Monthly Archives: March 2015

March is Brain Injury month, and I wanted to devote an article to this topic.

Brain injury is a broad topic that includes everything from concussions to gunshot wounds.  For this article I will discuss the topic of traumatic brain injuries (TBI).  Even though TBIs represent a subset of brain injuries, the causes are diverse, ranging from falls to anoxic brain damage (lack of oxygen to the brain resulting in brain cell death).  In 2010 the CDC released a study that looked at data from 2002 – 2006.[1]  In the study, among other findings, they concluded that the rate of death among all Emergency Department (ED) visits for TBI was a shocking 3.0%.  Meaning that of the 1.7 million visits to the ED per year, 52,000 resulted in deaths from TBI.  This number however, while unbelievable, does not portray those of us who do not die yet are left with the sequelae of brain cell death.  In fact, because of these unwanted effects, TBIs have been called “the silent epidemic”.[2]

Let me first define TBI: TBI occurs as a result of any external force that causes any trauma to the brain.  The most popular form of TBI is from concussion (the one you hear about on the news or Sportscenter), which occurs when someone strikes their head causing a bruising of the brain.  The results vary widely, from a brief headache to death.  However, for many of us TBI does not occur on a field.  For me, it took place in an Operating Room.  The CDC estimates that over 50% of all TBIs are the result of a fall.[3]

While the range of symptoms that TBI can cause is vast, the effects it can have on our lives is even more varied including little to no changes to our lives to permanent disability.  The National Institute of Neurological Disorders divide TBIs and their consequences into mild, moderate, or severe “depending on the extent of damage to the brain.”[4]

To us, these are merely numbers and definitions that serve to help us understand this entity, however the real ‘meat’ of this topic is rooted in recovery.  Yes, we now know that TBIs cause over 50,000 deaths each year and that its effects are far-reaching, but those are all events that have already occurred and are beyond our control; it is more important to know what we can do to help us recover.  The human body and the brain are amazingly resilient; thus part of the recovery process simply requires the ‘tincture of time’.  This is not a short process, as my physician told me in the hospital, “Chris, remember this isn’t a sprint, it’s a marathon.”  I often remember this when I find myself wishing my recovery process were quicker.

When I was discharged from the hospital last year, I was given a prescription for Speech, Occupational, and Physical therapy.  These are all modalities of rehabilitation that focus on different areas of recovery: Speech Therapy is self-explanatory, with the goal of improving speech deficits.  Occupational Therapy aims at helping with daily and work skills.  Physical Therapy attempts to aid patients through improvements to mobility and function.  Throughout this process I have been praised for my hard work.  While these comments are extremely kind, I assumed that everyone works hard and ‘gives 100%’ when rehabilitating in therapy.  However, I feel that a satisfying recovery requires two virtues: patience and determination.  Without patience, the thought of a long arduous journey to recovery would be daunting.  At times you will feel like Sisyphus pushing that boulder up the hill only to see it roll back down.[5]  Without patience you might not see that you are making progress, and that your hard work, your suffering does have a purpose.  This patience though will be useless without determination as well.  It is determination that pushes you to complete that extra exercise or begin that extra vocal activity.  Otherwise, while you may partake in those endeavors, without determination you’d simply be going through the motions and lack the true effort.

In medical school we are introduced to the incredible story of Phineas Gage.  On September 13 of 1848, Gage, working as a foreman for a railroad, underwent a horrific accident that resulted in an iron rod piercing through his left eye socket into his brain and out through his skull.  His story is an incredible one because not only did Gage live through this incident, but he became a productive member of society afterwards.[6]  In considering brain injuries and their effects, we should think about the story of Gage.  He reminds us of the power of the mind and its ability to overcome the greatest challenges.

[1] http://www.cdc.gov/traumaticbraininjury/pdf/blue_book.pdf

[2] Id.

[3] Id.

[4] http://www.ninds.nih.gov/disorders/tbi/tbi_htr.pdf

[5] http://www.mythweb.com/encyc/entries/sisyphus.html

[6] http://www.smithsonianmag.com/history/phineas-gage-neurosciences-most-famous-patient-11390067/?no-ist

This week I would like to discuss the clinical science of Epidermoid Brain Tumors. I hope to delve into what is known about this condition, however, it should by no means serve as a reference guide to these tumors. I will format this article like many medical publications format their data. I feel this format best highlights the salient points and is most appropriate for a discussion on the scientific background of epidermoid tumors.  You can blame my many years of reading solely medical literature but I have formatted this article like a medical article with sections like: Definition, Epidemiology, Symptoms, Diagnosis, Special Cases, and Treatment.

I am typically very inquisitive by nature; if I see or read about something new to me, I’ll immediately try to gather as much information on the subject that I can.  However, when I was diagnosed with a ‘probable Epidermoid Tumor’[1], I deliberately did not research the topic. I wanted to know as little as possible before undergoing surgery – maybe I was afraid of what I’d come across, or maybe I was simply too preoccupied with family and friends to have the time to delve into the condition, but regardless of the reason for my purposeful ignorance, the end result was the same: a confirmed diagnosis of an Epidermoid Tumor and a forever changed life.

It was not until several weeks ago that I finally performed a formal ‘literature search’ of Epidermoid Tumors.  The search returned over 200 articles of medical literature detailing the condition.  However, this number is misleading as many of these articles were of little use to me (i.e.: case reports[2], studies of Epidermoid Tumors on animals, rare gene mutations surrounding the tumor).  While each and every publication has its significance, my aim to provide you with an overview of the tumor did not require these details.  The important take-away of this search is that it revealed something I had long suspected: even though this tumor has been around for a very (very) long time (see this amazing article at http://anthropology.net/2009/06/15/first-neanderthal-fossil-dredged-from-north-sea/), little is still known about the tumor.  Allow me to attempt to summarize what I found:

–   –   –   Definition  –   –   –   

Epidermoid Tumors are benign cysts that typically develop in either the brain (intracranial) or in the spine.[3]  It is a congenital tumor, meaning it is present at birth.  It is postulated that the tumor begins its growth between the 3^rd and 5^th weeks of life (in the womb).[4]  The tumor arises from an abnormal growth of epithelial cells (a type of animal tissue) and is sometimes called ‘pearly tumors’ because of its white, curdy appearance.[5]

–   –   –   Epidemiology  –   –   –   

This section centers around demographics of the tumor.  It is estimated that 0.3 – 1.8% of all intracranial brain tumors are Epidermoids.[6]  In some studies, albeit small ones, there is a slight female preponderance.[7]  The tumor seems to like the back of the skull, with an estimated 37.3% of these tumors located in the back part of the brain.[8]  What I’ve given you above are numbers; figures that reflect statistics found in studies, take these numbers with a grain of salt as really this tumor, although rare, can effect anyone, anywhere.

–   –   –   Symptoms  –   –   –   

Describing the symptoms patients may experience as a result of this tumor is tough because the tumor can have such a wide array of effects.  The most common symptom described is headache.[9]  In fact, a published case study describes a patient with specific headache symptoms (called Trigeminal Neuralgia) who actually was suffering from an Epidermoid Tumor.[10]  The reason I tell you that these symptoms are hard to describe is because symptoms really depend on where the tumor is located.  These can range from conditions as severe as seizures to something as benign as taste difficulties.  There are many nerves (some called cranial nerves) that pass through this area making themselves vulnerable to being pushed on by the tumor.  For me, I awoke one morning with double vision.  This became worse until I finally told my Family Doctor about it, who obtained an MRI.

The bottom line is that because the symptoms are so varied, the diagnosis cannot be made solely on the basis of symptoms and physician evaluation.  By no means does everyone suffering from a headache have an Epidermoid Tumor, but this is when the clinician must pick up on other clues to facilitate further investigation or imaging, such as an MRI or neurological referral.  This is an ideal segue to the next section, ‘Diagnosis.’

–   –   –   Diagnosis   –   –   –  

As I alluded to above, because symptoms are so varied, imaging is needed to aid in the diagnosis.  Unfortunately, this tumor does not show up on regular x-rays.  (In the past, the only way for it to be seen on x-ray is when the tumor grew enough to erode the skull bone).

In medicine, it is generally accepted that Magnetic Resonance Imaging (MRI) is superior to Computed Tomography (CT) when examining masses.  There are reasons why CT is used over MRI in many instances: CTs are more sensitive at picking up bleeding in the brain, can be performed quicker, and cheaper, and any metal in your body (i.e. a pacemaker) precludes you from having an MRI done.  Epidermoid Tumors can be seen on CT, however, MRI is still a more accurate means of visualizing the mass.  It pains me to say this, but there are some health insurances that require the patient to get regular x-rays before they will cover an MRI.

While MRI can point you (and your clinician) towards the direction of an Epidermoid Tumor, actually looking at the tumor under a microscope is needed for a diagnosis.  The medical school adage, ‘tissue is the issue’ (for some reason doctors like phrases that rhyme) holds true here.  As I was told by my neurosurgeon before my surgery, “I can tell you that it’s probably an Epidermoid Tumor based on the MRI, but there is no way for me to be sure without actually going in, taking it out, and having a pathologist look at it.”

–   –   –   Treatment   –   –   –  

Treatment for these tumors is typically surgical.  This comes with a caveat: brain surgery is an incredibly invasive surgery, thus if it can be avoided you and your surgeon will elect against it.  For this group of patients, a wait and watch approach is taken.  The tumor is closely monitored by MRI for signs of growth.  The interval for this surveillance is typically yearly with variations depending on clinician comfort and overall gestalt.  But symptoms always trump the MRI.  For example, if you began developing debilitating headaches as a result of the tumor, your surgeon will advise you to have surgery to remove the mass, regardless of its MRI appearance.

Unfortunately, there are no special medications that can shrink the tumor.  Any medication given is typically to aid with symptoms, these do not serve the purpose of treating the tumor itself.

–   –   –   Special Cases   –   –   –  

There are a few special cases to note:

First off, what is to be done for Epidermoids in pregnancy?  Generally speaking, for both the health of the mother and fetus, surgery is avoided in child bearing women.  However, there are some instances where surgery cannot wait and be put off until after delivery, in these extreme cases surgery is undertaken.  Studies have shown good success in these cases, as the surgery does not involve the abdominal cavity.[11]

Also, I mentioned above that this is a tumor of epithelial cells (a type of tissue) and because of this transformation of this tumor from a benign to a malignant one is an occurrence that must be mentioned.[12]  This is an incredibly rare event (on the order of hundredths of a percent), but you should know that the tumor can potentially become a Squamous Cell Carcinoma (a big term for cancer).[13]

–   –   –   Questions to Ask  –   –   –   

When faced with this diagnosis, it is important to ask pertinent questions.  It can be a difficult to do at the time. I remember being told there was a large mass in my brain, but then after that everything else was a blur.  I know that I was so shocked with the news that I did not ask any questions, let alone pertinent ones.  This is usually a diagnosis that comes out of nowhere, thus if you need time to compute and comprehend it, then ask and inquire at a later time.  Remember, this is your health and body.  It is also a slow growing tumor, so time is your ally.  For some, like myself, time is needed to come to grips with what is going on.  Go home, research the tumor, and either return to the Neurosurgeon’s office or telephone them with your questions.  Here are some of the questions I wished I would have asked:

The first decision point made in the Neurosurgeon’s mind is whether or not surgery is needed.  Thus I will divide the questions to ask based on the decision to undergo or forgo surgery:

Surgery questions:

• What are the risks of surgery? (Your surgeon will inevitably give you the generic risks of surgery i.e. infection, blood loss, death; but are there risks specific to this surgery? My surgeon warned me of possible ensuing Cerebellar Mutism, a rare complication of surgery in this area of the brain, where the patient is unable to speak for several months after the surgery).
• What is your experience in this area? Even though the tumor is rare, it is possible to find surgeons with more (or less) experience in the tumor’s location.
• What should I expect with the recovery process? Will I be in an Intensive Care Unit following the surgery?  How long should I expect to be absent from work?
• What kind of surgical approach will you be taking? (The number of new surgical approaches changes by the minute, thus it’s important to know which one your Neurosurgeon will be taking).
• I’ve read that this tumor is often contained in a capsule. If this tumor is in a capsule that’s tightly adhered to the surrounding area, how aggressive in its removal will you be?
• After the surgery, how often will I be monitored for recurrence?

Wait and Watch Questions:

• How often will you be monitoring this tumor?
• Based on this tumor’s location, what symptoms should I look out for?
• Will the monitoring be done by you? (Or a Family Physician or Neurologist)
• What is your threshold to proceed to surgery?
• In your experience, what is the likelihood that my tumor will ultimately require surgery?

I understand that this diagnosis usually comes in the form of a whirlwind, and that my telling you to compose yourself to think about the diagnosis and its implications is easy in hindsight.  But realize that you have time on your side.  Remember this is your health, and you are your own best advocate.

[1] An image, like an MRI, cannot give you a definite diagnosis.  It can only (at best) strongly suggest a diagnosis.

[2] A Case Report is an article about a single case.

[3] Epidermoid, Dermoid and Neuroenteric Cysts.  Youmans Neurological Surgery, 6^th ed. Chapter 136, p 1523

[4] Id

[5] An Analysis of Intracranial Epidermoid Tumors with Malignant Transformation: Treatment and Outcomes. Nagasawa, DT, Choy, W. Clinical Neurology and Neurosurgery. 2013, p1071.

[6] Intracerebral Epidermoid Tumor: A Case Report and Review of the Literature.  Iaconetta, G, Carvalho, G et al. Surg. Neurology. 2001, p218-222.

[7] Id, p220

[8] Id.

[9] Epidermoid, Dermoid and Neuroenteric Cysts.  Youmans Neurological Surgery, 6^th ed. Chapter 136, p 1523

[10] Epidermoid Tumor Presenting with Trigeminal Neuralgia and Ipsilateral Hemifacial Spasm: A Case Report. Otsuka, S, Nakatsu, S et al. Archive for Japanese Surgery. 1989, p245-249.

[11] Management Strategy for Brain Tumour Diagnosed During Pregnancy.  Lynch, J, Gouvea, F et al.  British Journal of Neurosurgery.  2011, p225-230

[12] Intracranial Squamous Cell Carcinoma Arising in an Epidermoid Cyst. Acciarri, N, Padovani, R, et al.  British Journal of Neurosurgery.  1993, p 565- 569.

[13] An Analysis of Intracranial Epidermoid Tumors with Malignant Transformation: Treatment and Outcomes. Nagasawa, DT, Choy, W.  Clinical Neurology and Neurosurgery. 2013, p1071-1078.

For many of us, life drastically changes after we are given the diagnosis of an Epidermoid Tumor.  For some, a wait and watch approach is taken, and an MRI at regularly scheduled intervals dictates the next step in treatment.  For people in this group they must live knowing that a tumor, a foreign body, resides in their brain.  For others, surgery to remove the mass is deemed necessary.  This is by no means a benign surgery; it is brain surgery for which your skull must be opened.  The point is that regardless of the approach, life goes on and you must re-enter it at some point.

For me, this tumor was discovered in the midst of my medical training, as I was slated to enter the last year of my three-year residency.  Due to the severity of my symptoms surgery was needed.  Re-entrance back to my life has been rocky and continues to be an ongoing journey, but it is one that is necessary.  This re-entrance is not only defined by work; people re-enter into family life, friendships, and society in general.  I had not been back to the clinic for 20 months, had a newborn son waiting at home, and my wife and elder son were anxious for me to return from the hospital. It does not matter how long you’ve been away or even if you’ve been sidelined at all, after being diagnosed with an Epidermoid Brain tumor your life is changed and it is up to you to re-enter it.  This change may be subtle, or more overt (as in my case), but the mere act of being diagnosed with this condition alters you in one way or another.

I am not a fan of using sports metaphors, but I cannot think of another way to better tie it to the situation: when an athlete suffers from an injury, such as an ACL tear, the surgery to repair this injury only marks the beginning of the journey for return.  After the surgery this athlete must begin the process of rehabilitation (in this example, physically strengthening their knee).  The recovery though is not complete once strength and mobility is back to the pre-injury form; what many overlook is the mental component of the recovery: this mental aspect of recovery begins before the physical does – once the injury occurs, he/she must go through the process of coping with the injury. The Kübler-Ross model (an often used model in medicine that details stages of grief) states this begins with denial and ends with acceptance.  Then the athlete is faced with the decision to return or not:  is undergoing surgery and/or rehabilitation for a non-guaranteed outcome worth it?  Next there is the mental hurdle of returning to the field (or rink, court, pitch, pool), and facing questions like will my injury hold up?  What if I get injured again?  Will I ever be as I was before?  The point is that there is an endless mental battle that is often overlooked.  Now you may wonder, that’s great, but how does it relate to Epidermoid Tumors?  The reason I use this metaphor is that all of us also face these mental barriers when trying to return to our lives.  Yes, some of us like myself, are also confronted with physical limitations, but the mental rehabilitation is something universal to all of our experiences with this tumor.

The question then becomes, “Are we ready for the challenges this tumor carries with it?”  While the physical challenges can often be overcome with time in the gym and/or medicine, the mental hurdles are ones that no medicine or sweat can fix.  It is a battle that many of us do not foresee, but in order to re-enter into our lives completely, it is a battle that we must win.