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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.

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

Per the Abstractor Notes in the Heme DB, primary effusion lymphoma (PEL) is unusual in that the majority of cases arise in body cavities, such as the pleural, pericardial, and peritoneal cavities. Because there are no ICD-O-3 codes for the pleural space, pericardium, or peritoneal cavity, code the primary site to pleura C384 when the neoplasm arises in the pleural cavity, to pericardium C380 when it occurs in the pericardium, and to peritoneal cavity C482 when it occurs in the peritoneum.

Typically only one body cavity is involved. However, if multiple regions are positive for PEL as in this case, code the primary site to C809 per Rule PH27. Rule PH27 indicates one is to code the to primary site C809 when there is no evidence of lymphoma in lymph nodes AND the physician in the medical record that he/she that the lymphoma in an

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.

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

Per Rule PH30, use the Heme DB to determine the primary site and histology when rules PH1-PH29 do not apply. Code the primary site to C419 [bone, NOS], assuming there are multiple bones involved in this case. If only one bone is involved, code the primary site to the specified bone.

In the Abstractor Notes section in the Heme DB, it indicates the primary site may differ for LCH in the solitary disease and multisystem disease. This patient has multisystem disease with involvement of the bone, liver, spleen and retroperitoneum. The most common sites for multisystem involvement include three of the four above sites (bone, liver, and spleen).

Determine the primary site based on the knowledge of the usual sites of involvement for this disease, the actual sites of involvement for the case presented, and identifying which sites of involvement are likely metastatic and which are the potential primary sites. There are two potential primary sites of involvement: the bone and the retroperitoneum. Bone is a common site of involvement for LCH while the retroperitoneum is not. Code the primary site to C419 [bone, NOS] because multiple bones are involved for this patient and bone is the most common site for LCH based on the documentation in the Abstractor Notes.

The spleen and liver are typically not primary sites for this disease process. They become involved when there is multisystem involvement because they filter the blood. They are typically sites of metastatic involvement. This information will be added to the ABSTRACTOR NOTE section.

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.

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

Per PH Rule22, code the primary site to C779 [lymph nodes, NOS].

Rule PH22 is a default rule for lymphomas that is used when there is no other information regarding the primary site and the Heme DB does not indicate a primary site under its Primary Site(s) section. Rule PH27, code the primary site to unknown [C809], does not apply. Only use C809 [unknown] as the primary site when there is no evidence of lymphoma in lymph nodes AND the physician documents that the lymphoma originates in an organ(s).

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.

Because the site is specifically stated to "skin" of nipple [C44.5], this case is not reportable.

If possible, you may wish to confirm the type of biopsy performed. If the biopsy was done by FNA or needle biopsy, the biopsy tissue should contain a full-thickness of skin and subcutaneous breast (nipple) tissue. If that is the case, this tumor would likely be a reportable squamous cell carcinoma of nipple [C50.0]. If, however, this was a punch biopsy it is more likely a non-reportable squamous cell carcinoma of the skin [C44.5].

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.

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].

This is a single primary with the histology coded as infiltrating duct and infiltrating lobular carcinoma [8522/3].

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

Open the Multiple Primary and Histology Coding Rules manual. For a breast primary, start with the Breast Multiple Primary Rules because there are site specific rules for breast primaries.

Start at Rule M4 because this patient has multiple tumors in the same breast. The rules are intended to be reviewed in consecutive order within the applicable Module. Abstract a single primary as tumors that are lobular [8520] and intraductal or duct are a single primary.

Use the Breast Histology Coding Rules to determine the correct histology for these multiple tumors abstracted as a single primary. Start at Rule H20 as there were multiple tumors present but it is a single primary. Code the histology to 8522 [duct and lobular] when there is any combination of lobular [8520] and duct carcinoma.

The Note for Rule M10 indicates Table 1 and Table 2 are used to identify specific intraductal and duct carcinomas. Referring to Table 2 (Duct 8500/3 and Specific Duct Carcinomas) note that pleomorphic carcinoma is listed as a specific type of duct carcinoma. Pleomorphic is a word that describes the cellular appearance rather than a specific histology. It is coded when that is the only description/diagnosis given (pleomorphic carcinoma/pleomorphic duct carcinoma). In this case, both duct and lobular are describing the actual histologic types. Ignore the term "pleomorphic" and code the actual histologic descriptors, ductal and lobular. We will make appropriate changes to the breast rules in the MP/H revisions so this distinction is clear.

For cases diagnosed 2007 or later, accession two primaries, ductal carcinoma with mucinous differentiation [8523/3] and ductal carcinoma, NOS [8500/3]. 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 Breast MP rules because site specific rules have been developed for this primary.

Start at the MULTIPLE TUMORS module, rule M4. The rules are intended to be reviewed in consecutive order within a module. These tumors have ICD-O-3 histology codes that are different that the third (xxx) digit and are, therefore, multiple primaries.

Ductal carcinoma with mucinous differentiation is not a specific type of ductal carcinoma identified in either Table 1 or 2. (To locate Tables 1 and 2, go to Breast under the Terms & Definitions section of the manual.) It is ductal carcinoma mixed with another type of carcinoma (mucinous carcinoma in this case) see Table 3. Rule M11 does not apply.

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

This case should be accessioned as two primaries per Rule M15. Code the histology for the first primary to 9698/3 [nodular histiocytic lymphoma. Per the Alternate Names section in the Heme DB, this histology is synonymous with follicular lymphoma, grade 3. Code the histology for the second primary to 9823/3 [B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma].

Nodular histiocytic lymphoma does not transform into CLL/SLL (Transformations to), nor does CLL/SLL transform to nodular histiocytic lymphoma (Transformations from). Rule M15 indicates we are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries in this case because none of the rules from 1-14 apply. Per the calculator, the CLL/SLL is a new 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.