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Oliver’s Story

Malignant Peripheral Nerve Sheath Tumor

By Shelley Holshey

Malignant Peripheral Nerve Sheath Tumor  

(from a layperson’s perspective)

ü  MPNST is a soft tissue sarcoma, one type in a family of similar tumors that appear in the skin and fatty tissues of animals.  Tumors occur in the outer sheathing of nerves.

 

ü  These tumors are usually relatively slow growing and found most often in middle aged dogs on the extremities or lateral body surfaces.

   

ü  Surgery (or amputation), where “clean margins” can be achieved, is the most successful cure.  

 

ü  Radiation therapy (alone or in combination with surgery when clean margins are not achieved) is often successful in stopping tumor growth. Chemotherapy is ineffective in treating MPNST.

 

ü  Radiation therapy for MPNST is a 19 treatment protocol with treatments being administered daily (Monday through Friday) for 4 weeks.  Each treatment must be administered under anesthesia and with intubation.  The dog will develop burn-like sores after about the first week and may need steroids and pain medications.  Bone tumors in irradiated areas sometimes occur later.

 

ü  Radiation treatment is expensive at approximately $4,000 to $6,000 depending on treatment center.

  

Oliver’s Story

It all began with a new lump.  For our seven year-old Weimaraner “Oliver”, lumps and bumps were far from unusual.  Fatty tumors are common in this breed (and many others) and very easily observed since the Weimaraner coat is extremely short.  We regularly pointed out his lumps to his veterinarian during annual physicals.  Our local veterinarian (we’ll call her Dr. G) has always carefully monitored these areas and performed needle aspirations when one appears suspicious.  Thankfully, all had been simple lipomas (benign fatty tumors) and were no cause for alarm.  However, the new lump on Oliver’s right flank near his hip seemed to arise more quickly than others.  Just to be safe, we made an appointment to have it checked.  Dr. G. did a series of needle aspirations on five lumps and only the “new” lump produced a bloody mixture rather than the fatty material that usually comes from lipomas.  So, we made an appointment for two days later to have the lump removed and sent to a pathologist.   

Oliver suffers from severe separation anxiety so, over the years, Dr. G. has allowed us to pick him up immediately after he becomes conscious after anesthesia.  We picked up the groggy, anxiety ridden fellow and headed home with the hope that this was a false alarm.  However, unfortunately it was not.  Two days later, Dr. G. called and said “I’ve been putting off calling you because I don’t know how to tell you this, except to tell you directly.  Oliver has a Malignant Peripheral Nerve Sheath Tumor” (MPNST).  I nearly collapsed with shock, this dog is only seven and I’m not prepared to lose him.  Dr. G said that she would call the local veterinary referral service surgeon the next day and get back to us.  She explained that surgical removal of the tumor plus “clean margins” (an area of healthy tissue surrounding the malignancy and assuring that the cancer has not spread into the surrounding tissue) is the best chance for a “cure”.  The pathology report indicated that the original surgery had not produced clean margins.  It was a long, tear filled night for our family.  Shock, dread, confusion and despair were all rampant.   

Learning about MPNST

I decided that I needed to learn as much as I could about MPNST for two reasons.  The first was to be the best possible advocate for my beloved dog, but selfishly, the second was so that I could prepare myself for what might happen.  I started with the internet, and became quickly aware that very little information is available on MPNST; there were a few cases documented about human occurrences and a couple scholarly studies in advanced canine occurrences.  None of this seemed to apply directly to Oliver’s case.  He was an otherwise healthy dog with no symptoms.  The canine cases that I found all pertained to animals with more advanced tumors who initially presented with lameness or other symptoms.  I was getting scared.

One of the most valuable things I did next was to Google “pets with cancer” which linked me to this website and provided me with an email address so that I could reach out.  I was trying to find other people with pets diagnosed with MPNST.   As it turned out, I received several supportive emails; however none were from people with MPNST experience.  BUT, those supportive emails made a huge difference.  Suddenly, I wasn’t alone.  There were other people out there who were feeling, or have felt, all the same emotions.  I even received a phone call from one person, just offering support and understanding.  I can’t overstate the value of a supportive network when one is waging an unexpected battle.  The kindness and goodness of utter strangers became the “wind beneath my wings” as I now had a “team”.  I was researching and fighting for my Oliver, but also for other people who might receive the MPNST diagnosis. 

There are several descriptions available for MPNST.  These can be pretty formal, so I will just try to state my laypersons understanding here.  This is just the product of my analysis of information obtained during my research and shouldn’t be considered to be a medical opinion.  MPNST is a soft tissue sarcoma, one type in a family of similar tumors that appear in the skin and fatty tissues of animals.  What makes MPNST a differentiated condition is that it produces a cellular level tumor that occurs in the outer sheathing of nerves.  These tumors are usually relatively slow growing and found most often in middle aged dogs on the extremities or lateral body surfaces.  Metastasis is rare, but local recurrence is common when “clean margins” are not achieved surgically.  Depending on the tumor’s location and other obvious considerations, amputation of the effected limb is sometimes curative, though not a reasonable approach for Oliver.  Chemotherapy is ineffective in treating these tumors; however radiation therapy (alone or in combination with surgery) is often successful in stopping tumor growth.

So, with this knowledge, I set off to try to talk to specialists.  Dr. G. called and said that the surgeon she consulted felt that surgery was not an option (surprisingly, even without examining Oliver) and that he believed we should pursue radiation therapy. Dr. G recommended that we take Oliver immediately to Gaithersburg, MD, about 2 hours from our home, to consult with Dr. Eric Boshoven, a radiation oncologist.  Having had experience with humans suffering from life threatening conditions, it seemed to me that I should seek a consultation with an oncologist before going straight to radiation therapy, in an effort to have someone with cancer expertise coordinate Oliver’s total treatment, not just radiation.  I wanted someone to examine the dog and give me treatment options specific to Oliver’s case, not just based on a one-page pathology report. 

I began compiling recommendations from people in the “dog world” including my aunt who prevailed upon her friends in the Newfoundland community and from PWCL about treatment centers in driving distance from our home.  It came down to three locations:  the University of Pennsylvania in Philadelphia (about 2 hours from home), the office of Dr. Anne Jeglum in West Chester, PA (about 1.5 hours, although their radiation therapy office is in Chalfont, PA about 3 hours away) and Dr. Boshoven’s practice in Gaithersburg.  I called and requested appointments at Dr. Jeglum’s and at Penn.  The first appointment available was at Dr. Jeglum’s in a few days.  Since Dr. Jeglum was on vacation, we saw another doctor in her practice.  By the way, going to a veterinary oncologist’s office is a tough experience; there are lots of sick animals and sad people.  This was tough to get through.  The doctor who examined Oliver explained that he would need to undergo radiation therapy to treat his tumor.  

What Radiation Therapy entails:

 

Radiation therapy for MPNST is a 19 treatment protocol with treatments being administered daily (Monday through Friday) for 4 weeks.  Each treatment must be administered under anesthesia and with intubation.  The dog will develop burn-like sores after about the first week and may need steroids and pain medications.  Bone tumors in irradiated areas sometimes occur later.

The treatment would cost about $4,000.  Since Dr. Jeglum’s radiation therapy facility is in Chalfont, PA, the doctor we saw suggested boarding Oliver for the month and allowing him to be treated daily.  I tried to explain his separation anxiety, but I think that she may have thought I was overstating the condition.  Regardless, I know my dog better than anyone and I was absolutely certain that he would not survive this treatment plan.  I would need to take a leave of absence from work, get a hotel room and devote the month to taking Oliver back and forth from treatments. 

While I was preparing myself for the likelihood that radiation would be required, I decided to research whether there are other protocols that involve fewer or less frequent treatments.  I called Penn, but found that the radiation oncologist will not speak with non-clients (I learned that this is the standard response from specialists).  Since it was not yet our appointment date, I couldn’t determine what the regimen would be at Penn.  Finally, I spoke with Dr. Craig Clifford at Red Bank Veterinary in New Jersey (once Berry the Dog’s doctor, thanks for the “in”, Carol!!) who explained that the “state of the art” protocol (and one that I should anticipate any radiologist recommending) would be the 19 daily treatments.  This information was pivotal since it had now become abundantly clear that logistics would play a big role in our ability to successfully facilitate radiation treatments on a daily basis.  Therefore, I called and made a consultative appointment with Dr. Boshoven at the Gaithersburg VCA Veterinary Referral Associates.  Going to Penn would be more difficult and it seemed that the protocol would be the same.  I felt time was of the essence and it was necessary to move forward.  Interestingly, during my conversation with Dr. Clifford, he expressed his confidence that we would “be in good hands” with Dr. Boshoven, but that we also not give up on a surgical removal of the tumor in an attempt to get clean margins before going straight to radiation, given the location of Oliver’s tumor and its early stage.  I was so happy to have a doctor not dismiss the possibility of a surgical treatment.  If we could get clean margins, Oliver would not need to endure the radiation and we could be more certain that the cancer was eradicated. 

Our visit to Dr. Boshoven went well.  He was very kind and spent over an hour just talking with us.  Oliver, who is not generous with kisses, even bestowed one on the doctor!  At first, Dr. Boshoven thought that surgery would not be an option since the tumor site was at a point on the front of the flank near Oliver’s abdomen side.  We talked all about what radiation was going to be like and Dr. Boshoven was very, very reassuring.  We asked all kinds of questions including the repeated anesthesia risk.  Overall, the prognosis was good with a likely cure.  The cost would be around $5,500.  After he completed Oliver’s physical exam, Dr. Boshoven said that he wanted to retract his initial comments regarding surgery.  He said that he wanted to take Oliver to see Dr. David Saylor, a surgeon (who incidentally is a soft tissue sarcoma surgical expert) and VCA Medical Director.  After the consultation, we got the welcome news that Dr. Saylor thought he could go get any remaining tumor and get clean margins.  The surgery was set for three days later.   Dr. Boshoven said that he believed there was about an 85% likelihood that the pathology would come back with adequate margins and or no remaining cells.  If not, unfortunately radiation would be our last chance at a cure.

Surgery

The day of surgery was very stressful.  I was so nervous after they took Oliver back for surgery that I even asked the receptionist to call back to the surgeon and make sure that he knew that I had not approved an amputation.  I think they all thought I was totally nuts.  Maybe I am.  The surgery took about 1 ½ hours.  Afterward, I spoke with Dr. Saylor who said that the surgery went well and he saw nothing suspicious inside Oliver's leg, but that this cancer is at the cellular level, so it's just hard to say.  He did say that the difficulty they had settling Oliver resulted in them having to use very high levels of sedation. The assisting surgeon said that they used enough for a horse.  So, we went off to try to have some lunch since they said he would be released no earlier than 2 pm.  As I waited in line at the deli at 11:45, my phone rang.  It was the assisting surgeon saying that we needed to come get Oliver, that he was inconsolable.  So, we sped to the vet.  They brought him out and he was totally out of it.  The incision was twelve inches long, and they removed two inches of tissue in width, so there were multiple layers of stitches internally and staples externally.  We left with codeine for pain and Deracoxib for inflammation.  The ride home was horrific.  Oliver cried, panted, lost control of his bowels, and yelped in pain.  His tongue was so dry and numb that it wouldn't retract into his mouth.  Once we were home, he continued panting and crying, wouldn't lie down, and only wanted to stagger around.  I made a bed on the floor and tried to hold him there with me.  I thought my heart would actually break; it was so bad that I trembled.  Finally, at 6 pm, I called back to the vet.  He said that Oliver's personality makes him very stressed under sedation; he just doesn't like to feel that something is off.  As for the pain, he said that we could increase the codeine to four tablets every four hours, but that the risk was that the analgesia would stress Oliver in the same way the sedation did.  It was a long, difficult night for all of us.

Then the wait began.  While Oliver was suffering that first night, I said that this was the last thing we would put him through.  It was enough.  I was so afraid that if radiation was required, he wouldn’t be able to tolerate the daily anesthesia.  Even with radiation, the radiologist told us, they can't “see” this cancer, so they just guess on the size and depth of the radiation target and hope they get it all.  If the bad cells were further advanced than the huge tissue mass that they removed, it would be a pretty big cancer.  So, we tried to keep an open mind and wait until we had the facts and could make any final decisions.  This is a very special, but very different, dog.

Six long days later, we were awakened by a call from Dr. Saylor’s assisting surgeon.  It was the good news for which we had prayed.  The wide excision and aggressive removal of tissue seemed to contain all the "bad stuff" plus a clean one centimeter margin.  In fact, the pathology report said that they detected no malignant nerve sheath tumor cells in the sample; however, since there was a fairly large area of inflammation, the tumor cells could have been hidden by the huge number of cells present.  Regardless, the clean margin was enough that the doctor recommended forgoing radiation treatments, especially given Oliver’s reaction to the surgery and anesthesia.  It will be important for Oliver to be examined for recurrences every three months for two years.  The final report said that if recurrences were not present over the two years, the cancer could be considered cured. 

Four weeks on....

As I write this, it has been a little more than four weeks since Oliver’s diagnosis, and nearly three weeks since his last surgery.  The recovery from surgery has been a bit more difficult than I originally anticipated.   The staples closing the incision are under constant stress and pull on the skin remaining on either side.  This is due to the large size of the incision and “gap” that needed to be closed after removing enough tissue to achieve clean margins”.  As do most dogs, Oliver really wants to lick his wound.  This is very detrimental to healing so Oliver is usually attired in some combination of boys’ basketball shorts and/or an inner tube type device that is an alternative to the typical Elizabethan collar.  He finds ways to work around both, but the end is now in sight, with the final staples set to be removed this week.  

While we are hopeful that the end of this chapter will be a happy one, we will be diligent in checking new lumps and watching the original site for the rest of Oliver’s life.   We have been very blessed to have found the original lump so early and to have enjoyed such great medical care subsequently.  Having a pet with cancer is a big challenge.  It takes a great deal of dedication and stamina.  Consideration of the pet’s best interest is obviously paramount, and this requires a robust combination of research, networking and persistence - all tempered with the very private understanding of one’s individual pet.  I am very thankful that we were so well supported by the members of PWCL and our “family” of friends.  I don’t think we could have navigated these turbulent waters otherwise. 

You can read more about Oliver at:  http://shelley-oliver.blogspot.com/