Zebras on the Lawn: Uncommon Cancers in the Community

Derek Raghavan, MD, PhD; Janis C. Kelly


June 28, 2013

In This Article

What to Do if a Zebra Enters Your Office

Editor's Note: Derek Raghavan, MD, PhD, is an internationally renowned cancer researcher and senior editor of Textbook of Uncommon Cancer.[1] On the publication of the fourth edition of this landmark textbook, Dr. Raghavan talked with Medscape about recent advances in clinical management of the uncommon cancers and about the challenges that remain.

Medscape: What are the main challenges for the community oncologist when treating a patient with an uncommon cancer?

Dr. Raghavan: The community oncologist has been in something of a predicament when faced with an uncommon cancer. The first problem is that usually the person describing the tumor will be a general pathologist, not a rare tumors expert. The first step for the community oncologist should be to order a pathology review by a pathologist who has expertise in the suspected uncommon tumor type.

The community oncologist or the academic oncologist who has not seen a case of the suspected rare cancer needs to put his ego in the trash and consult one who has more expertise. If you happen not to be an expert in a particular tumor, find an expert.

Medscape: What kinds of mistakes might be made?

Dr. Raghavan: For example, we have on several occasions seen what were purportedly neuroendocrine small cell prostate cancers but were actually poorly differentiated adenocarcinomas. The pathologist was doing the best he could but just did not have the experience. So, without an expert pathology review, the community oncologist would be trying to treat the wrong disease.

Dr. Derek Raghavan

Medscape: How should a community oncologist go about finding an expert for consultation?

Dr. Raghavan: Look for names of expert panelists and presenters with expertise on uncommon cancers at national meetings such as the American Society of Clinical Oncology or the American Association for Cancer Research. Use PubMed to find someone who has published on the topic extensively. Without meaning to be self-serving, our textbook has focused on experts for many of the uncommon cancers and thus is a source of whom to consult. Look for an expert subspecialist in the tumor type in question.

Medscape: Are online resources helpful for the rare tumor case?

Dr. Raghavan: There is not currently a strong online resource for uncommon cancers. There is a welter of isolated case reports of unknown accuracy. A better approach is to use the textbook as a basic resource, then go online and see if there are any repeated case reports. If there are 8 or 10 case reports for unusual melanomas treated with the BRAF inhibitors, you could reasonably say that that seems to be a pattern, and this may give the clinician some options for treatment. Personally, I prefer using peer-reviewed papers.

You could then call a melanoma center and say, "I've read the chapter, I've looked up BRAF inhibitor reports online, and it looks like they may have a role in this case. Expert, do you agree that that's a reasonable thing to do?"


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