(May 6, 2009 - Insidermedicine) In this video, Dr. Owen O'Connor, MD, PhD, Director of the Lymphoid Development and Malignancy Program at Herbert Irving Comprehensive Cancer Center, discusses what he would do if he had a child with a rapidly growing jaw mass and suspected Burkitt's Lymphoma. Dr. O'Connor, Associate Professor of Medicine at Columbia University, is also Chief of the Lymphoma Service in the College of Physicians & Surgeons, Columbia University/New York Presbyterian Hospital.
At the annual American Society of Hematology meeting in San Francisco, we had a chance to speak to Dr. Owen O'Connor, MD, PhD, who is Associate Professor of Medicine and Director of the Lymphoid Development and Malignancy Program at Herbert Irving Comprehensive Cancer Center at Columbia University. Dr. O'Connor is also Chief of the Lymphoma service at the College of Physicians and Surgeons, Columbia University New York Presbyterian Hospital.
If I had a child with a rapidly-growing jaw mass...
Dr. O'Connor: So, if I had a child with a rapidly-growing jaw mass, I would be very prompt about bringing them to my local physician or their pediatrician very immediately. It would be very important for the physician or the parent to try to document or identify just how fast that lump is growing, because the symptom of a rapidly-growing lump can be concerning for a number of aggressive diseases, including Burkitt's lymphoma, which happens to be one of the fastest growing malignancies known to science.
What should I expect in terms of evaluation?
Dr. O'Connor: Your doctor is likely to spend a lot of time trying to understand the temporal relatedness, or temporal sequence of events, in terms of how fast the lesion is growing and what other symptoms you might have. He or she might ask you if you've been tired, if you've been losing weight, not eating or drinking, or about the development of any new pain syndromes. The first thing they're trying to do is understand your spectrum of symptoms to try to help them get a handle on what the extent of your disease might be.
What initial tests need to be ordered?
Dr. O'Connor: The first thing the physician might do is a physical examination, to try to determine if there's any new lesions elsewhere in the body. The first test to do would be to try to obtain some radiographic imaging studies, in the form of CAT scans. These studies are able to image the anatomy in your chest and abdomen, and will provide important information about the development of other masses in the body, or enlargement of your lymph nodes. It's conceivable that they may also order a test called a PET scan, which measures the metabolic activity of various cancers, and can be informative about finding the disease in other parts of the body, and may be more sensitive than CAT scans.
What kind of tests will confirm the diagnosis?
Dr. O'Connor: The most important test that your physician will need to do immediately will be to obtain a biopsy, and that's usually done by a surgeon or other physician depending upon the location of the mass. That biopsy can take any of a number of different flavors: it can be an aspiration of cells with a needle, it may include a small core needle to obtain a piece of the tissue, or it may require an open surgical procedure, where the surgeon needs to make a small incision over the tumor, and then surgically excise a small piece of the tumor, which would then be sent to the pathologist for reading and eventual determination of the underlying diagnosis.
What are the treatment options?
Dr. O'Connor: The treatment for Burkitt's lymphoma tends to involve complex combination chemotherapy, and fortunately, this is a disease that's highly-curable. Perhaps the most important recommendation for any child diagnosed with a rare disease like Burkitt's is to try to find yourself a medical oncologist who is very familiar with the disease and very familiar with the treatment of Burkitt's lymphoma. Most patients believe that, when they're diagnosed with rare diseases, it doesn't matter where they're treated initially, but it does matter in this particular case because Burkitt's lymphoma is highly curable when patients initially present, but tends to be much less curable after you relapse. So, you want to be sure that you take full advantage of the available treatment options and enhance your chances of cure with the initial treatment.
What are the outcomes of a natural history patient vs. a treated one?
Dr. O'Connor: The natural history of a patient with newly-diagnosed Burkitt's lymphoma tends to involve complaints revolving around the rapid development and appearance of a mass somewhere in the body. It could be in the neck or in the armpits, the groin or even a palpable mass in the abdomen. Typically, physicians can control the rapid growth of the tumor with simple chemotherapy options, especially if the rapid growth of the tumor is associated with uncomfortable symptoms. For patients who developed relapsed Burkitt's lymphoma, it tends to present very similarly, however relapsed Burkitt's lymphoma does not respond to chemotherapy at the same probability or in the same way that initially diagnosed Burkitt's lymphoma responds. At that point, there's very little consensus on how best to treat patients with relapsed Burkitt's lymphoma, but that's also another situation that should prompt you to consult with an oncologist specializing in the treatment of this rare disorder.
If I have Burkitt's, is there a risk that my family members will, as well?
Dr. O'Connor: For those patients who are diagnosed with Burkitt's lymphoma, there does not appear to be an increased risk amongst family members or siblings.
Is there a relationship between Burkitt's and Epstein-Barr Virus (EBV)?
Dr. O'Connor: Burkitt's lymphoma is a disease that is relatively rare in the United States, and it is found commonly in certain parts of Africa. We know that the disease in Africa is associated with infection by a virus known as Epstein-Barr Virus (EBV). That seems to be a very common association for patients who develop Burkitt's lymphoma in Africa, and in fact, there's a form of the disease known as Endemic Burkitt's lymphoma that's largely based on infection with this particular virus.
In North America, that does not appear to be the case. EBV may be found in some of these diseases, but it doesn't seem to have the same causal relationships to the development of the disease as it has in Endemic Burkitt's lymphoma in Africa.
Dr. O'Connor: If I had a child with jaw swelling, I would want to get to my pediatrician as quickly as possible, especially if the mass that was swelling in the jaw was happening at a very rapid pace. At that point, I would want to convey as much detail as I could to my pediatrician about the details of that tumor's growth, and how the child is feeling with that particular disease. I.e., Does the Child have any new complaints of pain, fever, chills or sweating? Following that, I would probably want to be referred to a good pediatric oncologist who specialized in the treatment of these rare hematologic blood disorders, and, at that point, get a CAT scan or PET scan to try to evaluate the extent of the disease.
Following a workup that involves imaging, I would want to get a biopsy from a surgeon who would obtain a piece of that particular tissue, which would then be forwarded to the pathologist, who would evaluate and make the diagnosis.
Following that, I would want my child to receive chemotherapy for the treatment of the disease, and fortunately, the chemotherapies we have for this disease are highly-effective and curative in the overwhelming majority of cases. I would want to be sure that I reported to my treating physician any new symptoms or complaints while in chemotherapy, especially given the higher risk of infection and perhaps some bleeding that can develop when you're on that therapy.
Assuming (my child) is cured of that disease, (he or she) would continue to be followed by a pediatric oncologist on a fairly regular basis. That might initially include visits with him or her every month or two months with some CAT scans at roughly every three or four months, at least for one to two years to make sure that the disease is gone.