Neuroendocrine cells are specialized cells found in many parts of the body, including the gastrointestinal (GI) tract, gallbladder, and lungs.
These cells receive signals from the brain to produce and release certain hormones that control many bodily functions.
Neuroendocrine tumors (NETs) develop when neuroendocrine cells become overactive and cancerous. NETs typically involve the GI tract and the lungs.
Once a doctor makes a diagnosis, they determine how much the cancer has spread through staging.
Stage 4 cancer means that cancer cells have spread from an original tumor to other parts of the body.
Some doctors may use the TNM staging system, though this is uncommon. TNM stands for tumor, node, and metastasis.
- Tumor: Refers to the size of the tumor.
- Node: Refers to the spread of cancer to nearby lymph nodes.
- Metastasis: Refers to the spread of cancer to distant organs, such as the other lung, brain, or bones.
Stage 4, also called the advanced stage, means that cancer has spread. At stage 4, the tumor can be any size and may or may not have spread to nearby lymph nodes.
Neuroendocrine lung tumors are a family of tumors that arise from the cells that line the bronchi and other parts of the lung.
This disease group has distinct clinical and pathological characteristics. It varies in cell structure, features, appearance, presence of dead cells (necrosis), and the number of dividing cells (mitotic index).
Typical carcinoid (TC)
TCs are low grade, slow-growing tumors that rarely spread (metastasize) outside the lungs. This type of tumor has a good outlook. Management and treatment typically involve surgery. Smoking is not associated with TCs.
Atypical carcinoid (AC)
ACs are intermediate grade tumors that
SCLC is the most common NET of the lungs. It is also the most aggressive. In
Large cell neuroendocrine carcinoma (LCNEC)
Similar to SCLC, LCNEC is also a fast-growing tumor. However, it is not as common as SCLC. Smoking is also
A 2021 article in the Annals of Oncology explains that people with carcinoids have a better outlook compared with those with SCLC and LCNEC. People who have carcinoids are also generally younger, and the disease is not strongly associated with smoking.
Most people receive an SCLC diagnosis when their cancer has already spread extensively. A 2020 review notes that while SCLC tends to initially respond to chemotherapy, it is likely that people with it will go on to experience relapse or the disease will progress.
A person with SCLC may have symptoms related to a localized lesion (in the lung or lungs) or distant metastases (cancer that has spread to other parts of the body). Symptoms of localized lesions typically involve airway obstruction. This is because the cancer is in one or both lungs, meaning it will affect a person’s airway.
Symptoms may include:
As the disease progresses and spreads, people may experience other symptoms, such as:
SCLC may also cause:
- sensory neuropathy
- and Lambert-Eaton syndrome
Endocrine involvement may include:
The majority of people with SCLC contact to their doctor because they experience symptoms of the disease. Before doctors ask people to undergo tests, they will first perform a thorough physical exam and take a medical history.
If results suggest the possibility of lung cancer, doctors may ask a person to undergo several tests. To make a diagnosis, specialists extract lung cell samples and examine them in a lab.
These tests include:
- Thoracentesis: During this procedure, a healthcare professional uses a hollow needle to extract fluid from around the lungs and check whether it contains cancer cells.
- Needle biopsy: During this procedure, a doctor removes tissue samples from a suspicious mass by passing a thin needle through it.
- Bronchoscopy: During this procedure, a doctor uses a thin, flexible tube to check the airway for blockages. They may also perform a biopsy during the procedure if they find a blockage or tumor.
- Endobronchial ultrasonography: A healthcare professional uses ultrasound to visualize structures within and beside the central airways.
- Open surgery: This procedure involves a surgical incision through the skin to get a sample from the tumor.
A pathologist will evaluate the samples and examine them under a microscope to check for the cell structure and appearance unique to SCLC.
The imaging techniques help identify suspicious masses or tumors that may be cancerous. They also help monitor cancer spread, its response to treatment, relapse, and whether it has returned.
Treatment for SCLC depends on the cancer’s aggressiveness and a person’s health status.
People with limited stage SCLC typically receive a combination of chemotherapy and chest radiation therapy. Surgery may also be an option for otherwise well individuals if the tumor is limited to one area. A person also receives chemotherapy after surgery.
Since brain metastasis is common, many people also receive radiation therapy to the head, called prophylactic cranial irradiation.
Surgery and radiation are not useful as initial treatments for extensive stage SCLC. The standard of care for this condition is radiation and a combination of the chemotherapy drugs etoposide and either cisplatin or carboplatin.
A 2020 review mentions that immunotherapy combined with chemotherapy is the new frontline treatment for SCLC. This combination may significantly improve the overall outlook for people with extensive stage SCLC.
Radiation therapy can also relieve symptoms caused by cancer growth in the lungs or when cancer has spread to other areas. People with additional health problems may benefit from low chemotherapy doses or supportive care to address complications and keep them as comfortable as possible.
Stage 4 neuroendocrine SCLC is the most common NET of the lung. Doctors often diagnose it when it has already spread.
Compared with other lung cancers, it has a poorer outlook. It is likely that a person will experience relapse even after their condition responds well to initial treatments.