SEER Inquiry System - Search

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

This case should be accessioned as a single primary: malignant lymphoma, mixed cell type, follicular [9691/3] diagnosed in 2003. The following describes how this determination was made.

This case is one in which the terminology for follicular lymphoma has changed over time. In 2003, follicular lymphoma was classified as small cleaved cell, large cell, or mixed cell (both small cleaved and large cell). Those designations are no longer used. This disease process is currently classified as follicular lymphoma NOS, grade 1, grade 2 or grade 3. The change was simply a change in classification/terminology.

Appendix A, Table A3 (Obsolete Terms as Defined in ICD-O-3, Lymphoid Neoplasm Obsolete Terms) should be used to determine the current term when an obsolete term is known/given. Per the Table, "Mixed cell type follicular lymphoma" is currently known as "Follicular lymphoma, grade 2" and the correct histology code is 9691/3. This is the correct histology for the 2003 primary.

Per Rule M15, the histologies must be check in the Multiple Primaries Calculator to determine the number of primaries. Enter [follicular lymphoma, grade 2 (malignant lymphoma, mixed cell type, follicular)] for Histology Code 1 and [follicular lymphoma, NOS] for Histology Code 2. The result is "Same Primary." As a result, accession a single 2003 diagnosed primary with the histology follicular lymphoma, grade 2 [9691/3] when the patient is subsequently diagnosed with follicular lymphoma, NOS.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

Report malignant myopericytoma as 8824/3 for cases diagnosed 2021 and later.

SEER will answer the question about the number of primaries to accession. Submit questions about stage to the CoC CAnswer Forum.

For cases diagnosed 2007 or later: Accession two primaries: a mixed adenocarcinoma, acinar and papillary types [8255/3] and a bronchioloalveolar carcinoma, non-mucinous [8252/3].

The steps used to arrive at this decision are:

Determine the histology code for each tumor prior to applying the Multiple Primary Rules to determine the number of primaries to accession. There are two non-small cell carcinomas, NOS; the histology code for these two tumors will be 8046/3. There is a single adenocarcinoma with acinar and papillary subtypes tumor, the histology for this tumor will be 8255. There is a single bronchioloalveolar carcinoma, non-mucinous subtype tumor; the histology for this tumor will be 8252/3.

Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three formats (i.e., flowchart, matrix or text) under the Lung Multiple Primary rules to determine the number of primaries.

Start at the MULTIPLE TUMORS module, Rule M3, because this patient has multiple tumors. The rules are intended to be reviewed in consecutive order within the module (from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient's adenocarcinoma with acinar and papillary subtypes [8255/3] and non-mucinous bronchioloalveolar carcinoma [8252/3] are multiple primaries.

Perform a second pass through the Multiple Primary rules to determine whether the two non-small cell carcinomas [8046/3] are multiple primaries or manifestations of the same primaries identified in Step 3.

Start at Rule M3 again because this patient has multiple tumors. Again, these rules are intended to be reviewed in consecutive order within the module (from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient's non-small cell carcinomas, NOS [8046/3] are a single primary when compared to the adenocarcinoma with acinar and papillary subtypes [8255/3] and non-mucinous bronchioloalveolar carcinoma [8252/3]. Both of these histologies are more specific types of non-small cell carcinoma per the Lung Histology Groups and Specific Types Chart (Chart 1).

You can also apply Rule M10 for both non-small cell carcinoma, NOS [8046/3] compared to adenocarcinoma with acinar and papillary subtypes [8255/3] and non-small cell carcinoma, NOS [8046/3] compared to non-mucinous bronchioloalveolar carcinoma [8252/3].

This is a 2006 diagnosis. The histology code is 9691/3 [follicular lymphoma, grade 2]. Do not code differentiation for hematopoietic cases.

For diagnoses 2010 and forward, a small number of cases of follicular lymphoma do have marginal zone differentiation. However, there is no code for this variant of follicular lymphoma. It would simply be coded as a follicular lymphoma because that is the most accurate histology code available. The marginal zone differentiation is not to be coded as a second primary (marginal zone lymphoma).

The histology codes for lung tumors are based on the World Health Organization Classification of Lung Tumors. Chart 1 in the MP/H Lung Equivalent Terms, Definitions, Charts, Tables and Illustrations (2007 MP/H Rules Manual) illustrates the WHO Classification of Lung Tumors.

Using Chart 1, note that papillary adenocarcinoma [8260] is located under the Adenocarcinoma (NOS) branch. The histology in question was stated to be "micropapillary adenocarcinoma" and not "papillary carcinoma." Papillary carcinoma, NOS [8050] is not actually located on the chart. However, papillary squamous cell carcinoma is listed under the Squamous Cell Carcinoma, NOS branch, histology code 8052.

Next, look up papillary carcinoma [8050] in the Morphology - Numerical listing section of the ICD-O-3. Papillary carcinoma, NOS is a Squamous Cell Neoplasm. (Refer also to SINQ 20091040.)

The key word used to determine the appropriate histology in this case is "adenocarcinoma." This is a papillary adenocarcinoma and not a papillary squamous neoplasm.

Code as a single primary. The histology is carcinoid.

Our expert pathology consultant replied as follows: "This patient clearly has a condition driving the proliferation of neuroendocrine cells. Possibilities include hypergastrinemia from a gastrinoma or from response of antral gastrin cells due to achlorhydria from long standing chronic atrophic gastritis, or multiple endocrine neoplasia (MEN1) syndrome (genetically driven). How should these cases be coded given we do not have a way to code the inciting situation. (I suspect the gastroenterologist knows what it is, but we haven't obtained that information.) We do not have an ICD-O-3 code for the underlying condition, MEN1 or hypergastrinemia. Therefore, the only choice is to code the resulting tumor, carcinoid [8240/3]."

This neoplasm is reportable because it is malignant as proven by the lymph node metastases. Code the behavior as malignant (/3) when there are lymph node metastases.