Wiki Neuroendocrine Tumors

[Lupita1983]

Hello everyone, I have a question regarding Neuroendocrine Tumors PLEASE!

When I see neuroendocrine tumor, I go to the words before "neuroendocrine" and/or after "neuroendocrine" which depending on who my pathologist is will say something like:
Poorly differentiated, Neuroendocrine Tumor = which will automatically take me to code C7A.1
Malignant poorly differentiated neuroendocrine carcinoma = C7A.1
Neuroendocrine tumor, WHO grade III = C7A.1

Those are given to me, but what about the following:
Neuroendocrine tumor, well differentiated = I believe it should be D3A.8
Neuroendocrine tumor, WHO grade I = I believe it should be D3A.8

I have asked for clarification from my pathologists who say Neuroendocrine should automatically be coded as MALIGNANT and I don't agree because if that were the case, then why would our book have a D code for them? I believe that WHO classifications of I and II should be D3A.8 and WHO grade III should be C7A.1

But of course, it's not that easy. I have the following case:
Left kidney: Neuroendocrine tumor, well differentiated, WHO grade I, see comment.

I automatically think its my D code, but then when I go to comment is says:
Comment: IHC findings favor primary site as GI or pancreas

Now I know we don't utilize FAVOR for diagnosing, but it makes me think that this is a malignant tumor because how does a benign tumor metastasize to a different organ? Doesn't make sense. I have never heard of it but again, I am sure there are things I don't know. Any help would be appreciated. Thank you for your time.

 

[nickelclaw]

I would code the C7B- since comment states primary site unsure/unknown
If it isn't documented as malignant default to benign. We can't code from the WHO scale as it is not in our book and the pathologist are using it.

Coding of Circulatory System Diseases and Neoplastic Diseases
CHAPTER 28 Neoplasms
BEHAVIOR CLASSIFICATION
Neuroendocrine Tumors


Neuroendocrine Tumors
Neuroendocrine tumors (categories C7A, C7B, and D3A) arise from endocrine or neuroendocrine cells scattered throughout the body. The most common sites are the bronchi, stomach, small intestine, appendix, and rectum. These tumors are commonly classified according to the presumed embryonic site of origin, such as the foregut (bronchi and stomach), midgut (small intestine and appendix), and hindgut (colon and rectum).

A carcinoid tumor is a tumor that develops from enterochromaffin cells. These cells produce hormones that normally are found in the small intestine, appendix, colon, rectum, bronchi, pancreas, ovaries, testes, bile ducts, liver, and other organs. Carcinoid tumors are capable of producing these same hormones, often in large quantities, and can cause carcinoid syndrome (E34.0). Carcinoid tumors can be found throughout the body, but the majority are found in the gastrointestinal tract. Approximately 25 percent of carcinoid tumors are found in the bronchial airways and the lung. In some cases, it may not be possible to locate the site of origin of the carcinoid tumors, although symptoms of carcinoid syndrome may be present. Carcinoid tumors can present as a primary malignancy (category C7A), as a secondary or metastatic tumor (category C7B), or as a benign tumor (category D3A). Codes Z85.020, Z85.030, Z85.040, Z85.060, Z85.110, Z85.230, and Z85.520 are used to describe the history of a malignant neuroendocrine tumor that has been previously excised or eradicated with no further treatment.

When multiple endocrine neoplasia (MEN) syndrome is associated with malignant or benign neuroendocrine tumors, code also the MEN syndrome (E31.2-). However, this code is not assigned when the health record documentation does not support the condition. If there is an associated endocrine syndrome, assign the appropriate additional code, such as carcinoid syndrome (E34.0). For example:

C7A.092 + E31.21 + E34.0 Malignant carcinoid tumor of the stomach, Wermer's syndrome, and carcinoid syndrome

 

[pookergirl]

It should be noted as well that all neuroendocrine tumors are not necessarily carcinoid. If our pathologists indicated these are to be coded as malignant that is how I could code them. I also read recently that all NETs are to be considered malignant.

The following is from mdanderson.org's website:

Are all neuroendocrine tumors cancerous?​

The short answer is that neuroendocrine tumors are almost always cancerous. But each situation is unique, so it's impossible to capture the entire breadth of this disease in a simple yes or no answer.

Some neuroendocrine tumors are only classified as such because of how they appear under a microscope. But from a clinical standpoint, they almost never grow or spread, whether we do anything about them or not. Those cases only account for about 1% of all neuroendocrine tumors. But it’s important to mention them.

In some of the older systems for classifying neuroendocrine tumors, pathologists would describe them as either “benign” (non-cancerous) or “malignant” (cancerous), based on what the tumor cells looked like under a microscope. Unfortunately, those terms did not always accurately reflect whether the tumor would metastasize and behave aggressively or not, so they have been abandoned.

Today, almost all neuroendocrine tumors are considered malignant. There are just differences in how aggressive they are. Some spread easily. Others do not. But we consider all of them cancer.