Limited Stage Small Cell Lung Cancer Overview

doctor talking with a patient about limited stage small cell lung cancer

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In This Article

Limited stage small cell lung cancer is the earliest stage of the disease. Unlike non-small cell lung cancer, small cell lung cancer is divided into only two stages; limited stage and extensive stage. Surgery is performed much less commonly with non-small cell lung cancer, with the mainstay of treatment being chemotherapy and radiation therapy. That said, after years of little progress in treatment, new options are being evaluated in clinical trials including the use of immunotherapy drugs.

Definition

Limited stage small cell lung cancer involves cancer that is present in only one lung and may have spread to nearby lymph nodes or the tissue between the lungs, but has not spread (metastasized) to other regions of the body.

Small cell lung cancer is separated into two stages by a staging system developed by radiation oncologists. With this staging, limited stage refers to tumors that are encompassed within a tolerable radiation field. Extensive stage small cell lung cancer, in contrast, refers to tumors that are too large or too widespread to be encompassed within one tolerable radiation field. When a small cell lung cancer has spread to distant regions of the body (metastatic disease) it is always considered extensive stage.

Your doctor may also describe your cancer in terms of TNM staging. Using this system, T stands for the size of the tumor, N stands for the presence of cancer in lymph nodes, and M is used to describe distant metastasis. With limited stage non-small cell lung cancer, a tumor may be of variable size (T) and may or may not have lymph nodes involved (N) but will always be M0 (M followed by a zero), meaning that the tumor has not spread to the other lung or to distant regions of the body.

Roughly 30% to 40% of people with small cell lung cancer are diagnosed early when their tumors are still considered limited stage, whereas in 60 to 70 percent of people the disease has already progressed to extensive stage.

Symptoms

Unlike cancers such as lung adenocarcinoma, which usually occurs in the outer regions of the lung, small cell lung cancer tends to occur more centrally, near the large airways. For this reason, symptoms often appear early and include those related to a tumor being present in a large airway (bronchus), such as coughing, coughing up blood, and repeated episodes of pneumonia due to obstruction of the airways.

Symptoms of limited stage lung cancer can include:

  • Persistent cough: A chronic cough is often the first symptom of small cell lung cancer
  • Coughing up blood (hemoptysis): Coughing up blood is a common first symptom of small cell lung cancer. Even a small amount of blood is of concern, and coughing up more than a teaspoon or two of blood is considered a medical emergency.
  • Swelling in the face and neck
  • Shortness of breath
  • Wheezing
  • Recurrent respiratory infections such as pneumonia or bronchitis (due to obstruction of the airways by tumor)
  • Hoarseness
  • Clubbing: A deformity of the fingers in which the end of the fingers takes on the appearance of an upside-down teaspoon.

Paraneoplastic Symptoms

Small cell lung cancer can also result in a broad variety of symptoms due to paraneoplastic syndromes, symptoms that are due to hormones secreted by a tumor or by the body’s immune response to a tumor rather than the tumor itself. Some of these include:

  • Muscle weakness in the upper limbs, vision changes, and difficulty swallowing (Lambert-Eaton myasthenic syndrome)
  • Weakness, fatigue, and a low sodium level in the blood (syndrome of inappropriate antidiuretic hormone secretion or SIADH)
  • A round full face and weight gain, excessive thirst, excessive hair growth, and weakness (Cushing's syndrome)
  • Loss of coordination and difficulty speaking (paraneoplastic cerebellar degeneration)

Symptoms of Metastasis

Limited stage lung cancer is defined as cancer which has not spread to distant regions of the body. Small cell lung cancer, however, tends to spread early, especially to the brain, and with extensive stage small cell lung cancer, the first symptoms of cancer may be those related to brain metastases, such as weakness on one side of the body, visual changes, speech changes, or seizures.

Diagnosis

The diagnosis of small cell lung cancer is often suspected on an imaging study such as a chest CT scan. A lung biopsy often follows in order to confirm the diagnosis.

Further tests are then done to accurately stage the tumor. This can include an MRI of the brain to look for brain metastases, a bone scan, an abdominal CT scan, and more. These tests are critical if surgery is to be considered.

Treatment

Treatment options for lung cancer include both local treatments such as surgery and radiation therapy and surgery, and systemic treatments such as chemotherapy. Clinical trials are also looking at the possible role of targeted therapies and immunotherapy for small cell lung cancer.

Since small cell lung cancers tend to grow rapidly, it's usually recommended that treatment be started as soon as possible, or within 30 days of diagnosis.

Chemotherapy and Radiation Therapy

Many people respond well to a combination of chemotherapy and radiation therapy, although many tumors eventually stop responding. With limited stage cancer, a 2019 study published in JAMA Oncology found that the combination resulted in long-term survival for some people with limited stage small cell lung cancer (with earlier stage disease classified stage stage I or stage II).

Chemotherapy usually includes a combination of a platinum drug (such as Platinol (cisplatin) and VePesid (etoposide) in a series of four to six infusions (more than this increases toxicity but does not appear to improve outcomes). Chemotherapy is most given at the same time as radiation, which is given one to two times daily.

Immunotherapy

Immunotherapy, specifically treatment with the type of immunotherapy drugs known as immune checkpoint inhibitors, has been quite successful in treating non-small cell lung cancer and there is hope that these drugs may be helpful for small cell lung cancer as well. These drugs are now being studied in combination with chemotherapy and radiation with some encouraging findings.

Drugs that are being evaluated include the CTLA-4 inhibitor Yervoy (ipilimumab) and the PD-1 inhibitors Opdivo (nivolumab) and Keytruda (pembrolizumab).

Surgery

Surgery is rarely performed for small cell lung cancer (it is usually considered inoperable) but is occasionally considered if a tumor is present in only one lung and nearby lymph nodes. Adjuvant chemotherapy (chemotherapy after surgery) is usually recommended if surgery is done for small cell lung cancer.

Prophylactic Cranial Irradiation

If individuals respond well to treatment (have either a complete or partial respond to chemotherapy and radiation), prophylactic cranial irradiation (PCI)—preventative radiation therapy to the brain—is sometimes recommended to lower the risk that any cancer cells that have spread to the brain (but aren’t seen on radiology studies) will grow and cause symptoms. This is currently an area of debate as PCI can have cognitive side effects (such as memory loss), but does appear to improve survival.

Clinical Trials

Clinical trials are in progress for both stages of small cell lung cancer, evaluating new treatments and treatment combinations for this aggressive cancer. The National Cancer Institute recommends that anyone with small cell lung cancer consider participating in a clinical trial.

Prognosis

Survival rates for small cell lung cancer have improved since the addition of radiation therapy to treatment and the use of PCI, but they still remain low. Survival rates for small cell lung cancer are very different according to the stage.

For people who receive standard of care treatment with chemotherapy and radiation for limited stage small cell lung cancer, the current 5-year survival rate is 30% to 35%.

Since small cell lung cancer grows rapidly, and we have come a long way with other fast-growing cancers such as leukemia, it is hoped that better treatments will be found in the future.

Coping

Studies suggest that learning what you can about your lung cancer can improve your treatment. Ask questions. Find out about clinical trials that might be appropriate for you. Getting a second opinion (or a third or fourth) at one of the larger National Cancer Institute designated cancer centers can be helpful with any type or stage of lung cancer. Consider joining a support group. Ask for and let your loved ones help you in your journey with cancer.

A Word From Verywell

It is important to be your own advocate when you have lung cancer. After many years of little change in the treatment of lung cancer, especially small cell lung cancer, progress is being made. That said, it challenging for any physician, even one who specializes in lung cancer, to stay abreast of all of the changes.

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Article Sources

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  2. Pakkala S, Owonikoko TK. Immune checkpoint inhibitors in small cell lung cancer. Journal of Thoracic Disease. 2018. 10(Suppl 3):S460-S467. doi:10.21037/jtd.2017.12.51

  3. Higgins KA, Gorgens S, Sudmeier LJ, Faivre-Finn C. Recent developments in limited stage small cell lung cancer. Translational Lung Cancer Research. 2019. 8(Suppl 2):S147-S152. doi:10.21037/tlcr.2019.05.13

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