Neuroendocrine (Carcinoid) Tumor Treatment

Different types of treatments are available. Treatments may include surgery, chemotherapy and or radiation therapy,. Patients may also be eligible for a clinical trial. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Non-Surgical Treatment

Localized Disease

Unlike most other cancers, there is no formal staging system for gastrointestinal neuroendocrine tumors. The absence of such a staging system in part reflects the straightforward treatment approach to localized neuroendocrine tumors, which is almost always surgical resection. Adjuvant therapy has no established role in this disease, even in cases where local lymph nodes are involved and the risk of recurrence is felt to be high.

Metastatic disease

Unfortunately, surgery is unlikely to cure patients with metastatic disease (that has spread to other organs), and standard chemotherapy is often toxic and not very beneficial. A number of other treatment approaches, including the use of somatostatin analogs, surgical debulking, and ablation of hepatic metastases offer palliative benefit for such patients. When patients have disease confined to their liver, radiolabeled microspheres and chemoembolization are also performed at Roswell. Novel treatment approaches, including the use of agents targeting vascular endothelial growth factor (VEGF) and other pathways involved in neuroendocrine cancer growth and metastases may provide additional therapeutic options. Clinical trials of new targeted agents that may benefit patients with advanced neuroendocrine tumors are ongoing all over the world and also offered at Roswell.

Surgical debulking

Removal of > 85% of all visible tumor is the goal when debulking is offered and often considered in healthy individuals with indolent tumor biology.

Somatostatin analogs

Given as a rapid release short acting injection initially and followed by a once a month depot preparation injected imtramuscularly once a month , these drugs have been shown to control symptoms as well as slow down tumor cell growth. Our group has studied the effect and safety of high doses and found that they can be very effective in slowing down cancer progression and thereby minimize the need for surgeries and other procedures.

Chemotherapies

These drugs (mostly given intravenously) were the mainstay of our treatment options until recently when newer oral drugs with more favorable response rates and toxicity profiles became available. Interferon alfa (given as a subcutaneous injection) continues to be used in clinical practice for treating patients with carcinoid tumors. Click on the drugs listed below for detailed information on doses and toxicities.

Targeted Therapies/Oral therapies

Carcinoid tumors are slow growing and have activated pathways and aberrant signaling between cells. These signaling molecules/ receptors can be blocked by newer targeted therapies. Recent clinical studies of novel agents, particularly those targeting the vascular endothelial growth factor pathway (SUNITINIB) and mammalian target of rapamycin (EVEROLIMUS), have demonstrated promising activity in patients with advanced neuroendocrine tumors. Ongoing randomized studies should help better define the role these and other targeted agents will play in the future treatment of patients with this disease. Other drugs/combinations we offer here include thalidomide and temozolamide.

Surgical Treatment

Carcinoid tumors can occur anywhere throughout the gastrointestinal tract including the stomach, duodenum, jejunum, ileum, appendix, colon, and rectum. They are most common in the lower small intestine (ileum) and appendix. The primary therapy is surgical resection and reconnection of the intestine together with removal of adjacent lymph glands. Small tumors of the appendix (less than 2 cm) can be treated with a simple appendectomy while larger tumors require a more formal right hemicolectomy.

Neuroendocrine tumors of the upper gastrointestinal tract require thoughtful multidisciplinary treatment decisions. There are 3 types of carcinoids that can occur in the stomach, some of which are treated conservatively with endoscopic resection while others are treated aggressively with surgical removal of a portion of the stomach (gastrectomy). Carcinoids can also occur in the duodenum, ampulla of Vater, and the pancreas where a Whipple procedure may be required for curative treatment. Gastrinoma is a neuroendocrine tumor that secretes an acid-stimulating hormone called gastrin. These can occur in a number of locations within the upper digestive tract. Since these are relatively rare tumors patients are encouraged to consult with providers who have experience in managing neuroendocrine tumors.

Carcinoid tumors can spread to both lymph glands and the liver. Even in the presence of liver metastases there may still be benefit to resecting the primary tumor and adjacent lymph glands. This is especially true when the liver metastases can be addressed with additional therapies. There is considerable evidence that aggressive surgical resection of the bulk of the carcinoid tumors can lead to improved long-term survival.

Treatment of carcinoid liver metastases

The liver is one of the most frequent sites of spread of carcinoid tumors. Fortunately, there are many treatment options for patients with carcinoid liver metastases. When the amount of disease and tumor numbers are more limited, surgical resection can be performed. Due to the regenerative capacity of the liver, up to 70% of the liver can be removed if necessary to remove the carcinoid tumors. In certain cases this can be done minimally invasively using a laparoscopic approach thus avoiding a large incision and decreasing the recovery time.

Radiofrequency ablation

Radiofrequency ablation (RFA) and microwave ablation are minimally invasive procedures that destroy tumors with heat. Rather than removing the tumor it is killed in place and becomes scar tissue over a number of months. Carcinoid tumors respond quite well to thermal ablation. RFA can be performed through the skin by radiologists or approached either laparoscopically or via an incision by surgeons. When performed in the operating room, RFA may be done alone or in conjunction with liver resection. For example, larger tumors can be surgically removed while smaller tumors undergo RFA to preserve as much healthy liver as possible. There is good evidence that surgical resection and/or ablation of 90% or more of the liver tumors can improve long-term survival.

Surgery for neuroendocrine (islet cell) tumors of the pancreas

Neuroendocrine tumors that arise from the hormone secreting cells of the pancreas are called islet cell tumors. These can release hormones such as insulin or glucagon and are designated as functional tumors. Islet cell tumors can also be nonfunctional and without symptoms. They are generally identified on contrast-enhanced CT scans and may have a characteristic appearance that distinguishes the islet cell tumor from the more common and aggressive pancreas cancer. Surgical resection of the portion of pancreas containing the tumor is the best treatment. In tumors of the head of the pancreas a Whipple procedure is generally performed while tumors of the body and tail are usually treated with a distal pancreatectomy. Distal pancreatectomy can be performed laparoscopically (minimally invasive approach) in certain circumstances.