What treatments are available?

Wow. That is like asking what fish are in the sea. It depends upon where you are, what your tumor type is, where your tumors are located in your body and a host of other things.

The National Comprehensive Cancer Network (NCCN) lists surgery as the first line defense for Carcinoid/NETs. Surgery can be done many ways, including open ablative to laparoscopic. They can remove the primary tumor, metastases, your gall bladder, or anything else. While “in there” they can also kill tumors via radio waves (Radio Frequency Ablation), freezing them (Cryoablation), and other methods.

Liver mets are dealt with commonly for those with Carcinoid/NETs. Surgery is common. Other treatments can include the use of radioactive spheres, embolization, chemoembolization and some procedures which blur the lines between surgery and oncology, such as Intraperitoneal Hyperthermic Chemotherapy (IPHC, or "heated" chemo).

You may go to a doctor and they declare you “inoperable”. First of all…is that doctor the surgeon? Only a surgeon can most accurately predict what can be done surgically. Secondly, not all surgeons or facilities are created equal. The surgeon may have limitations on their skills, they may not have the best equipment available to them, they may have restrictions on what they do placed by the hospital. Hospitals want “cowboys” even less than you, they hold the liability. Do not consider yourself inoperable until you have seen at least two or three carcinoid/NET skilled surgeons and they all say you are inoperable. We have some people in NJ who were considered inoperable in a large NYC hospital, yet saw another surgeon (in Philadelphia) and had fantastic outcomes. That is not to detract from the NYC surgery team, only that these cases were not right for them.

Be sure your surgeon and anesthesiologist follow the current pre-, during and postoperative protocols on using Octreotide for possible Carcinoid Crisis, which can be fatal.

The second line of defense has been recently added to the algorithm by NCCN, the use of Somatostatin analogues, such as Octreotide. Sandostatin LAR is the most commonly used drug for this role and has been shown to vastly improve quality of life and in some cases to have an effect on the tumors themselves. Dr. O’Dorisio has described Octreotide as a giant “off” switch for the excess hormones.

Going beyond this point, the lines begin to diverge depending upon your particular case, needs, and tumor location. Here are some of the options:

Chemotherapy. “Chemo” is a systemic treatment, meaning that it covers the entire body. It is useful for targeting micro tumors that may proliferate through the body, but it also affects every system of the body, some worse than others. Chemo works only on those tumors which are actively dividing at the time of administration (and a few hours after). This is why chemo destroys healthy cells that divide quickly (hair, blood, etc). In Carcinoid/NETs, the tumors are slower growing, so there are fewer tumors dividing when chemo is given, and therefore it is less effect. The typical chemo regimen is to heavily dose the body, then let the body rest for a couple of weeks. Chemo given for Carcinoid/NETs is better done with a specific dosing, usually smaller doses, more often. Specific chemo agents are also used. Dr. Richard Warner has pioneered much of this area. Some agents can be given orally.

Interferon is used, but not as often in the US as it is in Europe.

Interventional radiology therapies. Also called SirSpheres or TheraSpheres this kills tumor with radioactive material. They enter through the femoral artery in the groin, (newer techniques may allow other entry points) running a catheter to the liver. Under imaged guidance, they thread the catheter into the desired artery feeding part of the liver. Once in place, they inject an embolizing agent (small balls that block the tiny arteries). These have been laced with a radioactive isotope (usually Yttrium 90), The tumors get starved for blood and irradiated. You must be pretested for this procedure to make sure that there is no blood leakage from liver to lungs or stomach.

Traditional radiation is found to be of little use for Carcinoid/NETs.

Liver specific treatments include hepatic arterial embolization or hepatic arterial chemoembolization. Both work much the same way as the SirSpheres treatment except instead of a radiological agent, they use an embolizing agent (small balls to block the tiny blood vessels) with or without chemotherapy added. It works by cutting off the blood supply to the tumors, then (if a chemo-embo) feed them poison. It works because the liver gets most of its blood supply from the hepatic vein, where the tumors need an arterial blood supply which we cut off. Chemoembolization generally has a better success rate. Beware of old Internet information on this procedure. It took a while to “get it right” and adverse reaction statistics may be based on old data.

Targeted drug therapies. There are several new drugs out, and in trials, which target the pathways by which the cancer cells divide, replicate and die. Some of these are
---Bevacizumab, which blocks angiogenesis (the formation of new blood vessels to feed tumor growth)
---Evermolimus, which is an mTOR inhibitor, blocking signal pathways for cancer cells to replicate
---imatinib, an oral kinase inhibitor
---Sunitinib, a multi kinase inhibitor

Can you be cured? Sadly, most people with this disease are found well after the disease has progressed and spread. However, there are MANY treatment options available, even for advanced cases. With proper management, you have a very good chance of living a long time, with a good quality of life. The best cure is usually with surgery, but it is not what the surgeon can see and remove, it is what he/she can’t see and leaves behind (micro-metastases). Most “cures” are those found by accident during some other medical event.

In general, be sure to consult with a true expert on this disease. Given the complexity, you can see a surgeon and the surgeon will most likely give you a surgical solution. For the same disease, see an oncologist, and they will likely give you an oncology related solution (such as chemotherapy), while an Interventional Radiologist will likely give you a radiology type solution. None of them may be “wrong”, they are simply working within the realm in which they are comfortable, their own specialty.

Copyright 2011, 2012, 2013, 2014, 2015 NJCCN. Information here is not intended to be medical advice, but information for discussion with your medical team