Small cell esophageal cancer is a rare type of cancer that tends to spread quickly. It usually has a poorer outlook than other types of esophageal cancer.

The American Cancer Society estimates that more than 21,000 people will receive an esophageal cancer diagnosis in the United States in 2023.

Only 0.4–2.8% of these cases are expected to be small cell esophageal cancer (SCEC).

SCEC is a type of neuroendocrine cancer. It tends to have a poor outlook than more common types of esophageal cancer. Neuroendocrine cancers start in cells that release hormones in response to nerve signals.

Due to its rarity, doctors know relatively little about how to treat SCEC best. They often use some combination of:

Keep reading to learn more about this rare type of cancer, including risk factors, symptoms, and its outlook.

SCEC is a highly aggressive cancer that makes up less than 3% of esophageal cancers. Most people with SCEC have cancer spread to distant parts of their body when they receive a diagnosis.

The name “small cell” comes from the microscopic appearance of the cells. Doctors can differentiate SCEC from other types of esophageal cancer by taking a sample of the cancer and examining it under a microscope.

The origin of SCEC is controversial. Scientists theorize it develops in cells found in the outer part of the esophagus, called the mucosa. It generally develops in the lower or mid-esophagus.

SCEC is similar to small cell cancers that develop in other parts of the body, such as small cell lung cancer.

Other areas that small cell cancer can develop are the:

Symptoms of SCEC are often vague and similar to symptoms of esophageal squamous cell carcinoma. The most common symptom is trouble swallowing, which gets worse over time.

Other common symptoms include:

The average time between the onset of symptoms and diagnosis has been reported as around 4 months.

Learn more about the symptoms of esophageal cancer.

The development of SCEC is complex. It likely involves a combination of environmental and genetic factors. Males are diagnosed about three times more often than females.

Researchers are still investigating factors that increase your risk of developing SCEC. It’s thought that risk factors might be similar to those for squamous cell esophageal cancer. They may include:

A 2022 study suggests that as many as 90% of people with SCEC have a history of smoking.

There’s no standard treatment for SCEC due to its rarity. The most common treatment is chemotherapy combined with radiation therapy to reduce symptoms and prolong survival.

Stage 1 or 2A treatment

Most researchers believe that surgery should be the main treatment for stage 1 or 2A SCEC. Other researchers believe chemotherapy and radiation therapy should be the main treatments.

Stage 2B treatment

According to a 2020 review, some research suggests survival rates are similar in people who receive:

  • surgery by itself
  • surgery followed by chemotherapy
  • chemoradiation therapy, which is a combination of chemotherapy and radiation therapy

Other research suggests that nonsurgical treatment may be best.

Stage 3 and 4 treatment

Chemoradiation therapy is generally the primary treatment for cancers in stage 3 or above.

In a 2019 study, researchers found that the overall response rate of radiation therapy was 60% in a group of 42 people with SCEC.

Clinical trials are underway to see whether targeted therapies and immunotherapy may be beneficial. These treatments have been found to be beneficial for treating other types of esophageal cancer, but there’s a limited amount of research examining how they influence SCEC survival rates.

Is small cell esophageal cancer hereditary?

Only a very small number of esophageal cancers are passed through families.

In a 2022 study, only 2 out of 56 people (3.6%) with SCEC had a family history of cancer.

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Esophageal cancer tends to have a poor outlook. Only about 20% of people with esophageal cancer are alive 5 years after diagnosis.

SCEC tends to have a poorer outlook than other types of esophageal cancer because it is aggressive and lacks standardized treatment options. SCEC has spread to distant organs in 31–90% of cases at the time of diagnosis.

A 2022 review reports the following survival rates:

Time periodOverall survival rate
1 year56–86%
3 years27.3–35.7%
5 years6.7–18%

Most studies have also reported that half of people with SCEC live fewer than 8–13 months.

Cancer spreading to the lymph nodes and larger tumor size are some reasons linked to a poorer outlook.

Learn more about esophageal cancer survival rates.

SCEC is a rare but highly aggressive form of esophageal cancer. It tends to spread quickly to distant body parts and has a poorer outlook than other types of esophageal cancer.

Researchers are still investigating the best way to manage and treat SCEC. Little is known about the best way to treat it due to its rarity, but surgery, chemoradiation therapy, and immunotherapy are among the most common treatment options.