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What To Know About Small Cell Esophageal Cancer

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.

    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:

    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.


    Dear Doctor: Are Genetic Tests That Determine Risk For Developing Alzheimer's Trustworthy?

    Dr. Keith Roach is a physician at Weill Cornell Medical College and New York Presbyterian Hospital. He writes an educational column on infectious diseases, public health and sports medicine.

    DEAR DR. ROACH: Would you please share with your readership what the latest available testing is for dementia and/or Alzheimer's? I mean this in the sense of whether you are likely to develop it -- not to find out whether you already have it.

    I am in my mid-60s, and my mother and her father both developed dementia by their 70s. So, I am wondering if there is a way to test whether this will be my future (and then do my best to keep it at bay). I believe many people would like to know this. -- C.O.

    ANSWER: There are genetic tests that are commercially available to help determine a person's risk for developing Alzheimer's or dementia; however, I don't recommend them in general. While I fully understand the desire to know what one's future holds, the available tests for people whose parents developed Alzheimer's in their 70s are of limited value. The results can show an increased predilection in a person who will never get dementia, or a low risk in a person who will. For the most common genetic test -- the APOE4 -- 40% of people who will develop Alzheimer's will test negative.

    Furthermore, the test has implications for family members, so it's particularly important to think through testing. I recommend against direct-to-consumer testing. If you really want to consider further testing, speak with a genetic counselor. In people with a history of early-onset Alzheimer's (a family member diagnosed before the age of 60), I also recommend consultation with a genetic counselor.

    Advice on how to prevent Alzheimer's is the same whether a person has identified a genetic risk or not. The evidence supports a Mediterranean-style diet; regular moderate exercise; social interaction; and possibly cognitive activities such as games and puzzles. There is no established role for prescription medications or supplements, no matter how aggressively they are marketed.

    DEAR DR. ROACH: My brother was just diagnosed with a rare form of prostate cancer, called small cell. What I've read doesn't sound encouraging. Are there any new treatments? -- Anon.

    ANSWER: Most prostate cancers start from cells that form part of the prostate gland, whose primary job is to secrete prostatic fluid. One of the components of prostatic fluid is an enzyme that liquifies mucus. It's called gamma-seminoprotein, but is more commonly known as prostate-specific antigen -- or PSA.

    Cancer cells normally continue some of the jobs that the normal cells they derive from are supposed to do, and the PSA test is a way to both screen for and monitor progression of the common form of prostate cancer during treatment. Regular prostate cancer varies from a slow-growing, indolent form that is easy to treat in its early stages to a much more aggressive form.

    Small cells, in contrast, are derived from stem cells. Small cell cancers most commonly show up in the lung, but are also rarely found in the bladder, stomach, gallbladder and other sites, including the prostate. Only 1% of prostate cancers are small cell cancers, and unfortunately, they do have a poor prognosis. Although most cancers do initially respond to platinum-based chemotherapy, chemotherapy can't quite kill all the cancer cells, so the promising early treatment is commonly followed by a relapse.

    I did a search at ClinicalTrials.Gov and found many ongoing trials that recruit people with small cell prostate cancer, which is your brother's best way to get involved with the newest treatments. Since this is a rare cancer, people are needed to identify what promising new treatments will work.

    Dr. Roach regrets that he is unable to answer individual letters, but will incorporate them in the column whenever possible. Readers may email questions to ToYourGoodHealth@med.Cornell.Edu or send mail to 628 Virginia Dr., Orlando, FL 32803.

    (c) 2022 North America Syndicate Inc.

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    Types Of Small-Cell Lung Cancer

    Most lung cancers are classified as either non-small cell lung cancer (NSCLC) or small-cell lung cancer (SCLC). SCLC is less common but tends to be more aggressive. It's subdivided into two main types: pure SCLC and combined SCLC.

    Small-cell lung cancer (SCLC) makes up about 15% of the nearly 240,000 lung cancer cases diagnosed in the United States each year. Smoking of tobacco products is by far the top risk factor.

    The names "SCLC" and "NSCLC" come from the way the cancer cells look under a microscope. SCLC cells look small and round, and NSCLC cells look larger than usual.

    The classification system for SCLC has changed over the years, but the most recent guidelines from the World Health Organization (WHO) classify it into two categories: "pure SCLC" if the cancer's features are purely SCLC and "combined SCLC" if it also has features of NSCLC.

    In this article, we take a deeper look at these two types of SCLC, including their symptoms, treatment options, and outlook.

    SCLC is also called oat cell carcinoma because of the microscopic appearance of its cells. The most common form of SCLC is also called pure SCLC if it doesn't have any features of NSCLC.

    Pure SCLC makes up about 80% of cases of SCLC, although estimates vary across studies.

    SCLC is classified as a neuroendocrine tumor, meaning that it starts in neuroendocrine cells or cells that can produce hormones in response to neurological signals.

    Combined SCLC occurs when the cancer cells also have features of NSCLC. It makes up roughly 20% of cases of SCLC.

    People with combined SCLC most commonly have features of a type of NSCLC called large cell carcinoma.

    Combined SCLC may less commonly be combined with squamous cell carcinoma and adenocarcinoma.

    At least 10% of the cancerous cells in a lung cancer tumor need to have a large cell carcinoma, or LCNEC, component for doctors to diagnose combined SCLC. There are no percentage requirements if SCLC is mixed with other NSCLC subtypes.

    The symptoms, response to chemotherapy, and survival rates of pure SCLC and combined SCLC are similar.

    The main symptoms are:

    SCLC is also the disease most commonly associated with paraneoplastic syndromes, which occur when cancerous neuroendocrine cells start producing excess hormones that can lead to many groups of symptoms. The most common types in people with SCLC are:

    SCLC and combined SCLC have similar risk factors, such as advanced age, male sex, and smoking history.

    The median age range of people who receive a diagnosis of combined SCLC is 59 to 64 years. Males seem to be affected more often than females, possibly as a result of higher rates of smoking.

    A history of smoking is by far the top risk factor for developing SCLC. Only about 2% of SCLC cases occur in never-smokers (people who have smoked fewer than 100 cigarettes in their life). Almost all people with combined SCLC have a history of heavy smoking.

    The main treatments for SCLC are chemotherapy and radiation therapy. These treatments may be administered:

    Surgery is rarely used to treat pure SCLC because the cancer has usually already advanced too far to be removed by the time a doctor diagnoses it. Fewer than 1 in 20 people have SCLC that is isolated to a single tumor and hasn't spread to another organ or the lymph nodes.

    Combined SCLC seems to develop more often in the outer part of the lung and is more often surgically removable. Surgery is usually an option for only limited stage cancer.

    Only about 40% of SCLC cases are diagnosed in the limited stage, which is when the cancer is on only one side of the chest and may or may not have spread to nearby lymph nodes on the same side.

    More than 70% of cases of combined SCLC seem to be diagnosed in the limited stage.

    When chemotherapy is administered, doctors often use the EC/EP regimen, which includes the following drugs:

  • cisplatin
  • etoposide/carboplatin
  • etoposide
  • Learn more about how limited stage SCLC is treated.

    SCLC tends to be more aggressive than NSCLC and generally has a less favorable outlook. Many people do not receive a diagnosis until the cancer has spread to distant tissues.

    The American Cancer Society reports the following 5-year relative survival rates for SCLC:

    The 5-year relative survival rate is a measure of how many people with this type of cancer are alive 5 years later compared to people without cancer.

    The outlook for combined SCLC tends to be better than for pure SCLC since SCLC seems to be more commonly diagnosed in the early stages. However, tumors with NSCLC components also seem to show stronger resistance to radiation therapy and chemotherapy.

    SCLC is less common than the other main category of lung cancer, NSCLC, but tends to be more aggressive. The classification of SCLC has changed over the years, but the newest WHO guidelines classify it as either pure SCLC or combined SCLC. Combined SCLC has some of the same features as NSCLC.

    SCLC tends to have a poor outlook and be aggressive. Surgery is usually an option only if the cancer is limited to one lung, which occurs in most people with SCLC.

    Your doctor can help you figure out what treatment options may give you the best chances of survival. Your doctor will likely recommend some combination of radiation therapy and chemotherapy.






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