It’s Thursday evening in Paoua. It started raining hard before sunrise and continued until about 10am today. The sky remains overcast and the geography of the potholes and creases in the road from the runoff is all new.
Last Friday I was asked to see a nine-year-old girl with a mass in her abdomen. She had been seen a few months earlier in the clinic and was thought to have a swollen liver and spleen due to a parasitic infection called schistosomiasis. She had received treatment for that disease but the mass continued to enlarge. She was now in discomfort all the time and unable to eat much. When I examined Francoise, she was skinny even by local standards with arms and legs like sticks, and thin sticks at that. She stood up and her belly protruded like a pregnant woman’s. The mass was in her mid left torso, the 'left upper quadrant' in medical terminology. I could feel its texture through her tense skin and thinned abdominal wall; it was hard as a rock, rough and knobby in texture. It felt like a cancer.
At home, I only see patients this age for appendicitis or the infrequent trauma. I know there are only a handful of causes for a mass like this in a nine-year-old but off the top of my head I can only think of two, Wilms tumor which starts in the kidney and lymphoma. I remember that the current best treatment for a Wilms tumor of this size involves chemotherapy although there was a time when surgery was the first line treatment. We discuss whether this could be related to malaria which is endemic in this region and can cause swelling of the spleen, but she isn’t having fevers and her malaria test is negative. The handful of other tests that are available (creatinine to measure the kidney function, a single test called ALT to evaluate the liver and an x-ray of the abdomen) are normal except for a mildly low red blood cell count. The fact is that this looks like a tumor, growing progressively larger over a period of several months and causing symptoms primarily related to size and pressure on the rest of her organs. I tell the patient, her father and her doctor, an English/French internist working with MSF for a six month period, that I need to do some research but will get back to them in the afternoon.
I am thankful for the Internet connection at the office. I learn that Wilms tumor and lymphoma are the number two and three candidates but I’ve forgotten about neuroblastoma, the most common tumor that might show up in a nine-year-old girl’s belly. I read about the treatment of neuroblastoma plus the treatment of Wilm’s, abdominal lymphoma and a few other dark horse candidates. I also research so-called tropical splenomegaly syndrome related to malaria to be sure that this isn’t her problem and confirm that she would have obvious and active malaria plus the mass would be smooth and congested, not rock hard. At the end of my research, this is what I know:
- This is almost certainly one of the top three; a neuroblastoma arising from the adrenal gland or the chain of nerves imbedded near the long muscles running by the spine, a Wilms tumor arising from the left kidney or a lymphoma arising from a lymph node deep in the abdomen.
- If the tumor is a neuroblastoma or a Wilm’s tumor, and it can be removed in its entirety without cutting into the tumor, surgery alone offers her a chance to be cured long term though only maybe somewhere between a 20 percent and 50 percent chance.
- If the tumor is a lymphoma, an operation has nothing much to offer her except a painful incision and the risk of complications.
- If she was in the developing world, we would start with a battery of blood tests and CT or MRI scans. Using these we could have a better idea what her tumor was (and be sure it was a tumor), then treat her optimally with chemotherapy and or radiation to shrink the tumor before an operation, or chemotherapy alone if it was lymphoma. With optimal treatment including chemotherapy, radiation and surgery, depending on the exact diagnosis and the state of some very specialized pathology tests on the mass called 'tumor markers', she might have a chance of cure as high as 90 percent.
My father was a general surgeon in the US. I think we were both surprised when I decided to go to medical school five years after my college graduation (I certainly was), maybe a little less surprised when I chose to go into general surgery. He once told me that the surgical personality is someone who can make a timely decision with a limited amount of information and take action based on that decision. His first few decades of practice spanned the 1960s and 1970s before the advent of ultrasounds, CAT scans, MRIs and sophisticated tests for tumor markers in the US when a decision to operate was primarily based on history, physical exam and knowledge, much the same circumstances for decision making that we have here in Paoua today. There are no more tests to be ordered, no new information on the horizon that will help me know if an operation can help Francoise or only hurt her.
I returned to the hospital. Wanting to be sure that my initial impression was right, I re-examined her one more time before talking to her doctor. It still felt like a tumor. I described my research and my thought process to the other doctor, a native French speaker, who translated it to the father. With surgery there is a chance we can remove this tumor and either cure her or improve her symptoms for now, though even if we remove the entire tumor and “cure” her, there is a chance that the cancer could come back. Although surgery has its risks and recovery will be difficult for her, without treatment given the growth of the tumor over the past few months I doubt that she will survive one year, if that.
We operated the next day, Saturday. She was given a general anesthetic with intubation and I began with a small incision to check for signs of tumor spread. If we found any, surgery would not have anything to offer her and there would be no reason to subject her to a larger incision. Everything looked clear so I made the incision longer, from just below her breastbone past her belly button until I was just an inch from the pubic bone. What we found was a large, hard mass taking up more than one quarter of her abdominal cavity. In the upper abdomen, the tumor had invaded into her spleen, pushing it forward. There was also some direct invasion of the tumor into the first part of the stomach and the left lobe of the liver. In the lower abdomen, the tumor was pushing through the mesentery of the large intestine (the mesentery is a layer of fatty tissue that contains the blood supply and lymph node drainage of the intestine) but it appeared that the larger blood vessels to the colon (another name for the large intestine) and the colon itself were simply draped against the tumor but not encased.
So far so good; every organ or part of an organ that was involved with the tumor could be taken out attached to the tumor for a so-called 'en bloc' resection. My main concern now was whether the tumor invaded the blood vessels toward the spine, either the aorta or the main arteries and veins feeding the entire small intestine (the SMA and SMV). If the tumor invaded these vessels, it would be unresectable today. After undraping the colon off the tumor though leaving the involved part of the mesentery attached to the tumor, we tediously worked our way around the left and behind the tumor. At one point I thought I felt a second tumor deep and next to the spine but it was her left kidney, pushed down and toward the mid-line, kinked almost 90 degrees.
The kidney was not involved with the tumor and I had not come across any abnormally large lymph nodes so this was almost certainly a neuroblastoma. It was a relief to finally see that the aorta, SMA and SMV were free as well though the second half of the pancreas was embedded as deeply in the back of the mass and would have to be included in the resection. In short order we divided the attachments of the tumor in the back area where it had originated, divided the pancreas and vessels to the spleen, divided the liver two centimetres from the area of tumor invasion, did the same for the stomach and took her tumor out; a successful 'en bloc' resection. It took another 30 minutes to stop the bleeding from the cut surface of the liver, sew closed the stomach wall and the defect in the mesentery to the large intestine, suture the end of the divided pancreas to minimize the risk of leaking pancreatic juices, place a drain just in case any pancreatic juice did leak out, then sew her abdomen closed and we were done. The entire operation took almost two and a half hours.
As far as I can tell, there is little funding for cancer care in the CAR and none in Paoua. This may not be unreasonable in a place where malaria is the leading cause of death in children under the age of five but there isn’t even consistent availability of effective anti-malarial drugs, where the second leading cause of death in children is due to something as simple as diarrhoea and where the overall life expectancy is only 48 years. But in my short two weeks here, I’ve already seen four patients with cancer (A man with a recurrent sarcoma in his biceps that I removed on Tuesday, another man with a “benign” but unresectable tumor that began on his nose and over the years has invaded his eye socket on the right and now is impinging on nerves deep in his skull, an eight-year-old girl with probable Burkitt’s lymphoma plus Francoise) but I've only been able to help two of them.
The girl with Burkitt’s lymphoma might have a chance of cure as high as 90 percent if chemotherapy was available for her, but surgery has nothing to offer her and her life will be measured in months here. At the policy level, there is a fine line between accepting the realities of the situation and an inappropriate fatalism about the hopelessness of treating some diseases and instead focusing on others. I have the luxury of not being a policy maker who has to make the hard decision about which program to fund first and which program to not fund at all. I only need to decide whether I have any treatment to offer that will help the patient in front of me, and if not to bear witness to the ones I can’t help.
Francoise has done well since her surgery five days ago. She is up walking, drinking liquids without difficulty and we think her pain is less than before surgery. The drain remains in place, coming out the left side of her abdominal wall into a bag, but little fluid is coming out now; as a precaution I won’t remove the drain until after she is eating solid food and just before she leaves the hospital. Because we don’t have pathology here to examine the tumor, we will never know for sure what type it was or be able to check tumor markers to predict the likelihood that the tumor might come back. There will be no opportunity to give her post-operative chemotherapy or radiation therapy to 'mop up' any few remaining tumor cells; her own immune system will have to do the job. I will leave Paoua in October and likely never find out whether her cancer comes back in six months or two years or if it never comes back and Francoise grows into adulthood and leads a long and happy life. I’ll keep my fingers crossed for the latter.