First, what is a Tumor?
Then what is a Benign Brain Tumor?Benign brain tumors are usually defined as a group of similar cells that do not follow normal cell division and growth patterns and develop into a mass of cells that microscopically do not have the characteristic appearance of a cancer. Most benign brain tumors are found by CT or MRI brain scans. Benign brain tumors usually have clearly defined borders and usually are not deeply rooted in brain tissue. This makes them easier to surgically remove, assuming they are in an area of the brain that can be safely operated on. But even after they've been removed, they can still come back, although benign tumors are less likely to recur than malignant ones. Although benign tumors in other parts of the body can cause problems, they are not generally considered to be a major health problem or to be life-threatening. But even a benign brain tumor can be a serious health problem. Brain tumors can damage the cells around them by causing inflammation and putting increased pressure on the tissue under and around it as well as inside the skull.
So what causes Brain Tumors?No one knows what causes brain tumors; there are only a few known risk factors that have been established by research. Children who receive radiation to the head have a higher risk of developing a brain tumor as adults, as do people who have certain rare genetic conditions such as neurofibromatosis or Li-Fraumeni syndrome. But those cases represent a fraction of the approximately 28,000 new primary brain tumors diagnosed each year in the United States. Age is also a risk factor. People between the ages of 65 and 79 make up the population most likely to be diagnosed with a brain tumor.
'Benign' Does Not Mean 'Harmless' - Exploding the Myth Behind 'Benign' Brain Tumors
By Emma Flixton for It’s Just Benign, a non-profit organization serving benign brain tumor survivors.
The phrase ‘brain tumor’ brings panic and despair, the word ‘benign’ intense relief. So great is this sense of relief, in fact, that many people appear to equate the ‘benign’ status of a brain tumor with a declaration that the condition is nothing to worry about – or even with a sort of cure. The implication of the word ‘benign’ in many people’s view is that they no longer have to worry about and support the sufferer. This view fails to take into account that the symptoms which drove the sufferer to the doctor in the first place are usually still present, despite the tumor’s ‘benign’ status, and often still causing significant problems. Indeed, once a tumor is classified as benign, friends and family members who have previously supported the sufferer through their affliction may even come to think of the patient as a malingerer, making a mountain out of a molehill, and not in need of support. The prevailing ‘Oh, but it’s benign! You’re fine!’ attitude can be intensely frustrating for people who have to live with these supposedly ‘innocent’ brain tumors. Just because such tumors may not be immediately life-threatening (although some of them do, in fact, present a significant threat to life) does not mean that they are not life-affecting.The Brain Is Important
It goes without saying that the brain is an extremely important part of the body. It is a mass of vital tissues connected through a complex network of chemicals, hormones, nerves, synapses and electrical impulses which ricochet throughout the entire body. We do not yet fully understand the brain or how it works – possibly we never will - but we do understand its importance. The brain controls the functions of the body: speech, movement, breathing, even heartbeat. Within the brain we keep our memories, and the assortment of knowledge, memory, emotion and so on which make up what we think of as our ‘selves’. If personality – or ‘soul’, if you prefer – has a seat within the body, that seat is undoubtedly the brain. It should therefore be obvious that a cluster of rogue cells intruding upon this vital organ is a cause for concern, no matter how malignant or otherwise they may be. The old adage that human beings use only 10% of their brains has been thoroughly disproven. Every part of the brain is vital, so a tumor in any portion of it is bound to have an impact upon the functioning of one’s life. This fact does not alter simply because the tumor is benign. Though it may not be growing as fast as a malignant tumor, or eating away at healthy matter in the same horrific manner, it is nonetheless still there, and still causing problems for the sufferer.A Wide Variety Of Symptoms
The symptoms of a benign brain tumor seem almost limitless, depending largely upon factors like the location and size of the tumor. Sufferers commonly experience crippling headaches due to the pressure the tumor exerts upon the brain. Seizures are another common symptom. Nobody would blithely state that an epileptic was making too much of their condition and should be thankful that it is not worse. However, this is precisely what many benign brain tumor sufferers who have been effectively rendered epileptic by their tumor are told when they seek emotional support. Given the nature of the brain, different tumor locations cause different symptoms. A tumor in the occipital lobe may cause problems with vision. A tumor in the parietal lobe can have effects similar to that of a stroke – numbness in one side of the body, difficulty speaking and coordinating oneself, and so forth. A tumor in the cerebellum can cause sudden, unexpected vomiting, a stiff neck, difficulties with speech and movement, and a flicking of the eyes. A tumor in the brain stem can manifest through problems in swallowing, unsteadiness, and a droopy face. The list goes on and on, and the symptoms are by no means limited to those listed above. Some with ostensibly ‘benign’ tumors are killed by them – a tumor pressing on the brainstem (an area of the brain on which many doctors will not operate) can fatally interfere with breathing. Many with such tumors, if they survive, may spend their lives hooked up to life-support systems. Some have tumors which render them incapable of swallowing, and must be fed through an IV tube. Daily medication for seizures, depression, pain and so on are the order of the day for those with benign tumors, and many find their lives disrupted even more by the fact that they may be unable to drive or, indeed, work due to double vision, seizures, loss of hearing, and a whole plethora of other disabling conditions entirely due to the tumor. Furthermore, such tumors frequently grow back – so even if one undergoes complex and dangerous neurosurgery to have it removed, chances are that even this will not eliminate the problem.Changes In Personality
Perhaps most worrying of all for some are the personality changes which sometimes accompany brain tumors. Given the brain’s role in regulating hormone release, impulse control, and other things which affect actions and emotions it is perhaps not surprising that an obstruction like a tumor should result in some personality change. However, this is often very distressing for the sufferer, who frequently finds themselves unable to connect with their loved ones in the same manner as before. Such changes come with a host of attendant problems, such as depression due to emotional imbalance (or simply due to the agony of feeling that one has lost one’s ‘self’), or loss of inhibition. This latter can lead to all sorts of problematic avenues – such as problems with the law due to poor impulse control, the disruption of family relationships, or even a downward spiral into drugs or alcoholism which greatly increases the amount of help, support, and treatment needed.Recognition And Respect
It is clear, therefore, that ‘benign’ does not necessarily mean ‘harmless’. While benign tumors may not necessarily be directly life-threatening, they can still massively impact the lives of those who suffer from them. It is time to end the notion that a benign tumor is not a problem at all. People afflicted with benign tumors suffer from very real problems. While this argument is by no means intended to denigrate the undoubted suffering of those poor souls with malignant brain tumors, it is perhaps time to also afford those whose tumors are benign a little more recognition and respect for their struggles.
- Changes in speech or hearing
- Changes in vision
- Balance problems
- Problems with walking
- Numbness or tingling in the arms or legs
- Problems with memory
- Personality changes
- Inability to concentrate
- Weakness in one part of the body
- meningioma - tumor arising from the membranes covering the brain and spinal cord; this accounts for about 20% of brain tumors
- schwannoma (also termed acoustic neuroma) - tumor in the 8th cranial nerve arising from Schwann cells (insulating cells of the nervous system); this accounts for about 9% of all brain tumors
- pituitary adenomas - pituitary gland tumor; this accounts for about 8% of brain tumors
- hemangioblastomasa - vascular tissue mass, sometimes cystic; this accounts for about 2% of brain tumors
- craniopharyngioma - a cystic tumor from cell remnants of Rathke's pouch (nasopharynx), usually occurring in children; this accounts for about 1%-3% of brain tumors
- choroid plexus papilloma—choroid plexus tissue (the tissue responsible for the production of cerebrospinal fluid or CSF) mass that blocks cerebrospinal fluid flow, usually in children; this accounts for less than 1% of brain tumors
Five Things You Need to KnowA brain tumor diagnosis can sound like a life-threatening situation. But although the symptoms of most brain tumors are the same, not all tumors are malignant. In fact, meningioma is the most common brain tumor, accounting for about 30 percent of them. According to Johns Hopkins neurosurgeon Kaisorn Chaichana, meningioma tumors are often benign: You may not even need surgery. Here are five key meningioma facts you need to know:
2. Meningioma symptoms depend on their size and location. Dr. Chaichana says, “Meningiomas present with typical brain tumor symptoms such as headaches, vision problems or seizures. A bad headache on its own is seldom a symptom of meningioma or any other brain tumor.” Larger meningiomas can block the flow of cerebrospinal fluid, resulting in hydrocephalus (“water on the brain”) which can affect gait and memory. Other tumor locations can affect your sense of smell, vision, hearing or even the function of your pituitary gland.
3. A meningioma diagnosis may occur when the doctor is looking for something else. Dr. Chaichana says, “Brain tumor diagnosis is often incidental — that is, the doctor discovers a tumor on a CT or MRI while examining the individual for another reason like a head injury or another neurologic problem.” When a doctor diagnoses a meningioma, you will get further tests to find out how the tumor is likely to behave. Based on these data, a neurosurgeon will recommend removing the tumor or just watching it to see if it grows.
4. Most meningiomas do not spread. It can be shocking for someone to be diagnosed with a meningioma — especially a large one — but these tumors are usually benign. This means that the tumor cells are not likely to spread to other parts of the body. That said, meningiomas can quietly grow for years without causing any problems — and they can get surprisingly large.
5. Meningioma treatment: surgery — or not Sometimes, believe it or not, your doctor may recommend observation for meningioma, especially if it’s small and not causing problems. You’ll have regular MRIs to check on it.
Otherwise, the main treatment for meningiomas is surgery to remove it, through a craniotomy or other procedure. Your doctor will go over what the operation will involve, the approach to access the tumor and what you can expect afterward.
How does a neurosurgeon operate on a meningioma? It’s all about location. Dr. Chaichana says, “Depending on where the tumor is, each approach will be different. Tumors close to the surface are typically easier than those located along the skull base.”
Skull base tumors are those located deep in the skull, behind the nose or eyes. These can be challenging, and call for surgeons with skill and expertise in this kind of surgery.
“There are a number of new techniques in brain tumor surgery, even for tumors located deep in the skull, and some of these are less invasive.
“A new system we’re using involves a camera-assisted tube that gently moves brain tissue aside so we can reach the tumor with less cutting, so patients can recover faster,” he says.
After your treatment you’re likely to get back to life as usual, but you will have regular MRIs to ensure the tumor isn’t returning.
In many cases, it won’t. Dr. Chaichana says, “After 10 years, about 90 percent of patients who have had a meningioma have not seen a recurrence if the tumor is removed completely, including the part of the brain lining it originated from.”
Regardless, the best thing to do if you’re diagnosed with a meningioma or any other tumor is to get the facts, stay informed and work with the most experienced neurosurgeon and care team you can find.
BRAIN TUMOR FACTS
- An estimated 700,000 Americans are living with a brain tumor
- 550,042 tumors are benign
- 138,054 tumors are malignant
- An estimated 79,270 people will receive primary brain tumor diagnosed in 2017
- 53,200 will be benign
- 26,070 will be malignant
- The average survival rate for all malignant brain tumor patients is only 34.7%
- Male: 33.5%
- Female: 36.1%
- For the most common form of primary malignant brain tumors, glioblastoma multiforme, the five-year relative survival rate is only 5.5%
- An estimated 16,947 people will die from malignant brain tumors (brain cancer) in 2017
- The most prevalent brain tumor types in adults:
- Meningiomas, which make-up 36.6% of all primary brain tumors
- Gliomas (such as glioblastoma, ependymomas, astrocytomas, and oligodendrogliomas), which make-up 74.6% of malignant brain tumors
- More than 28,000 children (0-19 years of age) are estimated to be living with a brain tumor in the US
- An estimated 4,830 new cases of childhood and adolescent (15-19 years of age) primary malignant and nonmalignant brain and CNS tumors are expected to be diagnosed in 2017
- The average survival rate for all primary pediatric (0-19 years of age) malignant brain tumors is 73.8%
- Brain and CNS tumors are the most prevalent form of pediatric cancer in kids under 19
- Pediatric brain tumors are the leading cause of cancer-related death among children and adolescents ages 0-19, surpassing leukemia.
- It is estimated that, in 2009, a total of 47,631.5 years of potential life were lost due to brain tumors in children 0-19 years old
- The most prevalent brain tumor types in children (0-14):
- Pilocytic Astrocytoma
- Malignant Glioma
- Neuronal and mixed neuronal-glial tumors
- More than any other cancer, brain tumors can have lasting and life-altering physical, cognitive, and psychological impacts on a patient’s life.
- This means malignant brain tumors can often be described as equal parts neurological disease and deadly cancer.
- Even benign brain tumors can be deadly if they interfere with portions of the brain responsible for vital bodily functions.
- There are more than 140 different types of brain tumors, many with their own multitude of subtypes.
- Despite the amount of brain tumors, and their devastating prognosis, there have only been four (4) FDA approved drugs – and one device – to treat brain tumors in the past 30 years.
- For many tumor types, surgery and radiation remain the standard of care.
- There has never been a drug developed and approved specifically for malignant pediatric brain tumors.
- The four approved drugs for brain tumors have provided only incremental improvements to patient survival, and mortality rates remain little changed over the past 30 years.
- Between 1998 and 2014, there were 78 investigational brain tumor drugs that entered the clinical trial evaluation process. 75 failed. That is a 25:1 failure ratio in developing new brain tumor treatments over the past two decades.
- Brain tumors have the highest per-patient initial cost of care for any cancer group, with an annualized mean net costs of care in 2010 US dollars at well over $100,000.
- While, there is no official accounting of, and some significant disagreement regarding, the amount of cancer cases each year that metastasize to the brain. Estimates range on the low end from approximately ~56,000 cases to ~500,000 on the high-end of cancer patients developing brain metastases annually.
- Studies have cited that the percentage of cancer patients who will develop brain metastases is anywhere from 6-28%.
- What we do know is that approximately 80% of cancers have been associated with the ability to metastasize to the brain.
 “Researching Cancer: Setbacks and Stepping Stones,” PhRMA, 2014  J Natl Cancer Inst. 2011 Jan 19; 103(2): 117–128. (reference braintumor.org)
SurgerySurgery is the main treatment for non-cancerous brain tumours. The aim is to remove as much of the tumour as safely as possible without damaging the surrounding brain tissue. In most cases, a procedure called a craniotomy will be performed. Most operations are carried out under a general anesthetic, which means you'll be unconscious during the procedure. But in some cases you may need to be conscious and responsive, in which case a local anesthetic will be used. An area of your scalp will be shaved and a section of skull cut out as a flap to reveal the brain and tumor underneath. The surgeon will remove the tumor and fix the bone flap back into place with metal screws. The skin is closed with either sutures or staples. If it isn't possible to remove the entire tumor, you may need further treatment with chemotherapy or radiotherapy.
RadiosurgerySome tumors are located deep inside the brain and are difficult to remove without damaging surrounding tissue. In these cases, a special type of radiotherapy called stereotactic radiosurgery may be used. During radiosurgery, tiny beams of high-energy radiation are focused on the tumor to kill the abnormal cells. Treatment consists of one session, recovery is quick, and an overnight stay in hospital isn't usually needed. Radiosurgery is only suitable for some people, based on the characteristics, locations and size of their tumor.
Chemotherapy and radiotherapyConventional chemotherapy is occasionally used to shrink non-cancerous brain tumors or kill any cells left behind after surgery. Radiotherapy involves using controlled doses of high-energy radiation, usually X-rays, to kill the tumor cells. Chemotherapy is less frequently used to treat non-cancerous brain tumors. It's a powerful medication that kills tumor cells, and can be given as a tablet, injection or drip. Side effects of these treatments can include tiredness, hair loss, nausea, and reddening of your skin.
Medication to treat symptomsYou may also be given medication to help treat some of your symptoms before or after surgery, including:
- anti-convulsants to prevent epileptic seizures
- corticosteroids to reduce swelling around the tumor, which can relieve some of your symptoms and make surgery easier
- painkillers to treat headaches
- anti-emetics to prevent vomiting
This article has been contributed by: Shabbar F. Danish, M.D. Division of Neurosurgery,Robert Wood Johnson Medical School (RWJMS),New Brunswick, NJ
Gamma Knife surgery (GKS) represents a type of knife-less non-invasive surgery that serves as an alternative to traditional surgery. It is categorized as stereotactic radiosurgery since it involves both 3-dimensional (3D) images and single-session radiation therapy. GKS has fewer risks when compared to conventional surgical options, such as open-skull brain surgery. There is no risk of infection, bleeding, stroke, or seizures because there are no incisions or manipulation of the brain. Additionally, there is no anesthesia-induced risk since, typically, only mild sedation is needed. All cases are done as outpatient procedures with patients going home the same day, and in many instances going to work the following day. GKS is the only radiosurgery developed exclusively for treatment in the brain, and should not be confused with CyberKnife, Novalis, or XKnife- machines that specialize in treating regions outside the brain. The Gamma Knife differs from these other modalities in terms of accuracy, radiation delivery and distribution. The Gamma Knife uses a stereotactic frame to stabilize the patient’s head, therefore providing sub-millimeter accuracy (1.0 mm or less). Meanwhile the other modalities are frameless and rely on linear accelerometry, leading to relatively less accuracy. In terms of radiation delivery, GKS has less radiation scatter and thus less spread of the radiation to unwanted areas of the body. Today, nearly 500,000 patients worldwide have been treated with the Gamma Knife and nearly 20+ years of scientific literature support its efficacy.
The newest update of GKS, the Gamma Knife PerfeXion system, involves a sphere-like helmet with 192 sources through which the radiation is delivered. MRI-derived skull/brain reconstruction can be used to generate a 3D model. Through stereotactic mapping, the 3D model can be used to localize a specified lesion with x,y,z coordinates in 3D space. The Gamma Knife system utilizes gamma rays derived from the nuclear decay of a Cobolt-60 radioisotope. Each of the 192 sources can deliver a beam of gamma rays that then penetrate the tissue in the beam’s trajectory. By choosing the optimal combination of angles, the gamma ray beam can be precisely focused onto the lesion using 3D modeling from images. The PerfeXion should not be confused with older versions of the GammaKnife. The PerfeXion is fully automated, which results in reduced treatment times, and as a result less radiation scatter to normal body parts. It employs a much wider inlet, which is less claustrophobia-inducing.
Am I a candidate?
Those patients that have an abnormality or tumor in a difficult to reach location are considered as candidates. Other candidates include patients that are not healthy enough for standard open-skull surgery and those for whom less invasive treatment is preferred – such as those with other major co-morbidities. The typical conditions that are treated with the GKS include: brain tumors, trigeminal neuralgia, pituitary tumors, and arteriovenous malformations (AVMs).
Are there any risks?
The risks of Gamma Knife surgery are minimal when compared to standard open surgery. However, as with any medical procedure there are risks. Early complications are usually temporary and include: edema formation near the treatment site leading to headaches, scalp irritation or sensitivity, and fatigue for several days to weeks post-operatively.
What Can I expect?
Although the procedure is usually in an outpatient setting, the time frame usually involves a large portion of the day. It is recommended that you bring a family member or friend with you who can take you home. Prior to the procedure, you’ll have an intravenous (IV) tube placed to deliver medications and fluids to keep you hydrated. Before the procedure begins, a lightweight stereotactic frame will be attached to your head with four pins. The frame is designed to stabilize your head such that all points within your head remain constant relative to the frame. This is critical for accurate and precise lesion localization. A localized anesthetic will be given before the pins are placed – at 4 points total, 2 at the front and 2 at the back of your head. Usually, none of your hair will be shaved. After this, you’ll have additional MRIs taken of your brain and then you’ll lie with your head in the Gamma Knife helmet with the stereotactic frame in place. During the procedure, you won’t feel the radiation and will be able to communicate with the doctors through a microphone.
What Types of Tumors Can be Treated?
Benign or “not so benign” tumors, such as meningiomas, can be successfully treated with GKS. Since meningiomas are commonly treated with stereotactic radiosurgery, there is a need to elaborate on the scientific and clinical components of this tumor type. They originate from the arachnoid cap cells of the arachnoid meningeal layer. On the cellular and genetic level, the origin of typical meningiomas is closely linked to malfunctions in the neurofibromatosis type 2 (NF2) and 4.1B (DAL-1) genes. Other involved genes include BAM22, break point cluster region (BCR), and TIMP-1, the last being implicated in higher-grade meningiomas. Atypical meningiomas are also related to genetic malfunctions including mutations in chromosomes 1, 6, 10, 14, and 18. Through the World Health Organization (WHO), meningiomas are classified based on their pathological and histological morphology (Table 1).
Grade II and III meningiomas are more likely to develop invasive disease and have a higher rate of recurrence with a lower overall survival rate. Studies have shown that Grade I cases have a recurrence-free survival in nearly 90% of cases, while that with Grades II/III have recurrence in 30-50% of cases at 3-5 years, even after complete resection.
Typically, active observation is primary in asymptomatic and incidentally diagnosed cases. For symptomatic cases, surgical resection is the treatment of choice. Postoperative complications can occur in 5-30% of cases, depending on the location of the tumor. Radiosurgery is the treatment of choice when standard open surgery is contraindicated, there is incomplete resection, or there is a Grade II / III meningioma – the last due to the risk of recurrence. After radiosurgery, the risk of recurrence remains but careful monitoring can help to catch potential recurrences early and allow for a decision on additional radiotherapy. Studies of patients with varying types of meningiomas have shown that survival may approach 85% at the 5-7 year time point after GKS.
A recent study revealed that GammaKnife was just as effective as fractionated radiation in the control of skull base meningiomas, and provided much better quality of life to patients versus those who underwent open surgery. GammaKnife has control rates of 95% for cavernous sinus meningiomas, making open surgery for these lesions unnecessary and an obsolete treatment strategy in most cases. In a large study examining long term results after GammaKnife, patients experienced >95% control, with >70% of tumors shrinking over a 10 year observation period.
Adjuvant therapies such as radiation and medical therapy has been investigated in the medical community, however they remain controversial. The main question that remains to be answered is whether adjuvant therapy is better than simply watchful waiting. Medical therapies that have been investigated include hormonal therapies such as anti-progesterone therapy or immunotherapy with alpha-interferon. These approaches have shown modest results and may be useful when the tumor cells express receptors for these substances. More substantial results have been demonstrated with hydroxyurea, however, there are studies still investigating this. Recently, pre-clinical studies have pointed towards drugs such as trabectedin and histone deacetylase inhibitors as potential therapies. Nonetheless, several clinical trials are under way to test candidate drugs such as platelet-derived growth factor receptor and anti-angiogenic therapies.
What is the outcome like?
The outcome depends on the type of tumor, location, and patient’s medical history. For benign tumors, there is usually a failure of tumor cells to reproduce over the course of 1-2 years. The outcomes after meningioma treatment are typically positive; however, even a benign meningioma can be “malignant” if it’s in an “anatomically-bad” place. For malignant tumors, usually, the tissue shrinks faster (few months) since vascular supply is compromised as a result of GKS. However, other, non-tumor conditions, such as AVMs and trigeminal neuralgia, can take several months to years to fully go away.
What happens after the surgery?
Usually, the patient goes home the same day and then will follow-up as an outpatient with his or her neurosurgeon and radiation oncologist. Depending on the patient’s course, additional imaging such as CAT-Scan (CT) or Magnetic Resonance Imaging (MRI) may be ordered. At each visit, the physician will perform a neurological exam to evaluate the patient’s function.
- A typical treatment day. International RadioSurgery Association. http://www.irsa.org/treatment.html. Accessed Aug. 3, 2010.
- Gamma Knife® surgery. International RadioSurgery Association. http://www.irsa.org/gamma_knife.html. Accessed Aug. 3, 2010.
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- Vestibular schwannoma (acoustic neuroma) and neurofibromatosis. National Institute on Deafness and Other Communication Disorders.http://www.nidcd.nih.gov/health/hearing/acoustic_neuroma.html. Accessed Aug. 4, 2010.
- Trigeminal neuralgia fact sheet. National Institute of Neurological Disorders and Stroke.http://www.ninds.nih.gov/disorders/trigeminal_neuralgia/detail_trigeminal_neuralgia.htm. Accessed Aug. 3, 2010.
- Stereotactic radiosurgery overview. International RadioSurgery Association. http://www.irsa.org/radiosurgery.html. Accessed Aug. 3, 2010.
- Kavanagh G, et al. Complications of cranial stereotactic radiosurgery. http://www.uptodate.com/home/index.html. Accessed Aug. 4, 2010.
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- Sioka, Chrissa, and Athanassios P. Kyritsis. “Chemotherapy, hormonal therapy, and immunotherapy for recurrent meningiomas.” Journal of neuro-oncology 92.1 (2009): 1-6.
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Director, Stereotactic and Functional Neurosurgery Assistant Professor, Robert Wood Johnson Medical School New Brunswick, New Jersey USA