Neuroendocrine Tumor

Why choose us for Neuroendocrine Tumor care?

What you need to know about Neuroendocrine Tumor

Neuroendocrine tumors (NETs) are a diverse group of tumors that arise from cells of the neuroendocrine system. These cells are found throughout the body, but they are mainly located in organs such as the gastrointestinal tract, pancreas, and lungs.

Neuroendocrine cells have characteristics of both nerve cells and hormone-producing cells. They can release hormones into the bloodstream, affecting various bodily functions.

How to talk to your doctor about Neuroendocrine Tumor

Preparation

Research: Understand what NETs are.
Document Symptoms: Note frequency, severity, and impact on daily life.
Medical History: Be ready to share detailed personal and family medical history.
Questions: Prepare a list of questions in advance.

Initial Conversation

Describe Symptoms: Clearly explain your symptoms and concerns.
Express Fears: Be honest about your worries.

Key Questions to Ask

Diagnosis:
- What tests will confirm a NET diagnosis?
- Are there different types of NETs?
Treatment:
- What treatment options are available?
- What are the risks and benefits of each?
Prognosis:
- What is the typical outlook?
- How will progression be monitored?
Side Effects:
- What are potential side effects?
- How can they be managed?
Support:
- Are there support groups or resources?
- Can you refer me to a NET specialist?

Follow-Up

Clarify: Ask for simpler explanations if needed.
Take Notes: Write down key points.
Next Steps: Understand the plan for tests, treatment, and follow-up.

Support System

Bring Someone: Have a friend or family member for support.
Patient Advocate: Consider involving one if available.

Second Opinion

Another Perspective: Don’t hesitate to seek a second opinion if unsure about the diagnosis or treatment plan.

Example Script

“Doctor, I’ve been experiencing [describe symptoms] for [duration]. I’m concerned about a neuroendocrine tumor. Can we discuss what might be causing my symptoms and the steps for diagnosis and treatment?”

Additional Tips

Stay Informed: Use reliable sources like the American Cancer Society.
Manage Stress: Practice deep breathing, meditation, or speak to a counselor.

By being prepared and proactive, you can have an effective discussion with your doctor about neuroendocrine tumors.

Types

Neuroendocrine tumors are a diverse group of neoplasms that originate from neuroendocrine cells. They can occur throughout the body, but are most commonly found in the gastrointestinal tract, pancreas, and lungs. Here are the main types:

Gastroenteropancreatic Neuroendocrine Tumors (GEP-NETs):
- Gastric NETs: Arise in the stomach.
- Pancreatic NETs (pNETs): Include insulinomas, gastrinomas, glucagonomas, somatostatinomas, and VIPomas.
- Small Intestinal NETs: Often found in the small intestine, particularly the ileum.
- Colorectal NETs: Arise in the colon and rectum.
Lung NETs:
- Typical Carcinoids: Slow-growing and less aggressive.
- Atypical Carcinoids: More aggressive than typical carcinoids but less than small cell lung cancer.
- Large Cell Neuroendocrine Carcinomas (LCNEC): More aggressive and faster-growing.
- Small Cell Lung Cancer (SCLC): Highly aggressive and fast-growing.
Other NETs:
- Medullary Thyroid Carcinoma: Arises from the parafollicular cells (C cells) of the thyroid.
- Pheochromocytomas and Paragangliomas: Arise from the adrenal glands or extra-adrenal paraganglia.
- Merkel Cell Carcinoma: A rare, aggressive skin cancer.

Causes

The exact cause of neuroendocrine tumors is often unknown, but several factors can contribute to their development:

Genetic Mutations:
- Some NETs are associated with inherited genetic syndromes, such as Multiple Endocrine Neoplasia type 1 (MEN1), Von Hippel-Lindau disease (VHL), Neurofibromatosis type 1 (NF1), and Tuberous Sclerosis.
Hormonal Factors:
- Hormonal imbalances or overproduction of certain hormones can sometimes lead to the development of NETs.
Environmental Factors:
- Exposure to certain chemicals and toxins may increase the risk of developing NETs.
Lifestyle Factors:
- Factors such as smoking, excessive alcohol consumption, and a high-fat diet have been linked to an increased risk of certain types of NETs.
Chronic Conditions:
- Chronic conditions like atrophic gastritis, Zollinger-Ellison syndrome, and diabetes may increase the risk of developing NETs.

Symptoms

The symptoms of NETs can vary widely depending on the type, location, and whether the tumor is functional (producing hormones) or non-functional. Common symptoms include:

Gastroenteropancreatic NETs (GEP-NETs):
- Abdominal pain or discomfort
- Diarrhea or constipation
- Nausea and vomiting
- Unexplained weight loss
- Jaundice (yellowing of the skin and eyes)
- Gastrointestinal bleeding
Pancreatic NETs (pNETs):
- Insulinomas: Hypoglycemia, sweating, confusion, rapid heartbeat
- Gastrinomas: Recurrent peptic ulcers, abdominal pain, diarrhea
- Glucagonomas: High blood sugar, rash, weight loss
- VIPomas: Severe diarrhea, dehydration, low potassium levels
- Somatostatinomas: Diabetes, gallstones, diarrhea, steatorrhea (fatty stools)
Lung NETs:
- Cough, sometimes with blood
- Wheezing or shortness of breath
- Chest pain
- Recurring pneumonia
- Flushing of the skin
Medullary Thyroid Carcinoma:
- Lump in the neck
- Difficulty swallowing or breathing
- Hoarseness
- Diarrhea (due to elevated levels of calcitonin)
Pheochromocytomas and Paragangliomas:
- High blood pressure
- Headaches
- Sweating
- Rapid or irregular heartbeat
- Anxiety or panic attacks
Merkel Cell Carcinoma:
- Firm, painless nodule on the skin
- Skin lesion that may be red, purple, or skin-colored
- Rapid growth of the skin nodule

Diagnosis

Clinical Evaluation:
- Medical history and symptom assessment.
Imaging Studies:
- CT Scan: Locate and stage tumors.
- MRI: Detailed soft tissue images.
- PET Scan: Combined with CT or MRI for detailed localization.
- Octreotide Scan: Detects somatostatin receptor-positive tumors.
- Gallium-68 DOTATATE PET/CT: Highly sensitive for NETs.
Laboratory Tests:
- Blood Tests: Measure hormones and markers like chromogranin A (CgA).
- Urine Tests: Measure 5-HIAA, indicating carcinoid tumors.
Biopsy:
- Fine-Needle Aspiration (FNA): For accessible tumors.
- Core Needle Biopsy: Provides more tissue.
- Surgical Biopsy: If needed for definitive diagnosis.
Endoscopy:
- Gastroscopy/Colonoscopy: Visualize and biopsy GI tract tumors.
- Endoscopic Ultrasound (EUS): For pancreatic and upper GI tract tumors.

Treatment

Surgery:
- Curative: Remove localized tumors.
- Debulking: Remove as much tumor as possible.
- Liver Resection: For liver metastases.
Medications:
- Somatostatin Analogs (SSAs): Control symptoms and slow growth.
- Targeted Therapy: Drugs like everolimus and sunitinib.
- Chemotherapy: For high-grade NETs.
- Peptide Receptor Radionuclide Therapy (PRRT): Targeted radiation therapy.
Radiation Therapy:
- External Beam: For symptom relief.
- Selective Internal Radiation Therapy (SIRT): Radioactive beads in the liver.
Hormonal Therapy:
- Manage hormone-related symptoms in functional tumors.
Ablative Techniques:
- Radiofrequency Ablation (RFA): Destroys tumor cells with heat.
- Cryoablation: Uses cold to destroy tumors.
Supportive and Palliative Care:
- Symptom management and quality of life improvement.