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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 Rule PH25, code the primary site of the diffuse large B-cell lymphoma to C649 (kidneys) and laterality to 4 (bilateral). Per PH25, code the primary site to the organ when a lymphoma is present in an and that . This patient had involvement of an organ (bilateral kidneys) as well as regional lymph nodes for that organ. The retroperitoneal lymph nodes are regional for the kidney.

The diffuse large B-cell lymphoma is an acute transformation of the chronic lymphocytic leukemia. Because the DLBCL occurred more than 21 days after the CLL, it is a new primary per Rule M10.

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.

Code the primary site to C119 [nasopharynx] per Rule PH25.

Per our subject matter expert, use Module 7 Rule PH25 to code the primary site to an organ (nasopharynx and maxillary sinus) because an organ(s) and its regional lymph nodes are involved. The distant lymph nodes are simply part of the staging (the lymphoma has progressed to another lymph node region).

Diffuse large B cell lymphoma originating in the oral cavity and maxillofacial region is rare, but documented. The most common sites for this rare neoplasm are Waldeyer ring, tonsils, nasopharynx, base of tongue, and palatine tonsil. There are also rare cases of diffuse large B cell lymphoma originating in the maxillary sinus. The percentage of cases arising in the nasopharynx is greater than those originating in the maxillary sinus.

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.

Code grade to 6 [B-cell] for the histology malignant non-Hodgkin lymphoma, large B-cell type, with features consistent with T-cell rich variant [9680/3]. Under the Definition section for histology code 9680/3 it states there are morphologic variants of the disease: centroblastic, immunoblastic, plasmablastic, T-cell/histiocyte-rich, anaplastic.

Rule G3 in the Heme Manual confirms the grade listed in the Heme DB under its Grade section for the histology 9680/3. While the patient presented with a variant of DLBCL that is T-cell/histiocyte rich, it is still a B-cell phenotype. The grade is coded accordingly.

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.

Code the histology to 9591/3 [B-cell lymphoma, NOS].

After searching the Heme DB for the term , no B-cell leukemia/lymphoma NOS code is found. However, the diagnosis of B-cell lymphoblastic leukemia/lymphoma is found. This case scenario does not specify that this is a lymphoblastic leukemia/lymphoma; therefore, the histology code 9811/3 [B-cell lymphoblastic leukemia/lymphoma, NOS] cannot be applied.

A subsequent search of the Heme DB for the term returns "Non-Hodgkin lymphoma, NOS" [9591/3]. Under the Alternative Names section of the Heme DB, B-cell lymphoma, NOS, is a synonym for Non-Hodgkin lymphoma, NOS. Therefore, the B-cell lymphoma NOS code [9591/3] is the most appropriate histology code to use for this case.

This will be added to the next revision of the Heme DB and Manual.

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.

Epithelioid angiomyolipoma (AML) [8860/0] of the kidney is not reportable unless stated to be malignant.

If the pathologist confirms this is a malignancy, apply ICD-O-3 Rule F (Matrix principle) and assign the behavior code /3. If confirmation is received, accession the case using the morphology code 8860/3 [malignant angiomyolipoma].

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

Code the histology to 9732/3 [multiple myeloma].

Ambiguous terminology is used to accession cases (determine reportability) because it has been used for over 30 years to do so. Any deviation from using ambiguous terminology to determine case reportability would cause the reporting of incidence counts to vary. In this case, there was a reportable, ambiguous terminology diagnosis of multiple myeloma on the pathology report.

The instruction "Do not code histology based on ambiguous terminology" is intended to be used when there is a reportable and reportable stated in the diagnosis. Ambiguous terminology cannot be used to report the more specific diagnosis in cases of Heme & Lymphoid neoplasms. For example, if the pathology report final diagnosis was "Myeloproliferative neoplasm, probably Polycythemia Vera" the histology would be coded as myeloproliferative neoplasm, unclassifiable [9975/3]. The ambiguous terminology indicates that the genetic testing, immunophenotyping, etc., probably are not complete or are not diagnostic of the more specific disease. Wait to code the histology until there is a definite diagnosis given.

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 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].

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

Secondary polycythemia vera is not reportable. See Appendix F.

Primary polycythemia vera is a condition in which there is an overproduction of blood cells due to a neoplastic process. Secondary polycythemia vera is an over production of red blood cells caused by a co-morbidity, in this case, volume depletion.

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.