On October 26th, 2007, Joey was seen by an Endocrinologist. Although the doctor advised that we not panic yet,
he did admit Joey's brain scan displayed a "pituitary adenoma" or benign tumor.
However, it's currently unknown if the tumor is the cause for his seizures or an unrelated condition,
so treatment (surgery? medication? watchful waiting?) can't be decided.
When my ex-husband asked about symptoms to watch for, the doctor gave several examples.
One symptom of a pituitary adenoma is
gigantism or acromegaly,
as experienced by the actors who played Jaws in James Bond films and Karl the Giant in the film Big Fish.
This condition is also defined by exaggerated growth in the forehead, jaw, chest, hands, and feet.
Another symptom is declining peripheral vision and increasing blindness caused by the pressure on the optic nerve.
Yet another symptom is darkening of the skin.
Although Joey hasn't exhibited any of these symptoms, the Endocrinologist told us that changes are so gradual
they often go unnoticed until it's too late. Wisely, the doctor refrained from divulging further symptoms,
so as to "not give Joey nightmares."
The specialist wrote a script for more blood tests and asked to see Joey again in four months.
His appointment is scheduled for March 2008.
As we await the results of the blood tests, and most recent E.E.G. and brain scan, I once again turn to the Internet,
and am distressed to learn that pituitary adenomas may cause headaches and epilepsy, two symptoms Joey
has exhibited:
Non Functioning Tumors.
After reading that surgery is less invasive and damages fewer surrounding tissues when the tumor is small,
I wonder why our specialist decides to "wait and see" if the tumor becomes "functional" or begins to grow. Why not take
care of it now, before it does any more damage?
For another opinion, I speak with my cousin,
Dr. Carlos A. Rosende, the Chair of the Department of Ophthalmology at the
University of Texas Health Science Center in San Antonio. Although he hasn't seen Joey's records nor examined him,
my cousin concurs with our specialist's conservative approach. There is always a risk to any surgery, he explains, and
treating the tumor with surgery or radiation may damage surrounding brain tissue.
He agrees with our doctor's advice to carefully monitor the tumor at this point, but suggests
we take Joey for an eye exam to check his peripheral vision.
I call another cousin, Dr. Jose Bengochea, a pediatrician on the board of Miami's Children's Hospital, but am unable to get through to him
at the busy practice he shares with his wife, Dr. Eysa Marquez-Bengochea, and I'm not available when his nurse returns my call. I need to learn more about Joey's condition.
I need to make sure we're doing everything we can for him.
At Joey's appointment this week, I will address my cousin's advice about getting his vision tested. I will continue
to speak to doctors and specialists, and to go to online forums and support groups, and to conduct research on my own.
Hopefully, there is no reason to worry, and Joey's pituitary adenoma will not require treatment other than monitoring.
But, I will not be anything less than a partner in ensuring Joey gets the best medical care.
Brain tumors are scary. Whether they're non-functioning or functioning, malignant or benign,
they're not to be taken lightly.
From my research, I know this much: There are two main types of brain tumors: primary brain tumors that originate in
the brain, and metastatic brain tumors that originate from cancer cells that have come from other parts of the body.
Primary tumors can be either benign
(noncancerous) or malignant(cancerous).
Malignant tumors in the brain are life-threatening, because they are aggressive and invasive in nature. Slow-growing benign
tumors in the brain also can have life-threatening consequences, depending on the type of tumor and its location.
This is because the skull is closed, and when a brain tumor takes up space, it can compress vital tissues and structures
and cause serious neurological problems.
Some brain tumors are easier to remove and treat than others. Brain tumors are classified according to the type of cell
that makes up the tumor, or the cell type from which the tumor originated. Gliomas are tumors that are made up of glial cells
(cells that provide important structural support for the nerve cells in the brain). An astrocyte is one type of glial cell,
and the tumors that grow out of astrocytes are called astrocytomas.
Meningiomas are tumors of the meninges, the membranes covering the brain.
Pituitary adenomas (Joey's diagnosis) are tumors that start in the anterior pituitary
(gland at the base of the brain that secretes hormones important for growth and reproduction).
Lymphomas are tumors made up of lymph cells (lymph is a fluid that flows through all the tissues of the human body
and plays an important role in cleaning out bacteria and other foreign matter).
Metastic tumors are brain tumors that have originated elsewhere in body and have metastasized to the brain.
In addition to being classified on the basis of cell type, malignant tumors are typically assigned a tumor grade,
based on what the cancer cells look like under a microscope.
Primary Brain Tumors Gliomas
Gliomas are tumors that are made up of glial cells, cells that play an important structural role in the brain.
There are several types of gliomas and the two most common are astrocytomas, cancerous tumors, and oligodendrogliomas -
rare, usually benign tumors. A particularly malignant type of astrocytoma is known as a glioblastoma multiforme,
which can be either low grade (slow-growing and not very aggressive) or high grade (fast-growing and very aggressive).
Astrocytomas
About 25% of all brain tumors are astrocytomas, malignant tumors that originate in cells called astrocytes.
Astrocytomas are graded and named based on what the cells look like under the microscope.
Low-grade astrocytomas are made up of the least aggressive cancer cells, anaplastic astrocytomas are made up of more
aggressive cancer cells, and a glioblastoma multiforme is a type of astrocytoma that is made up of extremely aggressive cancer
cells. Glioblastoma multiforme is the most common adult primary tumor.
Astrocytomas usually develop between the ages of 20 to 50, but they can occur at any age.
One of the most common early symptoms is seizures. Although they can develop anywhere in the brain,
they usually develop in the temporal or frontal lobes of the brain and then spread into the adjacent tissues.
Glioblastoma Multiforme
Glioblastoma multiforme, a type of malignant astrocytoma, are the most common adult primary brain tumors.
They occur slightly more frequently in men. Patients with these types of tumors are usually over the age of 50,
but the tumors can develop at any age. Patients who develop the tumor at a younger age have a significantly better survival
rate than older patients. Symptoms are caused by rapid tumor growth, infiltration into adjacent tissues, swelling (edema)
and an increased accumulation of cerebrospinal fluid in the brain (hydrocephalus). Most patients experience headaches, seizures,
or a change in their mental status.
Oligodendrogliomas
Less than 5% of all brain tumors are oligodendrogliomas, tumors that originate in cells called oligodendrocytes.
The average age of patients with these kinds of tumors is arbout 40 years old. Oligodendrogliomas are very slow-growing,
usually benign tumors (less than 10% are malignant), and they occur most often in the frontal lobes. The first symptom for
many patients is a seizure.
There are two types of oligodendrogliomas: low-grade oligodendrogliomas are made up of less aggressive cells,
and anaplastic oligodendrogliomas are made up of more aggressive cells. More common than either low-grade
or anaplastic oligodendrogliomas, however, are tumors made up of a mix of oligodendrioglioma and astrocytoma.
These mixed-cell tumors are called oligoastrocytomas.
Ependymoma
Less than 5% of all brain tumors are ependymomas, which usually occur in the lining of the ventricles,
the structures in the brain that contain the cerebrospinal fluid. They also occur in the lining of the middle part
of the spinal cord. One of the earliest symptoms is hydrocephalus, an increased accumulation of cerebrospinal fluid that
causes swelling and neurological dysfunction.
Pituitary Adenomas
The pituitary gland is a small structure at the base of the brain that produces hormones necessary for normal growth
and metabolism. Tumors in the pituitary gland are called pituitary adenomas, benign tumors that account for about 10% to 20%
of all brain tumors.
There are two types of pituitary adenomas: secreting and nonsecreting. A patient with a secreting pituitary adenoma
has abnormally high levels of pituitary hormones circulating through their body, which in turn causes a range of symptoms
from impotence (erectile dysfunction) to amenorrhea (the abnormal ending of menstruation). For example, one of the more
common types of pituitary adenomas produces and secretes excess prolactin, a substance responsible for triggering milk
production when a woman is nursing.
In addition, because the pituitary gland is located near important visual pathways, a patient with a pituitary adenoma
may experience vision loss.
Meningiomas
The meninges is the thin outer covering that lines the spinal cord and brain. It is made up of three layers:
the dura matter (external), the arachnoid (middle) and the pia matter (internal). Tumors that originate in the meninges
are called meningiomas.
About 15% to 20% of all brain tumors are benign, slow-growing meningiomas. Even though they are benign,
they can still cause severe neurological dysfunction. Patients often suffer seizures, headache, weakness, and visual problems.
They occur more commonly in women and after the age of 40. Patients with neurofibromatosis, a genetic disorder
that predisposes to certain types of tumors, are at a greater risk for developing meningioma.
Patients who have had previous brain radiation are also at a greater risk for developing meningioma.
Many patients with meningiomas seem to have a genetic defect on chromosome number 22.
Nerve Sheath Tumors (Schwannomas)
Nerve sheath tumors, also known as schwannomas, are tumors that originate in the Schwann cells that make up
the protective sheath that surrounds the nerve fibers. Schwannomas are usually benign and slow-growing.
One of the most common types is known as a vestibular schwannoma, or acoustic neuroma. Another common schwannoma causes
facial paralysis.
Acoustic Neuromas
Acoustic neuromas are schwannomas that involve the eighth cranial nerve.
There are a total of 12 pairs of cranial nerves that originate in the brainstem (the bottom part of the brain that
connects to the spinal cord) and lead to various parts of the face and neck. The eighth cranial nerve is responsible for
balance and hearing. Acoustic neuromas cause early hearing loss in the ear on the side of the tumor, tinnitus (ringing in
the ears), vertigo (dizziness), balance problems, and facial weakness. These tumors occur most commonly in people
between 30 and 50 years old but can occur anytime.
Metastatic Brain Tumors
Cancerous tumors that spread to the brain from other parts of the body, such as the lung, are said to be metastatic.
Tumor cells spread to the brain through the bloodstream. In more than half of all metastatic brain cancer,
the tumors are found throughout the brain and are not localized to one particular spot, making them extremely
difficult to treat. Between 20% to 40% of all cancer patients develop metastases to the brain. In adults, the most common
types of cancer to spread to the brain are lung, breast, gastrointestinal, and urinary/genital tract cancer,
as well as malignant melanoma.
The main symptoms of metastatic brain tumors include seizures, headaches, weakness and confusion.
In general, the prognosis for patients who develop brain metastases is poor.
Spinal Cord Tumors
Primary spinal cord tumors are very rare and usually benign.
Only about 1 in 200 newly diagnosed tumors are spinal cord tumors.
Of these, less than 3% are malignant, or cancerous. Spinal cord tumors are made up of the same types
of cells that make up brain tumors. For example, they include meningiomas and gliomas. Most malignant spinal cord tumors,
again of which there are very few, are tumors that originate from cancer cells that come from other parts of the body.
The spinal cord has only a limited amount of space inside the spinal column (the backbone or spine),
so even a very small tumor can cause enough pressure to become problematic. Though sometimes the tumor grows so slowly that
the spinal column is able to adapt to it and make room for it such that a person suffers very few symptoms.
Very aggressive metastatic spinal cord tumors can cause paralysis very quickly - even within days.
Slow-growing tumors can also cause paralysis if left untreated.
Surgery is the usual course of treatment for primary spinal cord tumors. Metastatic spinal cord tumors
are not always treated surgically, however, especially if there is more than one metastasis.
Often surgery may be used as a way to relieve pain and other symptoms, even though it may not actually involve
removing the tumor.
In the following video, doctors discuss
Pituitary Tumors from a neurosurgeon's perspective.
On
http://www.professionals.epilepsy.com, authors F.T. Mangano, A.E. McBride, and S.J. Schneider,
write that brain tumors are a common cause of Epilepsy in adults. More than one-third of the 35,000 patients
per year with newly diagnosed brain tumors develop epileptic seizures. If the tumor involves the cerebral hemispheres,
seizures occur in at least 50% of cases.
17,18
Some predictive factors for seizure occurrence include:81,83
tumor location in the frontal or parietal regions
evidence of cerebral hemispheric dysfunction
incomplete tumor resection
Any brain tumor, benign
or malignant, common or uncommon, can cause seizures.19–23
Those more highly associated with the development of Epilepsy include:83,88
melanoma
hemorrhagic lesions
multiple metastases
slowly growing primary tumors
tumors near the Rolandic fissure
Patients with low-grade tumors may be more likely to develop Epilepsy, possibly because their longer survival
allows more time for seizures to develop.
81 One retrospective study found a median interval of 8 weeks between diagnosis of a brain tumor and a first seizure.83
The tumors most often presenting with seizures in adults are:24–30
In children, Epilepsy is associated with brain tumors less often than in adults. Tumors
still must be ruled out, however, even if the child has no neurologic deficits.32–34 If a tumor is diagnosed,
up to 46% of these patients may have
intractable seizures.
32,35,36 Most tumors occur in the temporal or frontal lobes. As in adults, epileptogenic brain tumors in children may be benign or malignant.
The most common tumors associated with Epilepsy in children are:30,36–38
gangliogliomas
low-grade astrocytomas
DNETs
oligodendrogliomas
(From: Mangano FT, McBride AE, and Schneider SJ. Brain tumors and Epilepsy.
In: Ettinger AB and Devinsky O, eds. Managing Epilepsy and co-existing disorders. Boston: Butterworth-Heinemann;
2002;175–194.) With permission from Elsevier (www.elsevier.com).
The Pituitary Gland and Pituitary Tumors
The pituitary is a small gland attached to the base of the brain (behind the nose) in an area called the pituitary
fossa or sella turcica. The pituitary is often called the "master gland" because it controls the secretion
of hormones. A normal pituitary gland weighs less than one gram, and is about the size and shape of a kidney bean.
The function of the pituitary can be compared to a household thermostat. The thermostat constantly
measures the temperature in the house and sends signals to the heater to turn it on or off to maintain
a steady, comfortable temperature. The pituitary gland constantly monitors body functions and sends signals
to remote organs and glands to control their function and maintain the appropriate environment. The ideal
"thermostat" setting depends on many factors such as level of activity, gender, body composition, etc.
The pituitary is responsible for controlling and coordinating the following:
Growth and development
The function of various body organs (i.e. kidneys, breasts and uterus)
The function of other glands (i.e. thyroid, gonads, and adrenal glands
Pituitary Anatomy and Functions
The pituitary gland has two distinct parts: the anterior pituitary is closest to the front of the head,
while the posterior pituitary is closest to the back of the head. These parts each contain unique cells
and release different hormones that are responsible for specific control duties. The anterior pituitary
is formed from the same tissue as the pharynx (the upper part of the mouth). The posterior pituitary develops
from the bottom portion of the brain, and is actually an extension of the hypothalamus. The pituitary gland itself,
is connected to and controlled by the hypothalamus, a region of the brain located right above the pituitary.
The hypothalamus and pituitary together comprise the neuroendocrine system.
The anterior pituitary accounts for about 80 percent of the pituitary gland, and is composed
of the anterior lobe and the intermediate zone. The anterior lobe is responsible for the majority
of the signaling hormones released into the blood stream.
The posterior pituitary develops very early in life and does not produce any hormones of its own. It does
contain the nerve endings of brain cells (neurons) that arise from the hypothalamus. These neurons produce
the hormones vasopressin and oxytocin, which are transported down the pituitary stalk into the posterior pituitary.
They are stored for later release into the bloodstream.
The pituitary and hypothalamus work together to regulate the daily functions of the body, as well as play
an essential role in growth, development and reproduction. The hypothalamus secretes hormones that control
secretion of hormones from the anterior pituitary. The hypothalamic hormones are known as releasing hormones
and inhibiting hormones, because of their influence on the anterior pituitary hormones.
The pituitary gland performs its key functions by releasing several signaling hormones that consequently control
the activities of other organs. The pituitary produces the following hormones:
Adrenocorticotropic Hormone (ACTH) ACTH triggers the adrenals to release hormones such as cortisol and aldosterone.
These hormones regulate carbohydrate/protein metabolism and water/sodium balance, respectively.
Adrenocorticotropic Hormone (ACTH) - ACTH triggers the adrenals to release hormones such as cortisol
and aldosterone. These hormones regulate carbohydrate/protein metabolism and water/sodium balance, respectively.
Growth Hormone (GH) - This is the principal hormone that regulates metabolism and growth.
Luteinizing Hormone (LH) and Follicle Stimulating Hormone (FSH) - These hormones control the production
of sex hormones (estrogen and testosterone).
Melanocyte-Stimulating Hormone (MSH) - MSH regulates the production of melanin, a dark pigment, through
melanocytes in the skin. Increased melanin production produces pigmentation or tanning of the skin. Some conditions
causing excessive production of melanocyte-stimulating hormone may lead to darkening of the skin.
Prolactin (PRL) - This hormone stimulates secretion of breast milk.
Thyroid Stimulating Hormone (TSH) - TSH stimulates the thyroid gland to release thyroid hormones. Thyroid
hormones control basal metabolic rate and play an important role in growth and maturation. Thyroid hormones affect
almost every organ in the body.
Vasopressin, also called anti-diuretic hormone (ADH) - This hormone promotes water retention.
Pituitary Adenomas
Pituitary adenomas are the fourth most common intracranial tumor after gliomas, meningiomas and schwannomas.
The large majority of pituitary adenomas are benign (not malignant) and are fairly slow growing. Even malignant
pituitary tumors rarely spread to other parts of the body. Adenomas are by far the most common disease affecting
the pituitary. They more commonly affect people in their 30s or 40s, although they are diagnosed in children as well.
Most of these tumors can be successfully treated. Pituitary tumors can vary in size and behavior. Tumors that produce
hormones are called functioning tumors, while those that do not produce hormones are called nonfunctioning tumors.
Symptoms
Tumors smaller than 10 mm are called "microadenomas" and often secrete anterior pituitary hormones.
These smaller, functional adenomas are usually detected earlier because the increased levels of hormones cause
abnormal changes in the body. Approximately 50 percent of pituitary adenomas are diagnosed when they are smaller
than 5 mm in size. Adenomas larger than 10 mm (the size of a dime) are called "macroadenomas," and
usually do not secrete hormones. These tumors are often discovered as they produce symptoms by compressing
nearby brain or cranial nerve structures.
The symptoms of a pituitary tumor generally result from endocrine dysfunction. For example, this dysfunction
can cause overproduction of growth hormones, as in acromegaly (giantism), or underproduction of growth hormones,
as in hypothyroidism. Hormonal imbalances can impact fertility, menstrual periods, heat and cold tolerance,
as well as affect the skin and body in other ways.
Because of the pituitary gland’s strategic location within the skull, tumors of the pituitary can compress
important brain structures as they enlarge. The most common circumstance involves compression of the optic nerves,
leading to a gradual loss of vision. This vision loss usually begins with a deterioration of lateral peripheral
vision on both sides.
The presence of three or more of the following symptoms may indicate a pituitary tumor:
Vision problems (blurred or double vision, drooping eyelid)
Headaches in the forehead area
Nausea or vomiting
Impaired sense of smell
Sexual dysfunction
Depression
Fatigue
Infertility
Growth problems
Osteoporosis
Unexplained weight gain
Unexplained weight loss
Easy bruising
Aching joints
Carpel tunnel syndrome
Disrupted menses
Early menopause
Muscle weakness
Galactorrhea (Spontaneous breast milk flow not associated with childbirth or the nursing of an infant)
Diagnosis
When a pituitary tumor is suspected, a physician will perform a physical examination, as well as
vision testing to detect visual field deficits, such as loss of peripheral vision. Hormone testing of
the blood and urine and imaging studies of the brain are used to confirm diagnosis. The most accurate
diagnostic imaging test is magnetic resonance imaging (MRI), performed with and without a contrast agent.
Treatment
Early intervention provides the best chance for cure or control of the tumor and its side effects.
There are three types of treatment used for pituitary tumors: surgical removal of the tumor, radiation
therapy using high-dose x-rays/proton beams to kill tumor cells, and medication therapy to shrink or eradicate the tumor.
Surgery
The transsphenoidal approach involves making an incision in the upper gum line or nasal cavity
and accessing the tumor through the base of the skull. This approach is usually the procedure of choice
because it is less invasive, has fewer side effects, and patients generally recover more quickly.
Patients can often leave the hospital as early as two to four days after surgery.
The transcranial approach through the upper part of the skull is used for larger tumors that cannot be
safely removed through the transsphenoidal approach.
Endoscopy is a newer, minimally invasive approach which allows neurosurgeons to utilize a tiny endoscope with a camera
on the end. A tiny endoscope inserted through the nostril is placed in front of the tumor in the sphenoid sinus, and the
tumor is removed with specially designed surgical tools. Postoperative discomfort is usually minimal. Endoscopic brain
surgery is another surgical option for removing pituitary adenomas, but can only be utilized in certain cases.
Radiation Therapy
Radiation therapy uses high-energy x-rays to kill cancer cells and abnormal pituitary cells and shrink tumors. Radiation therapy may be an option if the tumor cannot be treated effectively through medication or surgery.
Standard External Beam Radiotherapy uses a radiation source that is nonselective
and radiates all cells in the path of the beam. The radiation path beam may damage other portions
of the brain in the general area of the pituitary gland.
Proton Beam Treatment employs a specific type of radiation in which "protons", a form of radioactivity,
are directed specifically to the pituitary gland. The advantage is that less tissue surrounding the pituitary gland
incurs damage.
Stereotactic Radiosurgery (like Gamma Knife, and Cyberknife) combines standard external beam radiotherapy
with a technique that focuses the radiation through many different ports. This treatment tends to incur less damage
to tissues adjacent to the pituitary gland.
Medication Therapy
Prolactinomas are the most common secreting pituitary adenoma seen clinically. In general, medical therapy
is the first course of treatment in patients with a prolactinoma. About 80 percent of patients have prolactin
levels restored to normal through dopamine agonist therapy. The most commonly used agents are bromocriptine
or cabergoline. The size of the tumor will be reduced in the majority of patients to varying degrees, often
resulting in improved vision, resolution of headaches, and restored menses and fertility in women. Bromocriptine
has side effects, so is prescribed in gradual doses.
Cabergoline, an oral long-acting dopamine agonist, was recently approved by the U.S. Food and Drug Administration
for hyperprolactinemia. The advantage is that it can be taken twice a week and generally has fewer side effects than
bromocriptine. It has also been shown to be effective in patients whose prolactinomas are resistant to bromocriptine therapy.
In patients with microadenomas, dopamine agonist therapy is usually attempted first for a period of several months.
If the tumors do not respond to medication therapy, then surgery is considered, and in general, the recommendation is
that it is done within six months of the start of the medication therapy.
GH secreting adenomas may be treated medically by using somatostatin analog therapy. Somatostatin is a hormone
that inhibits secretion of growth.