That Thing was Growing Inside Me?

Sensory abilities and acuity vary drastically from person to person. Some people have partial or complete loss of one or more of their five primary senses.  They must adapt to the world around them using their remaining neurosensory capabilities.  The difficulties faced by those with who are blind, deaf, or saddled with other sensory deprivations are challenging, but also a testament to human spirit, tenacity, and strength.

My youngest daughter can hear a whispered conversation from two rooms away. It is unnerving when she yells out a comment or comeback to something we assumed she couldn’t hear.  She has hearing that makes me wonder if she has some evolutionary similarities to elephants, bats, dogs, or cats!  My wife and I sometimes think we need the “cone of silence” from the old television show “Get Smart”.  Unfortunately for my daughter, she enjoys listening to loud rock music much like I did in my younger years.  As middle age approaches for her, she may suffer from the same mid-range frequency deficits I developed.

Did you say something?

Other people have a highly refined palate. There are professional tasters of wines, other alcoholic spirits, foods, spices, and even olive oil.  Professional and amateur tasters amaze me with their discernment of subtle variations in foods and beverages I will never detect.  I probably burned out many of my taste sensing receptors as a boy eating very spicy chili from Chimayo (red) and Hatch (green), New Mexico.

The olfactory sense is keen in many animals, and some humans amaze me with their prowess detecting scents far beyond my scope. Once again, a wine connoisseur can describe relationships to a variety of fruits, woods, and other flavors based on the bouquet arising from a glass of wine.  I can smell a difference between a red or white wine, but I will not speak prolifically about the subtle fragrance of grapefruit, oak, pears, and so on.  I do have one highly refined odor I can discern even at a great distance.  Chocolate.  I was once taken to a locally renowned chocolate factory in an old city in Europe.  I was allowed to taste several of the delicious chocolates.  One afternoon two days later, I was wandering the city alone when I recognized a faint odor of chocolate.  With no map and no knowledge of the winding, hilly city streets, I followed my snozz for the next twenty minutes or so until I located the chocolate factory.  I entered the shop and the amused factory owner, amazed by my ability to locate his factory in a busy urban area, gifted me with a kilogram of his finest chocolate delicacies.

Winner! Winner! Chocolate dinner!

Vision is very important to all of us. If our visual acuity is not perfect, we undergo surgical procedures, wear glasses, or insert contact lenses to provide sharp, focused views of the world around us.  Surgeons rely on their vision to perform operations every day, and we frequently wear special magnifying loops to provide greater detail of fine structures like small blood vessels or tiny duct structures.  One of the putative advantages of laparoscopic or robotic surgery is the magnified view through the surgical telescope placed into the patient’s body cavity.

I am amused by the frequency of requests from patients to see the malignant tumor or tumors I surgically remove from their body. Not a month goes by without being asked to take a photograph, or in more extreme cases, to trot the specimen out to the family members, and subsequently to the patient to show them the offending malignancy.  When the latter request is presented, I politely decline to cause a commotion in the surgical waiting area by walking out with a liver lobe, a chunk of pancreas, or a portion of the gastrointestinal tract to show to patient’s family.  I do occasionally ask my pathology colleagues to take de-identified (no patient names or information) photographs of the resected tumor, particularly for rare or unusual cases, both for the medical record and for my own use in surgical lectures.  Depending on the specific patient and their family’s ability to tolerate graphic cancer pictures, I have at times shared these pictures with the patient and interested parties.  This is always a little risky because such photographs can induce an unexpected syncopal (fainting) episode in a family member, and I don’t want anyone going to ground with a loud thump, ending up in our emergency room with a large bump on their head.

Some people like seeing pictures or video images of surgical procedures. When my son was in the second grade, I was invited to come and speak at career day.  I called his teacher and asked permission to show a carefully edited 60 second video clip of a portion of a liver resection.  She hesitantly agreed as long as I promised there would not be “spurting” (her word) blood and disturbing images leading the students to complain to their parents about their frightening career day experience.  I appeared on the allotted day wearing my surgical scrubs, a surgeon’s cap, shoe covers, and a surgical mask. The children were delighted when I provided shoe covers, a paper surgical cap, and a mask for each of them.  I explained in second grade terms what I did as a surgeon, and then asked if they wanted to see a short movie of a liver operation.  They all cheered and screamed excitedly.  I looked at the back of the room at three teachers anxiously eyeing me, wondering if they had made a mistake.  I loaded the videocassette into the machine and pushed play.  I had asked the cameraman filming this hepatic operation for close up views only, and I had carefully edited the film to show use of an instrument to gently dissect through the liver.  As soon as the images of the liver being transected appeared on the screen, high-pitched screams emitted from many of the students.  I quickly turned the video off but was met with shouts of, “No, turn it back on!”  I complied, and the video resumed to the “Oohs” and “Ahs” of the wide-eyed second graders.

About 45 seconds into the video, I quickly turned the television off. This was met with a chorus of complaints and boos from the children.  I explained to them I was compelled to turn it off because I noticed one of the teachers in the back of the room had become pale, diaphoretic (sweating profusely), and looked like she was about to pass out.  The other two teachers fanned her furiously with papers and I went and sat her down in a chair to be sure she didn’t fall over.  She recovered and smiled wanly after a few minutes.  She thanked me for participating in career day.  She almost sounded sincere.

The next week my son presented me with a packet of brightly colored construction paper in a manila folder. He informed me the teachers asked the students to write thank you notes to all of the career day speakers.  As I went through the notes, most simply said “Thank you Dr. Curley” with a crayon picture of a surgeon, or their stick figure version of a surgeon, standing wielding a dangerously large knife, bordering on something the size of a sword, over a hapless patient lying on the table.  One note I framed and hung in my office.  It had a detailed crayon picture of a gowned surgeon standing beside a patient lying on an operating room table with a piece of liver protruding from an abdominal incision.  The written note, with perfect spelling and punctuation said, “Dear Dr. Curley, Thank you for the surgery movie. It was disgusting!  Your friend, Sean.”

Mission accomplished. You are welcome, Sean!

Many patients are flabbergasted when they see an image of the tumor I removed from their body. It’s remarkable when you think about all of the tissues, cells, and organs packed inside each of us.  My favorite surgical space, the abdominal cavity, contains a remarkable array of organs including the stomach, small intestine, large intestine, kidneys, pancreas, spleen, bladder, and my favorite organ, the liver.  The liver includes the gallbladder and bile ducts.  Occasionally, patients are diagnosed with a massive tumor at some site in their body.  Sarcomas arising in the retro-peritoneum, the area behind the gut where the kidneys, aorta, vena cava, and some significant muscles and nerves reside can be larger than a basketball.  Patients and family members are astonished a tumor can grow to a relatively huge size before being detected after causing mild discomfort or other subtle symptoms.

A few weeks ago, a new patient came to my office with a giant tumor. She is a patient in her early 70’s who has been healthy and active.  She takes a single medication for her mild hypertension, which controls and normalizes her blood pressure.  She and her several daughters noticed she was becoming less active and more easily fatigued over the past four or five months.  Blood tests obtained during a visit to her family physician one month ago revealed a red blood cell count only about half of normal; she was severely anemic.  The patient noted she was not eating well and her abdomen felt “swollen”, so the physician referred her to a gastroenterologist for endoscopy and ordered a CT scan.  The CT scan was obtained before the endoscopy, and a very large tumor of the upper abdomen, greater than 20cm in diameter, was readily evident.  The gastroenterologist performed a colonoscopy, which showed no abnormalities or sources of bleeding, while an upper endoscopy revealed a small area of ulceration along the final third of the stomach.  Biopsies were obtained of this area, which ultimately demonstrated a gastrointestinal stromal tumor (GIST).  The patient was then referred to me.

This soft spoken, delightful lady and her daughters agreed to allow several medical students and residents examine and palpate her abdomen. When she lay supine on the examining room table, it appeared she had an approximately full term pregnancy with a mass palpable from the tip of her sternum extending well below the umbilicus (belly button).

The residents, medical students, and I reviewed her CT images. In addition to the large mass arising from the greater curvature of her stomach, there were two 4 cm metastases in her left liver and one similar size lesion in the right liver.  GIST frequently metastasizes to the liver.  These tumors can be treated with a medication called imatinib, a drug targeted to a specific genetic gain-of-function mutation in a tyrosine kinase receptor called c-KIT.  This patient had already been started on oral imatinib, but she was extremely symptomatic with poor appetite, early satiety, and was requiring weekly blood transfusions for persistent bleeding from the ulcerated stomach tumor.  I had a long discussion with the patient and her daughters, and after answering all questions and discussing alternatives, they agreed to an operation early the next week to remove the malignant stomach tumor and treat the three large liver metastases.

A few days before the operation we transfused the patient with two units of packed red blood cells because her hemoglobin level, which should be 12-15 gm/dL, was only 6.5 gm/dL the day I saw her in clinic. We started the operation with an incision from the tip of her sternum, called the xiphoid, to her belly button.  Upon entering the peritoneal cavity, the soccer-ball size mass protruded from the incision.  As expected from the endoscopy and CT findings, despite the large size of the tumor, it arose from a short segment along the lower aspect of the stomach.  Working with a chief resident, we dissected this sizable tumor free from the surrounding structures adherent to it, including the pancreas, duodenum, and the transverse colon.  Once the tumor was dissected free from all of these structures, it was a simple matter of firing a gastrointestinal stapling device twice along the greater curvature of her stomach assuring we were clear of the malignant tumor.  The tumor and a small volume of her stomach was removed entirely.

Earlier in my career, if I wanted a picture of a tumor or a photograph during an operation, I had to arrange for a certified medical photographer to join us in the operating room. One of the joys and advantages (or disadvantages) of modern technology is all surgeons are now their own photographer using their cellular telephones.  Patients, including this lady and her daughters, often ask me to take a picture to show them the offending malignancy.  I wanted to take the large stomach tumor over to pathology myself, so I removed my surgical gown and gloves and took a couple of quick cell phone photos of the tumor with a sterile measuring tape placed below it to provide a size reference.  I never place any patient names of numbers in these photos, and I always delete the images from my phone after sharing them with the patient and their families.  Patient privacy and confidentiality must be conserved and respected!

I carried the bucket containing the gastric GIST over to pathology, and our pathologist confirmed the resection margins were tumor-free. I scrubbed my hands again and re-gowned and gloved.  We removed the left liver containing the two large tumors.  Intraoperative liver ultrasound revealed about a dozen small tumors (3-4 mm) scattered throughout liver.  This was not an unexpected finding with a GIST, and I knew treatment with imatinib could successfully control these small tumors for many years.  The larger tumor in the right lobe was deep in the liver, but was easily destroyed completely using ultrasound-guided microwave ablation.  At the conclusion of the operation, it was easy to sew close the abdominal wall muscles because there was no longer a large tumor protruding and pressing outward.

I walked out to speak with the patient’s daughters and described the findings and results of the operation. I reported their mother was stable and did very well throughout the operation.  She required no blood transfusions and was resting comfortably in the recovery room.  The eldest daughter finally asked the question I expected, “Do you have pictures of Mom’s cancer?”

I hesitated briefly as a few of the daughters squirmed or grimaced, and then responded, “In fact, I have some on my phone. Be careful what you wish for; are you sure you want to see this?”

After quickly exchanging glances among themselves, all agreed they wanted to see photos revealing the appearance of the large tumor corresponding to the CT images I had shown them in the office. I opened the photos on my phone and was met with common exclamations of disbelief and incredulity.  The usual questions ensued, “Oh my God, how can some thing like that grow in our mother?”

“How could she be walking around with that thing in her?”

“You said that thing is 23 cm in diameter. What is a centimeter?  How many inches is that?  How on earth can something that big not just kill you outright?”

All good questions ladies. I converted centimeters into inches for them, but still sensing some uncertainty, I finished the conversion for them by saying, “Surgeons like to compare the size of tumors to common objects like food. Your mother had the equivalent of a large canteloupe melon in her belly.”

The whole family was stunned. As more family members arrived, each wanted to see the pictures of the incomprehensibly large GIST.  Low whistles, exclamations of amazement, or grunts of distress and disgust emitted from each individual who saw the images.

Happily, this delightful lady recovered from her operation quickly and uneventfully. On the evening of surgery she grasped my hand, and eyes brimming with tears, thanked me for removing the large mass.  She stated unequivocally she felt better than she had in months, and the sensations of heaviness, pressure, and discomfort had vanished.  She had minimal pain from her abdominal incision and was discharged from the hospital four days after the procedure.  When I saw the patient and several of her daughters back in the office for her first post-operative visit, they asked for more details about the tumor.  I reported, like a pediatrician would do with a newborn baby, the pathologist had weighed her tumor.  It came in at a whopping 2.3 kilograms, or just over 5 pounds! Despite the presence of stage IV disease with several remaining small liver metastases, this patient in all probability will live many more years thanks to the availability of the targeted drug imatinib.  The patient now has a slightly concave rather than a very protuberant abdomen, and she is able to eat full-sized meals and enjoy her children and grandchildren.

This lady’s tumor was big, but is not my personal record for largest tumor removed. I am certain I have colleagues who could easily describe tumors larger than the biggest one I ever resected.  Sarcomas in particular can grow to a relatively enormous size in many locations in the body.  Slightly more than a decade ago, I received a phone call from a panicked primary care physician from a small town in West Texas.  The physician rapidly and breathlessly explained a patient came into his office two days before stating he was unable to eat, his waist size had increased from 34 to a 52 inches in only a few months, and the perfunctory CT scan revealed a mass extending all the way from the liver down into the pelvis.

I immediately accepted the patient and told the physician to send him to my office the next day. This 50 something year old gentleman, who had been previously completely healthy and an active, hard working farmer, was clearly miserable.  His abdomen was distended to the point of looking like it was going to burst.  The abdominal skin was taut and shiny, and he was breathing over 30 times a minute because he was unable to take a deep breath from the increased pressure upward on his diaphragm and lungs.  The muscles of his arms, legs, and face were wasted.  He had a typical appearance of cachexia; severe malnutrition with his pleading eyes protruding from sunken sockets.

After speaking with this gentlemen and his family and completing a physical examination, I reviewed the CT images obtained in West Texas. I realized his massive tumor was originating from the edge of the right lower liver, extending down into his pelvis and compressing the stomach, all of the small and large intestine, and even causing compression of the bladder and ureters, the small tubular structures draining urine from the kidneys to the bladder.  Despite the relatively immense size of this tumor, it was clear it arose from the lower edge of the liver and was not near any of the major blood vessels flowing into the liver or draining blood out of the liver.

I scheduled this man for an operation only two days later because of his debilitating symptoms. I started the operation with a right subcostal incision, a cut in the skin under the ribs on the right side.  A quick assessment confirmed the tumor was arising from a small portion of the liver lateral to the gallbladder.  Intraoperative ultrasound confirmed there were no major blood vessels involved by the tumor.  It was possible to remove a small section of the liver from which the tumor arose, less than 10% of the total liver volume, and then detach the tumor from other abdominal organs.  The tumor was so large I had to extend the incision simply to remove it from the abdominal cavity.  To my surprise, the tumor had very few attachments to other organs or structures, and once its origin in the liver was divided, I was able to gently deliver the mass out of the peritoneal cavity and clamp and divide the few remaining blood vessels supplying the tumor.

This tumor was so large and somewhat gelatinous I was concerned I would drop it or rupture it. We did not have a basin or container large enough to transport the tumor from the operating room to the pathology suite.  Therefore, the circulating nurse brought a wheeled cart into the operating room usually used to transport sterile instruments and supplies from the surgical core area to the operating room.  We wrapped the tumor in sterile towels and I pushed it over to the pathology suite.  The wide-eyed expressions from the experienced senior pathologist and the surgical pathology staff were hilarious.  The pathologist took one glance as I uncovered the tumor and exclaimed, “Holy shit, what is that thing?”  Why does everyone keep calling these giant tumors “things”?

I showed the pathologist the origin of the tumor from the edge of the liver and returned to the operating room. The intestine and other structures, which had looked compacted, blue, and pathetic, were already pink and contracting wildly.  It reminded me of times when I found myself in a large, relatively immobile crowd of people, and the wonderful feeling of relief once I broke free from the crowd and could move unencumbered.  We had plenty of room to spare closing his abdominal wall muscles and the skin.

This gentleman was one of the happiest people I’ve ever seen on the morning of his first post-operative day, despite a long bilateral subcostal incision. He was already wolfing down breakfast of eggs, pancakes, and sausage he had gone to the cafeteria to purchase.  We had not yet written an order allowing him to drink even liquids.  Absence of a formal medical order permitting him to drink broth and juice or enjoy jell-o in any color he ordered was not going to stop him; he had room for his stomach to distend and his bowel to work and he was not going to wait around for us.  He explained he was making up for lost time and was going to eat whatever he wanted.  Fortunately, he suffered no ill effects from his rapid self-progression to a regular diet.  He was discharged from the hospital four days after the operation.  When I saw him back for his first post-operative visit five days later, we discussed the rare hepatic angiosarcoma I had removed.  I informed this man and his family I had delivered a 45 cm greatest diameter tumor, almost 18 inches in size.  The final weight of the cancer emanating from his liver was 6.5 kilograms, or slightly greater than 14 pounds.  The pathologist admitted the total tumor weight was probably greater because when he cut into it a large amount of fluid and old blood drained from the tumor.  I estimate after lifting it from the patient’s abdominal cavity to the back table in the operating room it seemed like at least 20 pounds to me.

Sarcomas are unusual and uncommon cancers with a propensity to recur locally or metastasize to the liver and lungs. Individuals who are experts in sarcoma surgery will say sarcoma patients can train new surgeons for many years because of the numerous operations they may require for multiple recurrences.  In general, chemotherapy is not particularly effective for types of sarcoma other than GISTs.  This gentleman did well for almost four years before he developed recurrent tumors in his peritoneal cavity.  I operated and removed these intra-abdominal tumors, and he did well for another two years before he developed a few lung metastases.  These pulmonary tumors were removed during two operations over the next year; but the patient ultimately succumbed to multiple new tumors growing in his lungs.  Near the end of his life, he declined chemotherapy treatments when he learned the low probability of success in shrinking the tumors and the profoundly toxic side effects.  He did achieve over six years of what he proclaimed, “A really great life.”  He was upbeat, energetic, ate normally, ran his farm, loved to tell jokes, and travelled extensively with his wife and family.  He told me the experience with cancer taught him to enjoy every day he was granted.  After the initial huge tumor was removed, he was quickly able to eat and exercise and return to his normal appearance and level of activity.

Biology is remarkable. The complexity of the human body is difficult to comprehend.  There is much we still do not know or fully understand.  The development of cancer is an equally complicated process with a mind-boggling interplay of genetics, epigenetics, biochemistry, immunology, and dynamic interactions with the host.  The occasional patient we see with massive tumors represents an eye-opening experience for all involved; it is difficult to believe how large a tumor can grow to be in some locations in a human body before causing symptoms leading to detection.

When the inevitable question is asked by patients and family members after I remove a very large tumor, the answer is, “Yes, incredibly enough, that ‘thing’ was really growing inside you.”

And as amazing and wonderful as our primary senses are, I cannot explain why a tumor can grow to a massive size before we sense something is awry.

 

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