoms and complications
• Leptomeningeal seeding: In many cases, the tumors are known to spread to other regions of the central nervous system through the cerebrospinal fluid, called CSF dissemination. Leptomeningeal seeding is a typical complication of CNS Neuroblastoma
• The tumor may spread (metastasize) to other sites of the body leading to severe complications and treatment challenges. These tumors are known to metastasize outside the brain
• Complications due to an underlying genetic disorder, if any present

Neuroblastomas may have the ability to secrete hormones, which can affect distant ‘normal’ body tissues and cause symptoms. This can lead to a condition known as paraneoplastic syndrome. Paraneoplastic symptoms are rare in children; but, it can result in permanent neurological damage.

Complications may arise from surgery, radiation therapy, chemotherapy, or other treatment modalities.

Surgical complications:

• Damage to the muscles, vital nerves, and blood vessels, during surgery to remove the tumor; injury to unaffected brain tissue during surgery
• Post-surgical infection at the wound site is a potential complication
• Sometimes, removing the tumor can worsen the signs and symptoms in some individuals
• Recurrence of the tumor after surgery may be observed; malignant tumors often have very high recurrence rates

Chemotherapy side effects depend on the type of chemotherapy medication used and may include:

• Nausea and vomiting
• Hair loss
• Mouth sores
• Loss of appetite
• Diarrhea
• Fatigue
• Increased risk for infections
• Easy bruising

Radiation therapy can cause short-term side effects. It depends on the type used and may include:

• Nausea and vomiting
• Hair loss
• Dryness of mouth; loss of taste
• Headaches
• Fatigue
• Skin color changes; usually darkening of skin on the face and neck may be noted
• Speech and hearing difficulties
• Memory issues
• Seizures

Long-term side effects of radiation therapy (seen after 6 months or beyond) may include loss of memory, impaired brain function, and appearance of symptoms that mimic stroke.

How is Central Nervous System Neuroblastoma Treated?

The treatment modality for brain tumor is chosen, depending on the type, size, location, and stage of the tumor, tumor growth rate, age and health status of the individual. Often, a multidisciplinary team of specialists including ophthalmologists, neurologists, oncologists, surgeons, radiation therapy experts, and other healthcare professionals are involved in managing the condition.

Currently, there are no standardized treatment protocols available for Central Nervous System Neuroblastoma, since it is a highly uncommon tumor. According to some reports, the tumor may be treated similar to a medulloblastoma.

Some of the treatment measures may include:

• Symptomatic treatment may involve the use of antiseizure medications, painkillers, steroids (to reduce inflammation), and administration of anti-depressants
• Placement of ventriculoperitoneal (VP) shunt: It is an invasive procedure that involves the use of a medical device called a VP shunt. The device is placed in the ventricles of the brain to treat hydrocephalus, and thus helps relieve excess CSF pressure on the brain

Surgical treatment: Surgery to remove the entire tumor or just part of the tumor depends on several factors including the risk assessment by a neurosurgeon, with respect to the potential injury to nearby brain tissues. It is also considered based on the location, size, and overall health of the individual. A complete removal of the tumor can result in a cure in many patients. Although, in many cases, removal of the tumor offers a lot of surgical challenges to the healthcare providers, due to the complexity of the tumor site involved.

• Craniotomy: It is a surgical procedure wherein a tiny hole is created in the cranium to access and remove the tumor. The main goal of surgery is to remove as much tumor as possible without damaging the surrounding brain tissue
• Microsurgery: The surgery is performed using microsurgical techniques, which can help minimize damage to the accompanying nerve. Since the tumor mostly affects the covering around the nerves, it can be completely removed without affecting the nerve in most cases. The following techniques may be employed:
  • Subtotal removal; when the tumor is incompletely removed
  • Near total removal; when most of the tumor is removed and there is very little tumor remnants
  • Total tumor removal; when the tumor is removed completely
• In some cases, embolization of the tumor may be performed, in order to shrink the tumor size, before major invasive procedures are undertaken. In embolization, the blood supply to the tumor is cut-off, resulting in its shrinkage. This also helps reduce blood loss during a surgical resection
• Radiosurgery for individuals who cannot undergo surgery or have recurrent tumors
• Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals

Surgical procedures to remove brain tumors carry risks, including infection, possible damage to healthy brain tissue, swelling, or possible fluid build-up in the brain.

Radiation therapy: There are a variety of radiation therapy methods that can be used to treat the tumors. Radiation therapy is performed either after removal of the tumor (to destroy any remaining meningioma cells), or in individuals where surgical removal procedure is not an option. Radiation therapy is helpful to reduce the rate of recurrence. It is also recommended for individuals where the tumor is removed partially.

The types of radiation therapy include:

• Standard external radiation beam therapy using a beam of high-energy X-rays; it is the most common form of radiation therapy used to treat cancers
• 3-dimensional conformal radiation therapy, where 3-d images of the tumor are created using radiological imaging scans to help design appropriate radiation therapy beam size and angles
• Intensity modulated radiotherapy (IMRT): It is a procedure that uses computer-controlled linear accelerators to deliver precise radiation to a malignant tumor
• Stereotactic radiosurgery using Gamma Knife or CyberKnife technique
• Proton radiation therapy: It is a form of external beam therapy using high-energy proton beams to destroy the tumor cells
• Fractionated radiation therapy: It is a good option for individuals whose tumors are too large for radiosurgery, or if the tumor is in a sensitive location. In this therapy, small doses of radiation are administered for a certain set period

Some individuals may experience a few side effects, while others may experience none. These may include fatigue, headaches, hair loss, and scalp irritation. Radiation therapy beams may affect the pituitary gland causing it to dysfunction (bringing about hormonal changes in the body).

Chemotherapy: The response of the tumor to chemotherapy is an important consideration while using this treatment modality. High-dose chemotherapy is administered in CNS Neuroblastoma, particularly in very young children.

• Whether to use chemotherapy as a treatment modality is determined by healthcare provider on case-by-case basis
• Chemotherapy may be administered for those tumors that cannot be effectively treated by surgery or radiation therapy. The administration of some drugs may slow the rate of growth of the tumor

The following chemotherapy agents and methods have been used in higher grade tumors with varying beneficial results:

• Immunotherapy to stimulate an affected individual’s immune system
• Somatostatin analogs which prevent the release of growth hormones
• Hydroxyurea, epidermal growth factor receptor inhibitors, platelet-derived growth factor receptor inhibitors, and vascular endothelial growth factor inhibitors

There are side effects for chemotherapy, which depends upon the type and dose of drugs administered. The common chemotherapy side effects include nausea, vomiting, hair loss, loss of appetite, headache, fever, chills, and weakness.

Clinical trials: In certain advanced stage tumors, there may be some newer treatment options, currently on clinical trials, which can be considered for some patients depending on their respective risk factors.

Regular observation and periodic checkups to monitor the condition is strongly recommended following treatment. In order to avoid a relapse, or be prepared for a recurrence, the entire diagnosis, treatment process, drugs administered, healthcare recommendations post-treatment, etc. should be well-documented and follow-up measures initiated.

How can Central Nervous System Neuroblastoma be Prevented?

Presently, there are no specific methods or guidelines to prevent the formation of Central Nervous System Neuroblastoma. However, if it is associated with a genetic disorder, the following points may be considered:

• Genetic counseling and testing: If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Regular health check-ups might help those individuals with a history of the condition in the immediate family and help diagnose the tumor early

In general, the factors that may help reduce the incidence of brain tumor may include:

• Reducing exposure to ionizing radiation
• Avoiding cigarette smoke inhalation
• Weight loss, in case one is obese or overweight; maintaining a good BMI
• Availing early treatment for infections
• Using safety gear including wearing helmets when playing sports or while riding two-wheelers
• Wearing seatbelts while driving automobiles to minimize the risk for head/body injury

Cancer screenings are important tests in that they can detect cancer in its earliest stages, which would help optimize treatment efforts. Nevertheless, currently, there are no standardized screening tests for neuroblastomas.

Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory. Often several years of active vigilance are crucial and necessary.

What is the Prognosis of Central Nervous System Neuroblastoma? (Outcomes/Resolutions)

• The prognosis of Central Nervous System Neuroblastoma is extremely poor, since the tumor is a high-grade and aggressive malignancy
• The average survival period is around 12 months or less. Also, a higher rate of recurrence and leptomeningeal seeding is known to severely affect the prognosis

Typically, the factors that point to a poor prognosis for neuroblastoma include:

• Age of tumor onset beyond 18 months
• Advanced stage of tumor (including metastasis)
• Presence of N-MYC oncogene mutation: Tumors showing MYCN gene amplification have unfavorable histology
• Deletion of genetic material on the short arm (p) of chromosome 1
• Unfavorable Shimada histology index (a classification index for neuroblastomas)

In general, the prognosis of brain tumors may depend upon several factors that include:

• Tumor histology or grade of the tumor: The lower the grade, the better is the prognosis
• Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
• Age of the individual: Older individuals generally have poorer prognosis than younger individuals
• Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
• The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
• Location of the tumor is an important prognostic factor: Tumors at certain sites involving the central nervous system are more difficult to surgically excise and remove. They can also cause considerable neurological damage at certain sites in the brain and/or spinal cord. Tumors located near vital structures of the brain have poor prognosis
• Tumors that are slow-growing have better prognosis than rapidly-growing tumors
• Presence of certain genetic abnormalities may result in a rapid progression of tumor growth and development
• Sporadic tumors have generally better prognosis than syndromic tumors; tumors in a background of genetic disorders/syndromes may have additional complications
• The ease of accessibility and surgical resectability of the tumor (meaning, if the primary tumor can be removed completely). This is an important prognostic factor
• Extent of residual tumor in the body following a surgical resection: A complete surgical excision with entire tumor removal presents the best outcome. In some cases, large portions of the tumor may be removed; or, only a small portion for tissue biopsy
• Individuals with bulky disease may have a poorer prognosis
• Involvement of other vital organs may complicate the condition
• Metastatic spread: Spread of the malignancy to other body sites portrays a poorer prognosis
• Whether the tumor is occurring for the first time or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur 
• Response to treatment and absence of post-operative complications: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
• Progression of the condition makes the outcome worse
• Functional neurologic status based on neurological evaluation of the patient: A higher score on the Karnofsky Performance Scale may indicate more favorable outcomes

An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment.

Additional and Relevant Useful Information for Central Nervous System Neuroblastoma:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/cancer/