Neuroendocrine Tumours Flashcards
Definition of Neuroendocrine Tumours
Neuroendocrine tumors (NETs) are tumors that develop from neuroendocrine cells, which are present in organs such as the gastrointestinal tract, lungs, pancreas, and other locations. These tumors can be benign or malignant and can produce hormones.
Types of Neuroendocrine Tumours
Neuroendocrine tumors can be categorized into several types based on their origin and characteristics. The main types include:
Gastrointestinal neuroendocrine tumors (GI-NETs): These tumors originate in the digestive system, including the stomach, intestines, appendix, and rectum.
Pulmonary neuroendocrine tumors: These tumors develop in the lungs and are classified into subtypes, including typical carcinoid, atypical carcinoid, small cell carcinoma, and large cell neuroendocrine carcinoma.
Pancreatic neuroendocrine tumors (PNETs): These tumors arise in the pancreas and can be further classified into functional (hormone-producing) or non-functional tumors.
Neuroendocrine tumors of other sites: These are rare NETs that can develop in various locations, such as the thymus, adrenal glands, ovaries, or testes.
Diagnosis of Neuroendocrine Tumours
Medical history and physical examination: The healthcare provider assesses symptoms, medical history, and performs a physical examination.
Imaging tests: Imaging techniques like computed tomography (CT), magnetic resonance imaging (MRI), or somatostatin receptor scintigraphy (SRS) are used to locate and assess the size and spread of tumors.
Biopsy: Tissue samples are taken from the tumor for analysis and confirmation of the diagnosis.
Laboratory tests: Blood and urine tests may be conducted to measure hormone levels, such as chromogranin A, 5-hydroxyindoleacetic acid (5-HIAA), or specific markers associated with certain types of NETs.
Differential Diagnosis of Neuroendocrine Tumours
Distinguishing neuroendocrine tumors from other conditions may involve ruling out other potential causes of symptoms, such as gastrointestinal disorders, lung cancer, or pancreatic conditions. Differential diagnosis often requires a combination of imaging, pathology, and hormone level assessments.
Symptoms of Neuroendocrine Tumours
Flushing of the skin
Diarrhea
Abdominal pain
Wheezing or shortness of breath
Carcinoid syndrome (flushing, diarrhea, and wheezing associated with certain GI-NETs)
Weight loss
Hormonal abnormalities (e.g., Cushing’s syndrome, hyperglycemia)
Complications of Neuroendocrine Tumours
Metastasis: NETs can spread to other parts of the body, such as the liver, lungs, or bones.
Carcinoid syndrome: Certain GI-NETs can produce hormones that cause flushing, diarrhea, and wheezing.
Hormonal imbalances: Hormone-producing NETs can disrupt normal hormonal function, leading to various complications depending on the specific hormone involved.
Treatment of Neuroendocrine Tumours
Surgery: Surgical removal of the tumor is the primary treatment for localized NETs.
Somatostatin analogs: Medications like octreotide or lanreotide can be used to control symptoms and inhibit tumor growth in certain cases.
Targeted therapy: Drugs that target specific molecular abnormalities in NETs, such as everolimus or sunitinib, may be used.
Peptide receptor radionuclide therapy (PRRT): This treatment involves injecting a radioactive substance that selectively targets neuroendocrine cells to deliver radiation directly to the tumor.
Chemotherapy: Chemotherapy drugs may be used for more aggressive or advanced neuroendocrine tumors.
Liver-directed therapies: In cases of liver metastases, treatments like radiofrequency ablation, embolization, or selective internal radiation therapy (SIRT) may be employed.
