Granular Cell Tumor of the Sellar Region

Granular Cell Tumor of the Sellar Region

Article
Brain & Nerve
Diseases & Conditions
+3
Contributed byKrish Tangella MD, MBADec 01, 2020

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

  • Granular Cell Tumor of the Pituitary Region
  • Pituitary Choristoma
  • Sellar Region Granular Cell Tumor

What is Granular Cell Tumor of the Sellar Region? (Definition/Background Information)

  • A Granular Cell Tumor of the Sellar Region is a type of brain tumor. 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
  • Granular Cell Tumor of the Sellar Region is a slow-growing benign tumor that arises from the sellar and suprasellar region, at the base of the brain. It is type of a brain tumor that is mostly found in adults over the age of 40 years. It is a poorly-understood and difficult to diagnose tumor only via radiological imaging studies
  • A granular cell tumor (GCT) is a common benign tumor of the soft tissue that can occur anywhere in the body. Over 70% of GCTs involve the head and neck region. In a majority of the cases, the tumor is present as a single nodule; though rarely, the tumor can occur as multiple nodules too. GCT of the Sellar Region is a low-grade tumor, meaning that it is neither aggressive nor it spreads to other regions of the body
  • Per WHO, the tumor is part of a group designated as “tumors of the sellar region”. The sellar region includes the sellar turcica and the pituitary gland. It is located just below the center of the brain and bounded by other vital structures such as the hypothalamus, optic nerve, internal carotid artery, and the brainstem. The pituitary gland is the “master gland” of the body that regulates all the other endocrine glands
  • The cause of formation of Granular Cell Tumor of the Sellar Region is not well-established. It is reported that a combination of several factors may play a role in their formation, including genetic, environmental, and lifestyle-related. In general, the risk factors for brain tumors may include presence of certain genetic disorders, exposure to ionized radiation, viral infections, and head trauma
  • Granular Cell Tumor of the Sellar Region is characterized by slow tumor growth during the initial periods. The early signs and symptoms include visual impairments, headaches, nausea and vomiting, and pituitary gland dysfunction, which has the potential to disrupt major endocrinological functions in the body. Severe complications may develop during/from treatment too
  • The treatment for Granular Cell Tumor of the Sellar Region is usually challenging but may primarily involve surgery and radiation therapy, as necessary. Surgery is often challenging due to tumor infiltration into the pituitary gland, its proximity to adjacent critical structures (such as the optic pathway and hypothalamus), and high tumor vascularity. Hormonal therapy is an important component of treatment, depending on the specific combination of signs and symptoms noted in each individual
  • The prognosis for individuals with Granular Cell Tumor of the Sellar Region depend on a wide variety of factors, including the size of the tumor and overall health status of the individual. In a majority, the overall prognosis is unpredictable due to incomplete tumor removals, post-surgical complications, including consequent endocrine system abnormalities and tumor recurrences

Who gets Granular Cell Tumor of the Sellar Region? (Age and Sex Distribution)

  • Granular Cell Tumors of the Sellar Region are extremely rare tumors that may affect individuals of all age groups. Only about 80-100 cases have been described in the medical literature
  • The tumor is predominantly diagnosed in middle-aged and older adults; the mean age of presentation is 49 years
  • Both males and females are affected, but a slight female predominance is observed; over 65% of the tumors are observed in women
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Granular Cell Tumor of the Sellar Region? (Predisposing Factors)

No clearly identified risk factors are known for Granular Cell Tumors of the Sellar Region. The tumor is found with higher frequency in middle-aged and older adults, when compared to children.

In general, 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 types 1 or 2 (NF1 or 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 Granular Cell Tumor of the Sellar Region? (Etiology)

The exact cause of development of Granular Cell Tumor of the Sellar Region is not well-understood. Research scientists believe that the cause may involve a combination of several factors including genetic, environmental, and occupational factors.

  • The tumors arise from the modified gliocyte of ependymal cell lineage (pituicytes). These specialized cells are located in the neurohypophysis and pituitary stalk of the sellar region
  • Some researchers note that a granular cell tumor (GCT) may be a tumor of neural origin (i.e., related to a nerve)
  • Molecular studies on tumor specimens regarding genetic changes are currently limited. However, it is reported that a GCT is not associated with any known congenital syndromes

In general, it is known that tumors 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 Granular Cell Tumor of the Sellar Region?

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 Granular Cell Tumors of the Sellar Region primarily depend on the exact location and size of the tumor. Most GCTs are suprasellar, while some are observed in the intrasellar region. The tumors may arise sometimes in the posterior pituitary region and the infundibulum. Small-sized tumors may be confined to the pituitary stalk. It is reported that pituitary gland involvement/infiltration is observed in nearly 75% of the cases.

A vast majority of tumors are slow-growing and asymptomatic. Often, they may be present for several years before any significant signs and symptoms are observed. The signs and symptoms may also progress slowly over few years, gradually increasing in severity.

The commonly observed set of signs and symptoms may include:

  • Visual disturbances, such as partial blindness and loss of visual acuity, in about 90% of the individuals; this is usually the initial presentation of GCT of the Sellar Region
  • Chronic or persistent headaches in 50% of the individuals
  • Unexplained weight fluctuations; weight loss
  • Amenorrhea in women (absence of menstruation)
  • Fatigue
  • Loss of memory
  • Erectile dysfunction in some men
  • Pituitary gland dysfunction causing reduced production of hormones such as adrenocorticotropic hormone (ACTH), gonadotropin, growth hormone (GH), and thyroid-stimulating hormone (TSH)
  • Low blood pressure from suddenly standing up (orthostatic hypotension)
  • Hypopituitarism (lowered hormone secretion by the pituitary gland) resulting in abdomen pain, decrease in appetite, excess thirst and excess urination, fatigue, nausea and vomiting, anemia, etc.
  • Hyperprolactinemia or increased production of the prolactin hormone, causing menstrual irregularities, breast pain, lack of sexual drive, milky nipple discharge in women (galactorrhea), etc. is observed in some cases

Abnormalities in the endocrine system is observed in 50% of the cases. However, diabetes insipidus presenting increased thirst and frequency of urination is usually not observed.

In some cases, the following may be observed:

  • Seizures
  • Numbness and tingling sensation
  • Muscle weakness; loss of strength in the arms of legs
  • Neck pain
  • Hearing impairment or hearing loss
  • Speech problems
  • Insomnia or loss of sleep, or excessive sleepiness (usually during daytime)
  • Tremors
  • Increased intracranial pressure may be observed if there is obstruction of the cerebrospinal fluid (CSF) drainage, when the ventricular system is affected. This may result in nausea, vomiting, headaches, and seizures
  • Lack of coordination; unsteadiness and loss of balance (vertigo)
  • Dizziness and fainting
  • Confusion
  • Changes in one’s behavior; personality changes
  • Mental impairment

Large tumors can cause significant signs and symptoms. Most of the cases represent single tumors; 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 Granular Cell Tumor of the Sellar Region Diagnosed?

A majority of brain tumors may remain undiagnosed for prolonged periods, because they are asymptomatic and generally slow-growing. The slow development of symptoms may contribute to a delayed detection and diagnosis of these tumors. The tumors are detected when there is a sudden worsening of symptoms prompting the healthcare provider to perform radiological studies of the brain.

In a vast majority of cases, it is difficult to recognize a Granular Cell Tumor of the Sellar Region; the tumor may also be misdiagnosed as a pituitary adenoma. Generally, it is not possible to establish a pre-operative diagnosis of the tumor using imaging studies alone; a tumor biopsy following surgical tumor removal is necessary to make a definitive diagnosis.

The diagnosis of GCT of the Sellar Region may involve the following tests and examinations:

  • Complete physical examination and a thorough medical history
  • Assessment of the presenting signs and symptoms
  • Blood tests to assess hormonal imbalances and serum electrolytes
  • 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)
  • Electroencephalography (EEG)
  • Electromyography with nerve conductivity tests
  • Imaging studies that may be performed include:
    • X-ray of the head and neck region
    • Computerized tomography (CT) scan of the head and neck region; CT scan with contrast agent
    • Magnetic resonance imaging (MRI) scan of the central nervous system; 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 to identify genetic mutations, if necessary

Tissue biopsy: A biopsy of the affected region (brain or spinal cord) 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. 

Radiological differential diagnosis may include:

  • Chordoma
  • Craniopharyngioma
  • Meningioma
  • Metastatic tumor to the pituitary gland
  • Optic pathway glioma
  • Pilocytic astrocytoma of the neurohypophysis
  • Pituicytoma
  • Pituitary infiltration
  • Pituitary macroadenoma
  • Intracranial teratoma

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 Granular Cell Tumor of the Sellar Region?

The possible complications associated with Granular Cell Tumor of the Sellar Region include:

  • Emotional and mental stress for both the patient and the caretakers, due to the diagnosis and during treatment of a brain tumor
  • Hydrocephalus: When the tumor obstructs the free flow of cerebrospinal fluid in the brain, it can cause a condition known as hydrocephalus. This can result in lethargy, irritability, nausea and vomiting, loss of balance, seizures, urinary incontinence, and growth delays, among other symptoms
  • Large undetected tumors can severely affect brain function and be disabling or even life-threatening, due to mass effect/compression of surrounding tissue
  • In the brain, large tumors may also press against the skull (or other) bones causing it to expand, causing severe complications
  • Dysfunction of the hypothalamus can also present sleep disorders, abnormal heart rate and blood pressure, and body temperature variations
  • Bilateral optic atrophy
  • Delayed puberty in children
  • In very rare cases acromegaly, caused by increased secretion of growth hormone by the tumor leads to an increased length of the limbs and enlargement of the face
  • Infertility in men and women
  • Trouble with concentration
  • Severe loss of memory
  • 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
  • Malignant transformation of GCTs: These are known to take place particularly when the tumors are present on the arms and legs. However, malignant transformation of GCT of the Sellar Region is not reported
  • Complications due to an underlying genetic disorder, if any present

Complications may arise from surgery, radiation therapy, 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
  • Intra-operative hemorrhaging (severe bleeding) is a grave threat in GCT of the Sellar Region during its surgical removal, due to the highly vascular nature of the tumor
  • Post-surgical infection at the wound site is a potential complication
  • In many individuals, the symptoms are known to significantly worsen following surgical removal of the tumor
  • Local recurrence of the tumor after surgery is observed in many due to incomplete tumor resections; recurrences often necessitate multiple surgeries that increases the treatment burden

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 Granular Cell Tumor of the Sellar Region Treated?

The treatment modality for brain tumor is chosen, depending on the 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, endocrinologists, neurologists, oncologists, surgeons, radiation therapy experts, and other healthcare professionals are involved in managing the condition.

The treatment of Granular Cell Tumor of the Sellar Region is often difficult and challenging. 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. An important consideration is the anatomical location of this deep-seated tumor. In many cases, the involvement of nearby vital brain structures that include the pituitary gland, hypothalamus, and optic nerve pathway, is noted. Due to this, it is often difficult to completely resect the tumor. Also, large tumor sizes and the highly vascular (rich in blood and lymph vessels) nature of GCT prevent its complete excision.

The treatment measures for GCT of the Sellar Region may include:

  • Symptomatic treatment may involve the use of antiseizure medications, painkillers, steroids (to reduce inflammation), and administration of anti-depressants
  • Hormonal disturbances caused by pituitary dysfunction are treated using hormonal supplements that may include hydrocortisone, testosterone, thyroxine, etc.
  • 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
  • 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. Tumor embolization is a very important consideration for GCT of the Sellar Region, since these are highly vascular tumors
  • 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. In the case of granular cell tumor, the role of radiation therapy as an adjuvant treatment option is reportedly limited and recommended only on a case-by-case basis. Such indications may include recurrent tumors that are inoperable, tumors that show progression with worsening symptoms, and GCTs showing anaplastic features.

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. Chemotherapy may not be recommended as a treatment option for treating some tumors.

  • Chemotherapy may be beneficial in individuals diagnosed with higher grade tumors. 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 advanced stages of tumor progression, 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 Granular Cell Tumor of the Sellar Region be Prevented?

Presently, there are no specific methods or guidelines to prevent the formation of Granular Cell Tumor of the Sellar Region. 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, in order to 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

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

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 Granular Cell Tumor of the Sellar Region? (Outcomes/Resolutions)

The prognosis of Granular Cell Tumor of the Sellar Region may vary considerably from one individual to another and is dependent on a set of factors.

  • Even though GCT of the Sellar Region is a benign and slow-growing tumor with no aggressive tendencies, the outcomes can be unpredictable
  • In general, the prognosis can be good; but, this is highly dependent on the feasibility of complete tumor resection. Since, it is difficult to achieve a complete resection, often multiple surgeries are necessitated due to tumor regrowth
  • A complete surgical removal of the tumor can greatly increase the risk for post-surgical complications, such as endocrine system dysfunction, including severe bleeding, and irreversible damage to the pituitary gland and other vital brain structures. Hence, the prognosis may be unfavorable in individuals with significant signs and symptoms.

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
  • 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
  • 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
  • Functional neurologic status based on neurological evaluation of the patient: A higher score on the Karnofsky Performance Scale may indicate a more favorable outcome

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 Granular Cell Tumor of the Sellar Region:

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

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

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Krish Tangella MD, MBA

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