Rare types of cancer, such as chronic myeloid leukemia and Ewing sarcoma, affect fewer than 15 people per 100,000 each year. Rare cancers can affect any part of the body and have different risk factors, symptoms, and outlooks.
You may be familiar with some of the most common types of cancer. According to the
However, some cancers are much rarer. The
Many cancers are rare. In this article, we touch on just a few of the rarest types of cancer that you may or may not have heard of.
Language matters
You’ll notice that the language used to share stats and other data points is pretty binary, fluctuating between the use of “male” and “female” or “men” and “women.”
Although we typically avoid language like this, specificity is key when reporting on research participants and clinical findings.
Unfortunately, the studies and surveys referenced in this article didn’t report data on, or include, participants who were transgender, nonbinary, gender nonconforming, genderqueer, agender, or genderless.
Adrenal cortex carcinoma (ACC) affects the outer part of the adrenal gland, which makes hormones like cortisol. ACC is very rare,
ACC can develop at any time, but mostly occurs in middle aged adults. People with certain inherited conditions like Li-Fraumeni syndrome and Beckwith-Wiedemann syndrome are at a higher risk.
ACC tumors can produce hormones in excess. As such, the symptoms of this cancer are often related to those of an excess of cortisol or sex hormones like estrogen or testosterone.
A 2022 study that analyzed survival rates in 47 people with ACC found that the median overall survival was 3 to 4 years. Survival rates were reduced to 15 months for those with advanced or metastatic ACC.
The table below shows the
| Stage of cancer | 5-year relative survival rate |
|---|---|
| Localized | 73% |
| Regional | 53% |
| Distant | 38% |
| Overall | 50% |
About 5-year survival rates
A 5-year survival rate is the percentage of people with a certain type and stage of cancer that are alive 5 years after diagnosis.
The
- Localized means that the cancer is still limited to the organ or tissue it started in.
- Regional is when the cancer has spread to nearby tissues or lymph nodes.
- Distant means that the cancer has spread to other parts of the body, such as the lungs, bones, or liver. This is typically referred to as metastatic or stage 4 cancer.
The 5-year survival rates are only statistics. They don’t account for individual factors like age and overall health. They also don’t always reflect recent advances in cancer treatment.
Chronic myeloid leukemia (CML) is a slow growing type of leukemia that affects myeloid stem cells that eventually become blood cells like neutrophils.
Even though it’s one of the four main types of leukemia, CML is rare. In 2018, it was estimated to occur in 1 out of 100,000 people globally.
The risk of CML increases with age, and it’s also more common in males. Previous exposure to radiation is the only other known risk factor.
Symptoms of CML can include:
- weakness and fatigue
- fever
- night sweats
- unintentional weight loss
- bone pain
- enlarged spleen, which can cause a feeling of fullness on your left side below your ribs
The overall
Ewing sarcoma is an aggressive type of bone cancer that mainly affects children and adolescents. Despite being the
Most cases of Ewing sarcoma are diagnosed in adolescents. This cancer is also slightly more common in males.
The symptoms of Ewing sarcoma can include pain and swelling in the area of the tumor. Other more general symptoms can include fatigue, fever, and unintentional weight loss.
The table below shows the
| Stage of cancer | 5-year relative survival rate |
|---|---|
| Localized | 82% |
| Regional | 71% |
| Distant | 39% |
| Overall | 63% |
Eye cancer is any type of cancer that begins in the eye. Ocular melanoma is the most common type of eye cancer in adults, but only affects
- non-Hodgkin’s lymphoma of the eye
- retinoblastoma (which we’ll discuss later)
medulloepithelioma
The risks for eye cancer can depend on the specific type of cancer. For example, some risk factors for ocular melanoma include older age, a family history of melanoma, and having lighter colored skin or eyes.
General symptoms of eye cancer can include things like:
- eye redness
- eye pain
- vision changes like blurry vision or eye floaters
- reduced vision or vision loss
- changes to how your eye looks, such as a dark spot on your iris or white pupils
Survival rates for eye cancer can vary based on the type. The table below shows the
| Stage of cancer | 5-year relative survival rate |
|---|---|
| Localized | 85% |
| Regional | 67% |
| Distant | 16% |
| Overall | 81% |
Gallbladder cancer starts in the gallbladder, which stores bile used for digestion. In the
Some of the known risk factors for gallbladder cancer include:
- older age
- being female
- a family history of gallbladder cancer
- a history of gallstones
- other gallbladder or bile duct conditions like:
- obesity
The symptoms of gallbladder cancer can include:
- abdominal pain
- nausea and vomiting
- yellowing of the skin and eyes (jaundice)
- reduced appetite
- unintentional weight loss
- an abdominal lump that you can feel
The table below shows the
| Stage of cancer | 5-year relative survival rate |
|---|---|
| Localized | 69% |
| Regional | 28% |
| Distant | 3% |
| Overall | 20% |
Hairy cell leukemia (HCL) is a type of slow growing leukemia that affects B cells, which are immune cells that make antibodies. In HCL, the affected cells appear “hairy” under a microscope.
HCL is more common in men and is
People with HCL typically have low blood counts, which can lead to anemia, an increased risk of infection, and easy bruising or bleeding. The spleen may also become enlarged, leading to abdominal swelling and pain.
When it’s treated, the 5-year event-free survival for HCL is about 90%. Some
Hepatoblastoma is a type of liver cancer that mainly affects children. Despite being the most common type of liver cancer in this age group, it’s still very rare. Hepatoblastoma affects
While many cases of hepatoblastoma happen randomly,
Symptoms of hepatoblastoma can include:
- an abdominal lump that can be painful and continues to get larger
- abdominal swelling
- reduced appetite
- unintentional weight loss
- nausea and vomiting
Kaposi sarcoma (KS) starts in cells that line your blood vessels. It’s caused by infection with a virus called Kaposi sarcoma-associated herpesvirus (KSHV). KS affects
Throughout the world, there are
The symptoms of KS can include:
- skin lesions, which:
- most often occur on the lower body and face, may be raised or flat, and are typically red, brown, or purple in color
- can cause pain and swelling
- mouth or throat lesions
- lesions inside the body that may bleed or interfere with the functions of affected organs and tissues
The table below shows the
| Stage of cancer | 5-year relative survival rate |
|---|---|
| Localized | 81% |
| Regional | 65% |
| Distant | 47% |
| Overall | 75% |
Lip cancer is type of oral cancer that starts on the lips. In the United States, it affects
Some of the risk factors for oral cancers like lip cancer are:
- older age
- being male
- tobacco use
- heavy alcohol use
- exposure to ultraviolet (UV) radiation over a long period of time
- human papillomavirus (HPV) infection
People with lip cancer may have symptoms like:
- a sore or lesion on the lip that doesn’t go away
- a lump or area of thickening on your lip
- a white patch on your lip
- unexplained bleeding, pain or numbness affecting your lips
The table below shows the
| Stage of cancer | 5-year relative survival rate |
|---|---|
| Localized | 94.1% |
| Regional | 63.4% |
| Distant | 38.1% |
| Overall | 91.4% |
While breast cancer most often affects the breasts of females, it can also occur in the breasts of males. In 2017, the Centers for Disease Control and Prevention (CDC) found that this rare type of breast cancer affected
The factors that increase the risk of breast cancer in males include:
- older age
- a family history of breast cancer, especially if other fathers or brothers in the family have had it
- certain gene mutations, like those in BRCA1 and BRCA2
- previous radiation exposure
- high estrogen levels
- Klinefelter syndrome
The main symptom of breast cancer in males is a lump or bump in your breast that you can feel and that’s typically painless. Other symptoms can include:
- changes to the skin of your breast, such as redness or dimpling
- changes in the size or shape of your breast
- pain or discharge from your nipple
- a nipple that turns inward
The table below shows the
| Stage of cancer | 5-year relative survival rate |
|---|---|
| Localized | 95% |
| Regional | 84% |
| Distant | 20% |
| Overall | 83% |
Retinoblastoma is a type of cancer that affects the retina of the eye in children. It’s the most common eye cancer in children, affecting
Retinoblastoma is caused by having a gene change that can be inherited. Because of this, having a family history of retinoblastoma puts a child at a higher risk of having this type of cancer.
The symptoms of retinoblastoma can include:
- a white pupil
- crossed eyes
- red eyes
- eye pain
- bulging eye
- reduced vision
Vaginal cancer is cancer that starts in the vagina, which is the part of the female reproductive tract that connects the cervix to the outside of the body.
The CDC estimates that
The risk factors for vaginal cancer are:
- older age
- HPV infection
- a history vaginal, vulvar, or cervical precancers
- smoking
- exposure to the drug diethylstilbestrol (DES)
- HIV/AIDS
Some of the symptoms of vaginal cancer are:
- atypical vaginal bleeding, such as:
- abnormal vaginal discharge
- pain during sex
- urinary or bowel issues such as:
- constipation
- blood in your urine or stool
- pelvic pain
The table below shows the
| Stage of cancer | 5-year relative survival rate |
|---|---|
| Localized | 69% |
| Regional | 57% |
| Distant | 26% |
| Overall | 51% |
There are many types of rare cancer. These cancers can occur in any part of the body and can vary in their associated risk factors, symptoms, and outlook.
Generally speaking, the outlook for any cancer is best when it’s diagnosed and treated early. It’s recommended that you talk with a doctor if you develop concerning symptoms that are persistent or continue to get worse.