NUT Carcinoma

NUT carcinoma is a rare, fast-growing cancer that starts in squamous cells. These cells line organs inside your body. The tumors usually start in your body’s center, or midline. This is why NUT carcinoma was once called NUT-midline carcinoma. It most often forms in your lungs, head (especially your sinuses) and neck. But it can develop in other places, too.

NUT carcinoma forms when the NUTm1 gene inside a cell fuses with another gene by mistake. The new fusion gene causes out-of-control cell growth. The cancer spreads fast.

NUT carcinoma is one of the most aggressive forms of squamous cell carcinoma. It’s also one of the most challenging cancers to treat. But your experience will depend on factors that your healthcare provider will discuss with you.

Symptoms of NUT carcinoma

You may not have NUT carcinoma symptoms until the cancer is more advanced. At this point, the tumor has often grown into tissue. You may feel generally tired or unwell. Other symptoms depend on where the tumor is. They include:

• A painful lump
• Chronic cough
• Fatigue
• Losing your sense of smell
• Nosebleeds
• Nasal congestion
• Shortness of breath or trouble breathing
• Sinus pressure
• Unexplained weight loss

Remember that lots of conditions can cause symptoms like these. And most are noncancerous. Even so, it’s important to see a healthcare provider if you’re having symptoms and don’t know why.

NUT carcinoma causes

NUT carcinoma forms when there’s a mutation involving a gene called NUTm1. A mutation is an error in the instructions (genetic material) that tell your cells how to work. Usually, NUTm1 fuses with a gene called BRD4. The new fusion gene tells your cells to make copies when they shouldn’t. The excess cells damage healthy tissue.

Doctors don’t know what causes the mutation. But they do know it isn’t inherited. NUT carcinoma isn’t associated with other common cancer causes and risk factors. And it’s unrelated to smoking or exposure to cancer-causing toxins (carcinogens).

Risk factors

Most people diagnosed with NUT carcinoma are teens or young adults. But anyone can get this cancer. Infants have been diagnosed with NUT carcinoma and so have people in their 80s.

How doctors diagnose this condition

Healthcare providers diagnose NUT carcinoma with a biopsy. During this procedure, your provider removes a tissue sample from the tumor. A lab tests it for cancer cells and the NUTm1 gene.

You’ll also need imaging tests so your provider can see the tumor inside your body. This helps them stage it. Cancer staging helps providers know how advanced the cancer is. Stage is based on the tumor’s size, location and whether it has spread.

A CT scan or MRI can show where the tumor is in your body. A PET scan can show if the cancer has spread. Metastatic NUT carcinoma usually spreads to your lymph nodes and bones first.

How is NUT carcinoma treated?

Your healthcare provider will likely recommend a combination of treatments. You may need surgery to remove all or part of the tumor. You may need radiation therapy to shrink it or prevent it from growing back after surgery. You may need chemotherapy to treat cancer that’s spread.

Still, these treatments don’t always work with NUT carcinoma. This cancer is so rare that doctors haven’t been able to study it enough to know which treatments are best.

This is why your best option may be a clinical trial. Clinical trials test new therapies or treatment approaches. Currently, doctors are researching new treatments for NUT carcinoma, like targeted therapy. This treatment targets the genetic mutation that fuels cancer cell growth.

When should I see my healthcare provider?

You’ll see your provider regularly for check-ups. During these visits, you may need tests to see if the tumor is responding to treatment. If treatment gets rid of the tumor, you’ll need tests to see if it’s come back.

In the meantime, let your provider know if you’re noticing new symptoms or treatment side effects.

Is NUT carcinoma curable?

There isn’t a cure for NUT carcinoma. It’s a fast-growing cancer that often spreads to other parts of your body quickly. On average, people receiving treatment live about 10 months following diagnosis. For every 10 people diagnosed with NUT carcinoma, 2 to 3 live for two or more years.

As with any cancer, many factors shape your prognosis, or the likely outcome of your cancer with treatment. Your prognosis depends on where the tumor is located and whether your healthcare provider can remove it entirely with surgery or radiation. The type of gene that fuses with NUTm1 also plays a role in your prognosis.

Your healthcare provider will explain treatment goals based on your diagnosis.

Is there anything I can do to feel better?

One of the best things you can do is take advantage of all available resources that can help you navigate this diagnosis. Surround yourself with people you trust to support you.

Ask your provider whether you’re a candidate for a clinical trial, and see if they can connect you with palliative care resources. Palliative care professionals can help you manage the demands of having cancer. This includes managing chemotherapy and radiation side effects. They can also help you handle the responsibilities of everyday life.

What is the survival rate for NUT carcinoma?

More than 8 out of 10 people diagnosed with NUT carcinoma die the same year of their diagnosis. But not everyone’s experience is the same. Several people with NUT carcinoma are still alive 47 months after being diagnosed.