(April 17, 2009 - Insidermedicine) At the American Society of Hematology meeting in San Francisco, we spoke with Dr. Jonathan Friedberg, who is Associate Professor and Chief, Department of Medicine at the University of Rochester School of Medicine. Dr. Friedberg is also Associate Professor at the James P. Wilmot Cancer Center.
What is mediastinal lymphoma?
Dr. Friedberg: Mediastinal lymphoma can be a very aggressive disease, and when it presents with symptoms, I think that there is urgency to making a diagnosis. Often the diagnosis is made by a surgeon who does a biospsy. In this case, if there's a fullness or mass that can be easily biopsied, that's what I would pursue first.
What does the physical examination consist of?
Dr. Friedberg: Basically, the surgeon would try to determine the best biopsy site and presumably, the biopsy would confirm the diagnosis of mediastinal lymphoma. Then, the patient would visit a medical oncologist or hematologist with expertise in treating these lymphomas. And those type of questions would involve what type of symptoms the patient might have including cough, shortness of breath, any swelling of the face-in this case, the hoarse voice is present, and then other symptoms include fever, chills weight loss, night sweats and skin rash. Following that evaluation, there would be scans performed to determine exactly the extent of this disease, which is often localized to the chest. A bone marrow biopsy might be performed as well.
Are there any other relevant diagnostic tests?
Dr. Friedberg: In this case, there needs to be an urgency to these tests because sometimes blood flow in the chest can be obstructed or blocked by these large mediastinal masses. And if that's present, symptoms can be significant. There may be some FLOW studies or fancy ultrasound studies to try to explore that. The most important thing is to establish a diagnosis and start therapy very quickly.
What are the therapeutic options?
Dr. Friedberg: Although radiation may be a part of therapy, it's generally reserved for the end of treatment. These diseases tend respond very well to chemotherapy. Generally, you want to start chemotherapy relatively urgently to minimize progression of the symptoms.
Are there any risks associated with treatment?
Dr. Friedberg: The chemotherapy and antibody treatments that are used for mediastinal lymphoma, in general, are outpatient regimens that are tolerated reasonably well. I sort of categorize side effects into annoying side effects and serious side effects. Annoying side effects include hair loss, nausea (which is usually controlled), constipation, and some numbness and tingling in the fingertips or toes. More serious side effects we see with these regimens include blood count problems that can lead to infection, and organ damage. These side effects are generally rare.
Is there a genetic component to the disease?
Dr. Friedberg: This does not appear to be an inherited disease. It's a very uncommon disease--probably less than 1% of all Non-Hodgkins lymphomas, and NHL in general are less common than the other major cancers. This is an important disease to think about though because it often affects younger people, often women in their 20s and 30s. So because it's a curable disease, it's important that it's recognized quickly and dealt with by an expert.
If I had mediastinal lymphoma...
Dr. Friedberg: If I had hoarseness of the voice, difficulty swallowing, and fullness above the collarbone and was diagnosed with mediastinal lymphoma, I would want an urgent evaluation by a hematologist or a medical oncologist with expertise in treating that disease. I would want a rapid institution of the appropriate tests and then to begin chemotherapy as quickly as possible, and then look forward to a reasonable cure rate with chemotherapy, antibody treatment and the possible addition of radiation therapy.