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

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is not reportable. Per Appendix F, HLH is caused by an over stimulated immune system (infection, etc.). It is a clinical syndrome associated with a variety of underlying conditions. To be reportable, a child's diagnosis must state "fulminant hemophagocytic syndrome" to be reportable (9724/3). This is not the situation in this case.

"Hemophagocytic lymphohistiocytosis" is also listed in Appendix F: Non-Reportable List for Hematopoietic Diseases.

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.

First, make sure that the seven lymph nodes removed were actually designated to be sentinel nodes and not a combination of sentinel nodes and other regional nodes. Code sentinel nodes only when the nodes are stated to be sentinel nodes or when the surgical procedure includes the injection of dye to identify sentinel nodes.

If all seven nodes removed are sentinel nodes, follow the instructions in SINQ 20091076 and assign codes 41 [simple mastectomy] and 2 [sentinel lymph node biopsy only].

The SEER Note does not pertain to nodes designated as sentinel nodes.

Arimidex has not been approved to treat endometrial cancer. It is not prescribed for pre-menopausal women. Clarify with the physician why the drug was being used. If the physician states Arimidex was given to reduce tumor burden, code as hormone therapy.

See the SEER*Rx interactive database, http://seer.cancer.gov/seertools/seerrx/

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

There is no time limit set to update histology to a more specific disease process if a patient has an initial NOS histology identified. Unlike solid tumors, hematopoietic and lymphoid neoplasms may take a year or more to manifest the specific disease. This is simply a part of the "disease characteristics."

Abstract a single primary per M2, a single histology represents a single primary. Code the histology to 9983/3 [MDS/RAEB-2.]

The Heme DB guidelines were interpreted correctly. MDS/RAEB can transform to AML and would be two separate primaries there had also been a reportable diagnosis of AML. The 7/2/2010 peripheral blood showed MDS and a clonal abnormality that was "compatible with MDS/AML." The 10/7/2010 bone marrow biopsy showed only RAEB-2 with "evolution towards AML with myelodysplasia related changes." Ambiguous terminology is only used to help determine reportability; it not used to code a more specific histology. In this case, there was only ambiguous terminology used to describe the AML.

It is important to understand the implication of incorrectly assigning histology codes for hematopoietic and lymphoid neoplasm using ambiguous terminology. Using this case as an example, the patient was not treated until three months after the 7/2/2010 peripheral blood diagnosis of MDS compatible with MDS/AML. The medical literature indicates that AML, if left untreated, is usually fatal within 1-3 months. The treatment given 10/6/2010, 3 months after the "compatible with" diagnosis, was a drug used to treat MDS and not AML.

The other issue with this case is that the bone marrow examination, which is more reliable than peripheral blood, showed only "evolution towards AML." This means that the bone marrow is exhibiting the changes seen in the final stages of MDS prior to progression to AML. Wait for a definitive diagnosis of AML and/or treatment for AML before abstracting the second primary.

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.

This case is reportable. Code the primary site to C25.9 [pancreas, NOS] and the histology to 8240/3 [neuroendocrine tumor (NET), Grade 1].

Per the 2012 SEER Manual, code the site in which the primary tumor originated. This neoplasm arose in pancreatic tissue and will behave accordingly, even though this pancreatic tissue is not located in the usual place.

Pancreatic endocrine and neuroendocrine neoplasms are essentially the same thing. However, they are described in two different WHO classifications; the endocrine classification and the digestive system classification. The digestive system classification is more recent, and is preferred by our expert pathologist consultant. The term "neuroendocrine" is to be used now, rather than "endocrine." In the pancreas, "well differentiated endocrine tumor" is synonymous with "neuroendocrine tumor (NET) Grade 1" and is coded 8240/3.

For cases diagnosed 2007 or later, code the primary site to ovary [C56.9] and the histology to papillary carcinoma, follicular variant [8340/3].

The steps used to arrive at this decision are:

Refer to the 2012 SEER Manual for help to determine the primary site. This neoplasm is arising in a teratoma of the ovary. Per the 2012 SEER Manual, in this case the site is coded to ovary [56.9] because that is where the tumor originated. Although the teratoma contains thyroid tissue, it arose in the ovary. Teratomas are unusual in that they contain all three germ cell layers from which an embryo forms. It is not unusual to have malignancies that are usually primary to the thyroid, liver, brain, lung, etc., originate in a teratoma.

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Other Sites Histology rules because site specific rules have not been developed for this primary.

Start with the SINGLE TUMOR: INVASIVE ONLY module, rule H8. The rules are intended to be reviewed in consecutive order within a module. Code the histology as papillary carcinoma, follicular variant [8340/3].

For cases diagnosed 2007 or later, the correct histology code for both cyst associated renal cell carcinoma and cystic renal cell carcinoma is 8316/3. The histology code for cystic renal cell carcinoma, clear cell type is 8255/3.

The steps used to arrive at these decisions are:

Step 1: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Kidney Histology rules because site specific rules have been developed for this primary.

Step 2: For the first histology, cyst associated renal cell carcinoma, start at the SINGLE TUMOR module, Rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at Rule H5. According to this rule you are to use Table 1 if you have a renal cell carcinoma and mention of a more specific renal cell type. To locate Table 1, go to Kidney under the Terms & Definitions section. Per Table 1, titled Renal Cell Carcinomas and Specific Renal Cell Types, "cyst associated" is a specific type of renal cell carcinoma. Code the histology to 8316/3 [cyst associated renal cell carcinoma].

Step 3: For the second histology, cystic renal cell carcinoma start at the SINGLE TUMOR module, Rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at Rule H5. As in the previous example you are to use Table 1 if you have a renal cell carcinoma and mention of a more specific renal cell type. Per Table 1 "cystic" is a specific type of renal cell carcinoma. Code the histology to 8316/3 [cystic renal cell carcinoma].

Step 4: For the third histology, cystic renal cell carcinoma, clear cell type, start at the SINGLE TUMOR module, Rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at Rule H6 which states you are to code histology to 8255 (adenocarcinoma with mixed subtypes) when there are two or more specific renal cell carcinoma types. To determine whether "clear cell" and "cystic" are types of renal cell carcinoma use Table 1 again. According to Table 1, both cystic and clear cell are specific types of renal cell carcinoma. Code the histology as 8255/3 [adenocarcinoma with mixed subtypes].

The following answers depend on what this pathologist means by "ductal (tubular)." According to the WHO classification, tubular is not a duct subtype. Check with the pathologist if possible to determine if the intended meaning is "tubular carcinoma" or "duct carcinoma".

If the pathologist uses the expression "ductal (tubular)" as an equivalent of "tubular carcinoma": Accession two primaries, a tubular carcinoma [8211/3] and a ductal carcinoma in situ, solid type [8230/2].

For cases diagnosed 2007 and later, the steps used to arrive at this decision are:

Determine the provisional histologies of these tumors in order to apply the Multiple Primary rules. Open the Multiple Primary and Histology Coding Rules manual. For a breast primary, use the Breast Histology rules to determine the histology codes because there are site specific rules for breast primaries.

Determine the histology of in situ carcinoma, solid type ductal carcinoma in situ. Start at Rule H1. The rules are intended to be reviewed in consecutive order within the applicable Module. Code the more specific histologic term when the diagnosis is intraductal carcinoma and a type of intraductal carcinoma. Solid is a specific type of DCIS. The histology is 8230/2.

Determine the histology of the invasive carcinoma, tubular carcinoma. Start at Rule H10. Code the histology when only one histologic type is identified, Tubular carcinoma was the only type identified. The histology is 8211/3.

Go to the Breast MP rules found in the Multiple Primary and Histology Coding Rules Manual after determining the histology of each tumor. Start at the MULTIPLE TUMORS Module, Rule M4, because the patient has a single invasive tumor and separate foci of DCIS. These tumors have ICD-O-3 histology codes that are different at the third (xxx) number and are, therefore, multiple primaries.

If the pathologist uses the expression "ductal (tubular)" as an equivalent of "duct carcinoma": Accession a single primary, a duct carcinoma [8500/3].

For cases diagnosed 2007 and later, the steps used to arrive at this decision are:

Go to the Breast MP rules found in the Multiple Primary and Histology Coding Rules Manual. Start at the MULTIPLE TUMORS Module, Rule M4 because the patient has a single invasive duct carcinoma and separate foci of solid type ductal carcinoma in situ. Multiple intraductal and/or duct carcinomas are a single primary. Table 1 identifies solid type as a specific type of intraductal carcinoma.

Go to the Breast Histology rules found in the Multiple Primary and Histology Coding Rules Manual. Start at the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY Module, Rule H20. Code the invasive histology when both invasive and in situ tumors are present. Code the histology as 8500/3 [duct carcinoma].

For cases diagnosed 2007 or later, code the histology as papillary carcinoma, poorly differentiated [8260/33].

The WHO classification lists grade III papillary carcinoma as one of the synonyms for poorly differentiated thyroid carcinoma.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Other Sites Histo rules because site specific rules have not been developed for this primary.

Start with the SINGLE TUMOR: INVASIVE ONLY module, rule H8. The rules are intended to be reviewed in consecutive order within a module. Per rule H13 "phenotype" is not a term used to code a more specific histology. Moving to Rule H14 the histology is coded 8260/3 [papillary adenocarcinoma].

For cases diagnosed 2021 and later

VIN II-III is reportable based on ICD-O-3.2 which lists squamous intraepithelial neoplasia, grade II as 8077/2 making it reportable. Also see SINQ 20120094.