Neuroendocrine tumors (NET) occur when the cancer cells are found in the specialized hormone-producing and specialized nerve cells. These tumors are rare and can occur anywhere in the body, but are commonly found in the lungs, small intestine, appendix, rectum and pancreas. According to Best Endocrinologist in Lahore these tumors can be functional, i.e. hormone-producing or non-functional. Read on to know more about neuroendocrine tumors and how to deal with them:

What are neuroendocrine tumors?

Neuroendocrine tumors are considered to come from the endocrine or hormone-producing cells of the body. They help to regulate functions of the body including growth, metabolism and reproduction. Presently, all neuroendocrine tumors are considered malignant, though some of them can be less aggressive than the others, depending on their location and the types of cells they involve.

The cause of neuroendocrine tumors is not exactly understood; however, the chances increase with certain inheritable conditions like:

• Multiple endocrine neoplasia type 1 (MEN-1)
• Multiple endocrine neoplasia type 2 (MEN-2)
• Von Hippel-Lindau syndrome (VHL)
• Neurofibromatosis type 1
• Tuberous sclerosis

What are the types of neuroendocrine tumors?

The types of neuroendocrine tumors include:

• Paraganglioma
• Adrenal cancer
• Carcinoid tumors
• Pheochromocytoma
• Pancreatic neuroendocrine tumors
• Merkel cell carcinoma

What are the symptoms of neuroendocrine tumors?

Neuroendocrine tumors can present with the following symptoms:

• Pain from the growth site
• Unrelenting fatigue
• Lump under the skin
• Weight loss
• Excessive thirst
• Flushing of skin
• Diarrhea
• Tremors and shakiness
• Dizziness
• Frequent urination
• Skin rash

How are neuroendocrine tumors diagnosed?

For diagnosis of neuroendocrine tumors, investigations include:

• Physical examination: a thorough history and examination of the patient helps to establish the diagnosis of neuroendocrine tumors. The healthcare provider examines the patient to look for swollen lymph nodes and signs of excessive hormone production.  
• Hormone levels: if a neuroendocrine tumor is suspected, the level of various hormones is checked to see if they are within range or higher than usual. In case of higher than normal blood levels of hormones, the suspicion of neuroendocrine tumor rises.
• Imaging investigation: to look at the detailed picture of lumps and suspicious masses, imaging investigations such as ultrasound, CT scan and MRI are done.
• Biopsy: depending on the location of the tumor, sample of small tissue is obtained through a minimally-invasive procedure like bronchoscopy for sample of lungs, endoscopy for sample of esophagus and colonoscopy for the biopsy sample from the rectum. Certain locations may require small surgery.  

What are the treatment options?

The treatment options for neuroendocrine tumors depend on their location and tumor type. These include:

• Surgery: surgical removal of the tumor is done when possible. Most surgeons try to remove as much of the tumor as they possibly can if the tumor is large, to be followed by radiation and chemotherapy. Or they remove the complete tumor and obtain tumor-free margins if the tumor is small. Thus, surgical resection is often done to get rid of the tumor.
• Targeted treatment: for specific abnormalities within the tumor cells, targeted drug therapy is done. For advanced tumors, targeted therapies are combined with radio- and chemotherapy. The idea behind using these targeted treatments is to shrink and kill as many of the tumor cells as possible.
• Chemotherapy: strong drugs called chemotherapeutic agents are used to kill the tumor cells. These drugs are given through intravenous route or the oral route. Chemotherapy helps to prevent the recurrence of a tumor if done following surgery. For tumors that are quite large and cannot be resected, chemotherapy is done prior to surgery.
• Radiation therapy: powerful beams with x-rays and protons are used to kill the tumor cells with radiation therapy. Only some of the neuroendocrine tumors respond to radiation therapy. Apart from chemotherapy, radiation therapy is also an option for non-operable advanced tumors.
• Peptide receptor radionuclide therapy (PRRT): PRRT allows radiation therapy to be delivered directly to the cancerous cells. PRRT for neuroendocrine tumors mostly involves lutetium Lu 177 dotatate (Lutathera).
• Medication: for neuroendocrine tumors that produce excessive hormones, medication to combat these hormones are often prescribed by Endocrinologist in Karachi. While these drugs do not decrease the size of the tumors, they help to control the signs and symptoms of these tumors.