Rare Disease Day - An Opportunity to Educate and Advocate

by Jane Ashley

Rare Disease Day, the last day of February every year, is a day to raise awareness of rare diseases throughout the world. We, at WhatNext.com, hope to raise awareness of rare types of cancer on this day.

Rare Disease Day

What is the definition of a rare disease?

In the United States, a disease is considered rare if it affects less than 200,000 people at any one time. Europeans define a rare disease as one that affects less than 1 in every 2,000 people. Worldwide, there are at least 6,000 rare diseases.

Genetic mutations cause about 80 percent of rare diseases. Most of the rest are the result of bacterial or viral infections, allergies or degenerative. Sadly, about half of the rare diseases affect children.

Rare diseases are sometimes misdiagnosed because their symptoms mimic common diseases. Symptoms of rare diseases vary from patient to patient. Many physicians may only encounter one patient in their lifetime with a rare disease.

Estimates suggest that as many of 25 to 30 million people suffer from a rare disease. Individual cases of a particular rare disease are low, but the combined total of people with rare diseases is high.

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Is cancer considered a rare disease?

Yes. According to the National Cancer Institute, a cancer is rare when diagnosed in less than 15 people per 100,000 per year. According to the American Cancer Society, a cancer is rare if fewer than six people in every 100,000 people are diagnosed each year.

So what does this translate into for a country as large as the United States? The population of the U.S. in 2018 was about 327 million. Divide 327 million by 100,000, and we have 3,270. Multiply 3,270 by 15 cases per 100,000, and we arrive at 49,050 people diagnosed in one year meets the criteria of being a rare disease. If we use the American Cancer Society’s more strict definition, then a rare cancer is one where about 19,500 are diagnosed annually.

What this actually means is that many of the cancers that we know about are rare. Let’s look at a list of cancer types that qualify as rare, according to the United States definition. Generally speaking, experts in the U.S. consider any cancer with fewer than 40,000 people diagnosed annually as rare.

Leukemia. There are about 60,000 total cases every year. But even the most common types, chronic lymphocytic leukemia (CLL) and acute myeloid leukemia (AML), are rare with around 20,000 cases per type.

Stomach. Just over 26,000 cases occur annually.

Ovarian. We hear lots about ovarian cancer, but only about 22,000 cases are diagnosed annually in the U.S.

Brain and nervous system. Not quite 24,000 people develop this catastrophic cancer annually.

Hodgkin lymphoma. Only 10 percent of lymphomas are Hodgkin lymphoma (about 8,500 cases annually) compared to Non-Hodgkin lymphoma . Most Hodgkin lymphomas occur in younger people, ages 15-24 or in people over 60. Non-Hodgkin occurs in all ages.

All of us here at WhatNext.com learn from each other. We see your diagnosis, and we hear your questions. So we begin to feel comfortable hearing your diagnosis. It becomes “familiar” to us. But many of the cancers that we know about are rare.

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Rarer cancers present many challenges.

Harder to diagnose because doctors see fewer patients with those unique symptoms.

Might be diagnosed at a later stage, because they are less common.

Fewer treatment options.

Less research.

Few clinical trials.

What are some of the rarest cancers in adults?

The American Cancer Society compiled a Rare Cancers in Adults: Special Section. This gem of cancer research confirms my belief that cancer never picks a convenient place to occur. Here are some of the unusual places that we humans experience cancer.

Oral Cavity Cancers. This part of the body is home to some rare cancers including the floor of mouth, hypopharynx, lip, nasopharynx, and oropharynx – all of these rare cancers occur at less than one person per 100,000. Other rare oral cancers include tonsil and salivary gland.

Digestive System. Less than one per 100,000 cancers are in the mesentery, omentum, peritoneum, and retroperitoneum. Other rare cancers in the digestive system include gallbladder, anal and small intestine.

Respiratory System. Rare locations in the respiratory system include the middle ear, nasal cavity and trachea.

Genitourinary System. Ureter and vaginal cancers are the rarest forms —less than 1 case per 100,000 persons. Cancer of the penis is rare as is cancer of the vulva. Testicular cancer is uncommon, occurring in only 7.2 men per 100,000.

Other Rare Cancers. Heart (Mayo reports seeing 1 case annually), Kaposi sarcoma, primary bone cancer, eye and the orbit of the eye, male breast cancer, and mesothelioma.

Many of these rare cancers are treatable and have encouraging five-year survival rates. Lip, salivary gland, eye, vulva, testicular and male breast cancer all show 5-year survival rates of 80 percent or more.

Rare cancers that have low survival rates are those that don’t have obvious symptoms until the disease is already advanced. These cancers include gallbladder and mesothelioma.

Even rarer cancers, occurring only once in a million people, exist. Mesenchymal chondrosarcoma is so rare — just 1,000 cases since 1959. Another uncommon cancer is adrenal cancer. Epithelioid sarcoma only occurs once in every 2.5 million people – it usually begins as foot pain.

Share your story — share your symptoms. Rare Disease Day brings attention to rare, uncommon diseases, including rare kinds of cancer.

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