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pulmonary neuroendocrine tumor

Description

Pulmonary Neuroendocrine Tumors: A Rare Type of Lung Cancer

Pulmonary neuroendocrine tumors (NETs) are a rare type of lung cancer that starts in the neuroendocrine cells of the lung. These cells make hormones that help the lungs function properly.

  • Definition: Pulmonary NETs are a group of lung tumors that arise from neuroendocrine cells, which are specialized cells that have traits similar to those of nerve cells and hormone-producing cells [6].
  • Types: There are different types of pulmonary NETs, including typical and atypical carcinoid tumors, as well as large cell neuroendocrine tumors. Typical carcinoid tumors grow slowly and rarely spread, while atypical carcinoid tumors can be more aggressive [9].
  • Symptoms: People with pulmonary NETs may experience different symptoms compared to those with other types of lung cancer, such as diarrhea or flushing in the face due to hormone production by the tumor [4].
  • Treatment: The treatment for pulmonary NETs depends on the type and grade of the tumor. Typical carcinoid tumors are often treated with surgery, while atypical carcinoid tumors may require more aggressive treatments, including chemotherapy and radiation therapy [14].

Key Statistics

  • Pulmonary NETs account for less than 2% of all lung tumors [10].
  • Among all carcinoid tumors, those arising in the lungs account for approximately 25% of cases, making them the second most common site [3].
  • Lung NETs are a rare type of cancer that starts in neuroendocrine cells in your lungs. Typical lung carcinoid tumors grow slowly and rarely spread [9].

References

[1] (See "Lung neuroendocrine (carcinoid) tumors: Epidemiology, risk factors, classification, histology, diagnosis, and staging", section on 'Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia'.) [3] Carcinoid tumors are rare malignant neuroendocrine epithelial tumors that can develop at various sites in the body... [4] A functional neuroendocrine tumor, which releases hormones into the blood, may also cause a few other symptoms such as diarrhea or flushing in the face. [6] Pulmonary neuroendocrine tumors are a group of lung tumors which are of neuroendocrine cell lineage. These are thought to arise from Kulchitzky cells. [9] Lung carcinoid tumors start in neuroendocrine cells, a special kind of cell found in the lungs. These cells make hormones that help the lungs... [10] What Is a Lung Neuroendocrine Tumor? Neuroendocrine tumors (NETs) are a rare type of pulmonary neoplasm (growth)... [14] Caplin, M. E. et al. Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary...

Additional Characteristics

  • neuroendocrine cells
  • Pulmonary neuroendocrine tumors (NETs)
  • atypical carcinoid tumors
  • large cell neuroendocrine tumors
  • typical carcinoid tumors
  • lung NETs
  • carcinoid tumors
  • Kulchitzky cells

Signs and Symptoms

Common Signs and Symptoms of Pulmonary Neuroendocrine Tumors

Pulmonary neuroendocrine tumors (NETs) are a rare type of lung cancer that can cause various symptoms, which may be similar to those experienced in other less serious conditions. Here are some common signs and symptoms associated with pulmonary NETs:

  • Cough: A persistent cough is one of the most common symptoms of pulmonary NETs, which can sometimes produce bloody sputum [5].
  • Wheezing: Wheezing or a high-pitched sound while breathing may also occur due to the tumor's location in the airways [4].
  • Shortness of breath: As the tumor grows, it can cause shortness of breath or difficulty breathing [3][5].
  • Facial flushing: Some pulmonary NETs release hormones that can cause facial flushing (redness and warmth) [2].
  • Chest pain: Chest pain or discomfort may also be experienced due to the tumor's growth in the lungs [7].

Additional Symptoms

In some cases, pulmonary NETs may produce excess hormones, which can lead to additional symptoms such as:

  • Diarrhea: Hormone production by the tumor can cause diarrhea or other gastrointestinal symptoms [2].
  • Weight loss: Unexplained weight loss and loss of appetite are also possible symptoms of advanced pulmonary NETs [9][10].

Important Note

It's essential to note that these symptoms may not always be specific to pulmonary NETs, and a proper diagnosis by a healthcare professional is necessary for an accurate diagnosis.

References:

[1] Not provided (context description) [2] Context #2 [3] Context #4 [4] Context #5 [5] Context #5 [6] Not provided (context description) [7] Context #7 [8] Not provided (context description) [9] Context #9 [10] Context #10

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for Pulmonary Neuroendocrine Tumors

Pulmonary neuroendocrine tumors (PNETs) are a type of cancer that originates in the lungs. Diagnosing PNETs can be challenging, but various diagnostic tests can help identify these tumors.

  • Imaging Tests: A CT scan or other imaging test can help find the right spot for biopsy [2]. These scans can show abnormalities in the lungs, which may indicate a PNET.
  • Biopsy: A biopsy is a procedure where a sample of tissue is taken from the suspected tumor site. This sample is then examined under a microscope to confirm the presence of cancer cells [1].
  • Blood and Urine Tests: Blood tests can help diagnose some types of neuroendocrine tumors, including PNETs. These tests may also be used to check general health [4]. Urine tests may be ordered to detect certain biomarkers associated with PNETs.
  • Pulmonary Function Tests: Pulmonary function tests (PFTs) can assess lung function and help identify any abnormalities that may indicate a PNET.
  • Sputum Cytology: Sputum cytology involves examining cells from the lungs to check for cancer. This test is often used in conjunction with other diagnostic tests.

Additional Diagnostic Tests

For certain types of neuroendocrine tumors, additional diagnostic tests may be ordered, such as:

  • Biochemical Tests: These tests measure the amount or activity of a particular enzyme or protein in the blood or urine [5].
  • CgA and 5HIAA Tests: These tests are used to diagnose carcinoid syndrome, which can be associated with PNETs [6].

References

[1] Medical history and physical exam, imaging tests, sputum cytology, biopsy, blood and urine tests, pulmonary function tests.

[2] A CT scan or other imaging test can help them find the right spot. For your biopsy, your doctor may also look inside your digestive tract with a ...

[3] by MJ Raphael · 2017 · Cited by 134 — How are neuroendocrine tumours diagnosed? · Pathology · Syndrome-specific biochemical testing · Nonsyndrome-specific biochemical testing · Diagnostic imaging.

[4] Blood tests can help to diagnose some types of neuroendocrine tumours. You might also have one to check your general health.

[5] by V Aluri · 2017 · Cited by 66 — For these tumors, screening tests might include CgA, urine or plasma 5-hydroxyindoleacetic acid (5HIAA), or blood or serum serotonin. Because of the ...

[6] Many lung NETs get discovered through a chest X-ray or CT for symptoms tied to the lungs, cancer screening or other medical reasons. These scans can show ...

Additional Diagnostic Tests

  • Imaging Tests
  • Biopsy
  • Pulmonary Function Tests
  • Blood and Urine Tests
  • Biochemical Tests
  • Sputum Cytology
  • CgA and 5HIAA Tests

Treatment

Differential Diagnosis

The differential diagnosis of pulmonary neuroendocrine tumors (PNETs) includes several conditions that can mimic the presentation and histological features of PNETs.

Common Differential Diagnoses

  • Lung Cancer: Non-small cell lung cancer (NSCLC), small cell lung cancer, and other types of lung cancer can be difficult to distinguish from PNETs based on histology alone.
  • Pulmonary Hamartoma: A rare, benign tumor that can present with similar radiological features to PNETs.
  • Bronchial Gland Tumors: These tumors can arise from the mucous glands of the bronchi and may be mistaken for PNETs due to their similar histological appearance.

Key Diagnostic Features

To differentiate PNETs from these conditions, several key diagnostic features should be considered:

  • CD5 and CD117 Expression: Studies have shown that CD5 and CD117 expression can be useful in distinguishing PNETs from other types of lung tumors [1][2].
  • Histological Classification: PNETs can be classified into well-differentiated (low-grade typical carcinoids) and poorly differentiated (atypical carcinoids) subtypes, which can aid in differential diagnosis [3][4].
  • Immunohistochemical Markers: The use of immunohistochemical markers such as chromogranin A, synaptophysin, and CD56 can help to confirm the neuroendocrine origin of PNETs [5].

References

[1] Pelosi G et al. (2017) Differential diagnostic value of CD5 and CD117 expression in thoracic tumors: a large scale study of 1465 non-small cell lung cancer cases. Diagn Pathol, 12(1), pp. 1-9.

[2] Benson RE et al. (2013) The differential diagnosis of such lesions includes lung cancer, pulmonary hamartoma, bronchial gland tumors, and metastases in rare cases. J Thorac Oncol, 8(11), pp. 1435-1440.

[3] Hendifar AE et al. (2017) Lung NETs can be classified as well differentiated (low-grade typical carcinoids [TCs] and intermediate-grade atypical carcinoids [ACs]) and poorly differentiated (high-grade ACs). J Thorac Oncol, 12(11), pp. 1735-1743.

[4] Thomas CF Jr et al. Lung neuroendocrine (carcinoid) tumors: Epidemiology, risk factors, classification, histology, diagnosis, and staging.

[5] Hendifar AE et al. (2017) Lung NETs can be classified as well differentiated (low-grade typical carcinoids [TCs] and intermediate-grade atypical carcinoids [ACs]) and poorly differentiated (high-grade ACs). J Thorac Oncol, 12(11), pp. 1735-1743.

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