Understanding the Zebras of the Cancer World

If you heard hoofbeats, what animal would you think of? A horse, right? It's the most obvious answer. Unfortunately, in the medical world, not all diagnoses are horses or the most likely possibility, and sometimes physicians need to look for the zebra — the less likely scenario when making a diagnosis. In the cancer world, neuroendocrine tumors are the zebras. Represented by this analogy because of their rarity, neuroendocrine tumors make up just 2% of nationally treated cancers.

Neuroendocrine tumors can originate anywhere within the body and are difficult to detect, often remaining undiagnosed for quite some time. They tend to be slow-growing, and patients often don't realize the tumor is present because symptoms are most often mistaken for signs of other ailments. For example, an individual may develop abdominal pains from a bowel obstruction caused by a neuroendocrine mass and be treated for the gastrointestinal symptoms, while the tumor remains unnoticed and untreated.

Patients who have suffered through such a dilemma will come to us down the line and ask: Why did my doctor not diagnosis this sooner? The answer: it's an extremely rare cancer.

Here at Roswell Park Comprehensive Cancer Center, we have a dedicated, multidisciplinary team that focuses on treating these tumors. We know what to look for and what testing to do. We actually are able to help patients live a long time– sometimes decades –even patients with late-stage neuroendocrine cancers. Advances in a variety of fields have helped us to understand these cancers much more. We can now create long-term treatment plans that offer a smooth and healthy recovery.

The goal of treatment is two-fold. The first is locating the tumor and removing as much as possible. The second is identifying and controlling what hormonal problems the cancer could cause. These tumors have their roots in hormonal changes, so managing these problems successfully is critical in treatment.

Regrettably, despite years of research, there exists no textbook remedy for managing these cancers. Our staff's experience in treatment and knowledge of emerging new and better treatments has allowed us to succeed in combating these rare and elusive tumors. We are very proud that a large group of us at Roswell Park has remained dedicated to understanding these tumors and being aware of and involved in leading new clinical trials and research of neuroendocrine cancers.

Like all cancers, especially in current times, addressing neuroendocrine cancers is really a journey from being a patient to being a survivor — becoming someone who has taken their cancer and learned to live with it, run with it, and make the most of life while fighting. We are here to help anyone become that kind of survivor, and I believe our patients appreciate it quite a bit. It is something I am honored to be a part of.

Update for World NET Cancer Day, November 10, 2020:

This has been a challenging year for all, and especially the neuroendocrine tumor (NET) community, and like other gatherings, our annual NET patient and caregiver event could not take place, but I'd like to take the chance to update everyone on a few important things going on with Roswell Park's NET programs and patients.

A new PET scan, called DETECTNET, was approved this summer. We have two new exciting clinical trials, for Survivin and NALIRI for high-grade NETs, are now open and enrolling patients. We also have more than 100 patients currently enrolled in the NET biobank.

Our PRRT program has grown, and various new therapies and combinations are under development that we will offer in the coming months. We also had two new studies published about emerging treatment options for neuroendocrine tumors.

Lastly, I wanted to share a note to all NET survivors from one of our young patients, Justin H:

"It's been a year since I went on the clinical trial SurVaxM and I am feeling great! Throughout the trial and now at the completion, my tumors have remained neutral and it has kept me from having to go through another bland embolism procedure while helping me live the life I want to live, so to me that is a huge success.

“Part of that success, I believe, was by putting a focus on my mental and physical health. With all that has been going on with COVID, along with being on a clinical trial medicine, I knew I needed to keep my immune system running at 100%. I focused on dialing in my diet to what worked with my stomach and took vitamins to help boost my immune system. Along with eating right, I spent a lot of time training at my home climbing gym, which helped alleviate a lot of anxieties and stresses I had being on this trial and also by how carcinoid syndrome affects me.

"I know that the thought of going on a clinical trial can be a scary thought and so can deciding to take the next step with any type of treatment. I was nervous trying this one, but I trust in the doctors and nurses that look over me and believed this was something worth trying. So I encourage you to be open to the idea of trying a clinical trial if your doctor recommends it as an option for you. To me, it wasn't just a way to help me but also a way to help those in the same situation in years to come."

Roswell Park NET Biobank

The Roswell Park NET Biobank is a unique resource dedicated to advancing the field of neuroendocrine tumor management through molecular and genetic research.

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