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

• PDLM (Primary Diffuse Leptomeningeal Melanomatosis)
• Primary Diffuse Leptomeningeal Melanomatosis (PDLM)

What is Meningeal Melanomatosis? (Definition/Background Information)

• A 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). The brain along with the spinal cord constitute the central nervous system (CNS) of the body
• Broadly, brain 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
• Meningeal Melanomatosis is an uncommon tumor of the brain and/or spinal cord. The tumor is mostly observed in adults, but children are occasionally affected too. According to WHO, it is classified as one of the four subtypes of primary CNS melanocytic tumors. Melanocytic tumors may be mostly described as high-grade malignancies, which 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
• Meningeal Melanomatosis usually develops from the melanocytes (or pigmented cells) present within the subarachnoid space in the meninges; it is observed to spread diffusely in the region. Hence, the tumor is also known as Primary Diffuse Leptomeningeal Melanomatosis (PDLM). Many experts consider Meningeal Melanomatosis to be an aggressive form of meningeal melanocytosis (another melanocytic tumor). Some experts refer to meningeal melanocytosis and Meningeal Melanomatosis as diffuse melanosis
• Meningeal Melanomatosis is associated with several genetic conditions such as neurofibromatosis and tuberous sclerosis. In some, it may develop against a background of neurocutaneous melanocytosis, which is a rare, non-heritable congenital condition characterized by an abnormal proliferation of melanocytes in both the skin and brain. If the tumor is associated with cutaneous melanocytic lesions, the condition is called neurocutaneous syndrome (or phakomatosis)
• The signs and symptoms of Meningeal Melanomatosis depend on the location of the tumor. These diffuse melanocytic proliferations can invade the surrounding brain tissue and also involve the spinal cord. Individuals may experience headaches, weakness in different parts of the body, convulsions, vision and speech disturbances. Spinal tumors may cause pain at the site of the tumor, weakness in upper and lower limbs, one-sided paralysis, and loss of bowel or bladder control. Complications associated with malignant tumors include metastasis to other organs of the body
• The treatment modalities for Meningeal Melanomatosis may include a combination of surgery, radiation therapy, and chemotherapy; although, there are no well-established treatment protocols for the tumor. The prognosis is determined by a wide variety of factors, such as age of the individual, tumor size, and overall health status. Nevertheless, in most cases, the prognosis is reportedly unpredictable to poor

Who gets Meningeal Melanomatosis? (Age and Sex Distribution)

• Meningeal Melanomatosis is an infrequently occurring subtype of CNS melanocytic tumors that may be seen in a wide age range of adults. Some cases are rarely reported children
• An age range of 20 to 70 years is reported in the literature, with a peak incidence between 20 and 50 years. The average age of presentation is 42 years
• Both males and females are equally affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Meningeal Melanomatosis? (Predisposing Factors)

The following predisposing factors are associated with Meningeal Melanomatosis:

• Neurocutaneous syndromes: When Meningeal Melanomatosis is associated with cutaneous melanocytic lesions, the condition is called neurocutaneous syndrome (termed phakomatosis). These conditions are known to increase the risk for cancer development. They include:
  • Neurofibromatosis types 1 or 2 (NF1 or NF2)
  • Sturge-Weber syndrome
  • Tuberous sclerosis
  • Von Hippel-Lindau syndrome
• Neurocutaneous melanocytosis (NCM, or neurocutaneous melanosis): Meningeal Melanomatosis is associated with NCM that typically manifests as a giant melanocytic nevus (pigmented birthmark) at birth, along with increased intracranial pressure, headaches, seizures, decreased alertness, and numbness/tingling sensation on the face
• A family history of the above genetic conditions may place one at a higher risk for tumor formation

Typically, 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) conditions, such as Turcot syndrome, are responsible for the formation of certain brain tumors
• 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 Meningeal Melanomatosis? (Etiology)

The exact cause of development of Meningeal Melanomatosis is not well-understood. The tumor is generally associated with certain genetic conditions called neurocutaneous syndromes (phakomatoses).

• Meningeal Melanomatosis is a form of primary melanocytic tumor of the central nervous system (CNS). Melanocytic neoplasms are uncommon tumors that arise from the melanocytes present in the leptomeninges
  • Melanocytes are pigment producing cells that originate from neural crest during embryological development. As the fetus develops, the melanocytes migrate from the neural crest to a variety of structures in the body such as to the skin, eyes, oral and genital mucosa, and leptomeninges
  • The meninges are the membranous tissue encasing the CNS. It consists of three layers - the dura mater, arachnoid mater, and pia mater; which are the outer, middle, and inner layer respectively. Leptomeninges refers to the inner meningeal layers of arachnoid mater and pia mater
• Melanocytic proliferation within the CNS occur from melanocytes in the leptomeninges. The melanocytic tumors are usually located in the perimedullary and cervical region of the spinal cord, and in the posterior fossa of the brain. Rarely, they can be present in the thoraco-lumbar and cauda equina of spinal cord

Research scientists believe that the cause of brain tumors may involve a combination of several factors including genetic, environmental, and occupational factors. In general, it is known that cancers form when normal, healthy cells begin transforming into abnormal cells - these cancer cells grow and divide uncontrollably (and lose their ability to die), resulting in the formation of a mass or a tumor.

What are the Signs and Symptoms of Meningeal Melanomatosis?

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.

The signs and symptoms of Meningeal Melanomatosis primarily depend on the location and size of the tumor. In some cases, both the spinal and brain meninges may be invaded by the tumor (intracranial and intraspinal tumors). The tumor can cause severe leptomeningeal enhancement in the brain or spinal cord.

In the brain, an involvement of either the infratentorial or supratentorial leptomeninges may be noted. In some, the superficial tissues of the brain (brain parenchyma) may also be involved. Tumors are known to affect the spinal cord including the spinal canal, anywhere along its length; 43% of the Meningeal Melanomatosis cases involve the spinal cord. This may commonly include the thoracic region, and less commonly the cervical and cauda equina region.

Meningeal Melanomatosis can develop as a diffuse mass that spreads around the region. In brain, these tumors can involve the perivascular spaces with direct invasion into the surrounding brain tissue.

The signs and symptoms may include:

• Headaches that may increase in intensity and be persistent (seen in 46% of the affected individuals)
• Nausea and vomiting (seen in 37% of the cases)
• Irritability
• Numbness and tingling sensation
• Muscle weakness (seen in 22% of the cases)
• Visual impairment such as blurred vision, double vision, or poor eyesight
• Ringing in the ears (tinnitus)
• Hearing impairment or hearing loss
• Lack of coordination; unsteadiness and loss of balance (vertigo)
• Speech problems
• Cranial nerve palsy
• 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 (observed in children)
• When associated with neurocutaneous melanocytosis, they may also present congenital nevi (pigmented skin moles) and neurological and behavioral symptoms; this may be observed in children

When the spinal cord is involved, the following may be noted:

• Neck pain and stiff neck (in 24% of the individuals with spinal tumors)
• Back pain and/or chest pain (in 24% of the individuals)
• 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
• Spinal cord compression leading to numbness and pain
  • Nerve root compression (radiculopathy)
  • Compression injury to the spinal cord due to tumor (myelopathy)
• Severe cases may result in loss of bowel and bladder control; observed in lower spinal tumors
• Paralysis

Additionally, in some individuals the following may be noted:

• Some tumors behave like low-grade tumors, by not invading into the surrounding tissues
• Loss of reflex
• Hearing impairment or hearing loss
• Speech problems
• Insomnia or loss of sleep, or excessive sleepiness (usually during daytime)
• Tremors
• Lack of coordination; unsteadiness and loss of balance (vertigo)
• Dizziness and fainting
• Confusion
• Changes in one’s behavior; personality changes
• Mental impairment
• Memory loss

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, may be noted.

How is Meningeal Melanomatosis Diagnosed?

Some brain tumors may remain undiagnosed for prolonged periods, because they may be slow-growing and remain asymptomatic. The tumors are 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.

The diagnosis of Meningeal Melanomatosis may involve several tests and examinations. However, because of tumor rarity and a set of non-specific signs and symptoms, there is often a delayed diagnosis or even a misdiagnosis. The tumor features may resemble a host of other conditions including metastatic tumors to the CNS region (metastatic melanoma), aseptic meningoencephalitis, infectious meningitis, viral encephalitis, or lymphomas.

Thus, the healthcare provider should have a high index of suspicion for Meningeal Melanomatosis. A diagnosis of this primary tumor of the central nervous system is often one of exclusion; thus, an exclusion of several conditions may be needed to arrive at a diagnosis of Meningeal Melanomatosis. Moreover, according to some reports, biopsies along-with special studies, such as immunohistochemical analysis, are more effective than imaging scans and CSF analyses. It is also a lot easier to diagnose spinal tumors,

The diagnosis of Meningeal Melanomatosis 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, and sensory perceptions (space, sight, hearing, touch, etc.)
  • 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)
• Electroencephalography (EEG)
• Electromyography with nerve conductivity tests
• The thickening of the leptomeninges, termed leptomeningeal enhancement, may be a prominent finding on radiological imaging studies. The 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
• 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
• Molecular testing, as needed

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. Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, histochemical stains, molecular testing, and very rarely electron microscopic studies.

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:

• Aseptic meningoencephalitis
• Bacterial meningitis
• CNS lymphoma
• Fungal meningitis
• Meningeal melanocytoma
• Meningeal melanocytosis
• Metastatic carcinoma involving the central nervous system
• Primary malignant melanoma
• Viral encephalitis

Radiological differential diagnosis may include:

• Cavernous malformations
• Hemangioblastoma
• Hemorrhagic lesions
• Hemorrhagic metastases
• Intracranial metastatic melanoma
• Melanotic meningioma
• Other melanotic lesions
• When present in the spine:
  • Astrocytoma
  • Ependymoma
  • Hemorrhagic intramedullary spinal tumors

Note: Since the Meningeal Melanomatosis tumors involve the perivascular spaces and invade directly into surrounding brain tissue, it helps distinguish them from meningeal melanocytosis tumors.

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 Meningeal Melanomatosis?

The possible complications associated with Meningeal Melanomatosis 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 may spread extensively and severely compromise brain or spinal cord function and be disabling or even life-threatening, due to mass effect/compression of surrounding tissue
  • In the brain, large tumors may press against the skull (or other) bones causing it to expand, causing severe complications
  • In the spinal cord, large tumors may press against the vertebral bones causing it to expand or break easily when subjected to minor trauma including falls and accidents
• Chronic meningitis
• Spinal arachnoiditis from inflamed meningeal layers of the spinal cord causing severe burning/stinging pain and associated neurological symptoms; this can be a persistent problem
• Syringomyelia, which is the formation of a cyst in the spinal cord that can destroy spinal cord tissue when it grows and expands
• Heart-lung failure
• Trouble with concentration
• Severe loss of memory
• Brain atrophy, causing severe dysfunction of certain regions of the brain, and mental decline
• Dementia including personality changes causing a reduced quality of life
• Peritumoral brain edema (PTBE): Presence of fluid around the tumor region in the brain resulting in severe symptoms and complications
• Intracranial hemorrhage or bleeding in the brain
• Congenital tumors, those that are present at birth, may cause developmental delays in the child
• Malignant tumors may spread (metastasize) to other sites of the body leading to severe complications and treatment challenges
• Complications due to an underlying genetic disorder, if any present

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

Surgery:

• 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 Meningeal Melanomatosis 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 internists, pediatricians, neurologists, oncologists, surgeons, radiation therapy experts, and other healthcare professionals are involved in managing the condition.

Currently, there are no definitive treatments available for Meningeal Melanomatosis. 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
• In some individuals, the tumor is partially removed, so as not to cause brain damage, resulting in unwanted additional symptoms. A partial removal is usually considered in slow-growing grade I benign tumors
• 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
• Osteoplastic laminotomy: It is a minimally-invasive procedure to treat spinal cord tumors. The technique also helps minimize post-operative complications and provides increased spinal stability. If necessary, osteoplastic laminotomy may be followed up by a spinal column reconstruction procedure
• Laminectomy: An invasive procedure that is performed to relieve compression on the spinal cord or spinal nerves, caused by the tumors, by widening the spinal canal through removal of the laminae (part of the vertebra)
• In some cases, a debulking surgery, to remove “as much tumor as is feasible” may be considered by the healthcare expert following a detailed assessment
• 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/spinal tissue, swelling of spinal cord, 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 (spine) 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.

Chemotherapy: The response of the tumor to chemotherapy is an important consideration while using this treatment modality. Chemotherapy may not be recommended as a treatment option for treating some tumors.

• Whether to use chemotherapy as a treatment modality is determined by the healthcare provider on a 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 Meningeal Melanomatosis be Prevented?

Presently, there are no specific methods or guidelines to prevent the formation of Meningeal Melanomatosis. 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

According to the U.S. Preventive Services Task Force (USPSTF), currently there is no standard testing protocols available for brain tumors.

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 Meningeal Melanomatosis? (Outcomes/Resolutions)

The prognosis of Meningeal Melanomatosis may vary considerably from one individual to another and is dependent on a set of factors.

• In many cases, the prognosis is reportedly poor. But, the course of the tumor may be varied, and some are rarely known to respond well to treatment and portray better outcomes
• Factors that adversely affect the overall outcomes are aggressive tumors affecting the brain (as against the spine), metastasis, coexistence of neurocutaneous syndromes, and tumor recurrences
• Tumors that are highly malignant may show, on biopsy studies, a high degree of cellular pleomorphism, tumor necrosis, high mitotic rate (cell division), and hemorrhaging

In general, the prognosis may depend upon several factors, which 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 Meningeal Melanomatosis:

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

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