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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 M13. Code the histology for the 7/31/08 diagnosis to 9680/3 [diffuse large B-cell lymphoma] and the code the histology for the 10/14/2010 diagnosis to 9698/3 [follicular lymphoma, grade 3A of 3]. Rule M13 applies to this case because the neoplasm was originally diagnosed in the blast or acute phase (DLBCL) and reverted to a less aggressive or chronic phase (follicular lymphoma) after treatment.

Per the "Transformations to" section in the Heme DB for follicular lymphoma, grade 3 transforms to diffuse large B-cell lymphoma [9680/3]. This means that the follicular lymphoma is the chronic neoplasm and that DLBCL is the acute neoplasm. In this case, the chronic neoplasm was diagnosed after the acute neoplasm was diagnosed and treated (with chemotherapy).

Do not Stop at Rule M4 because diffuse large B-cell lymphoma and follicular lymphoma (both NHL's) were not present in the same node(s) AT THE SAME TIME.

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.

This case is accessioned as a single primary. Code the histology to 9680/3 [diffuse large B-cell lymphoma] and diagnosis date to 2004. Per Rule M2, abstract as a single primary when there is a single histology.

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.

Abstract two primaries for this scenario per Rule M7. The first primary is the renal pelvis in Oct. 2006; the second primary is the ureter in Nov. 2009. The bladder tumor in July 2008 is not a new primary per Rule M8.

Compare the diagnosis date of the current (most recent) tumor to the diagnosis date of the original tumor. This applies even if the patient had six occurrences in-between these dates; you still compare the current tumor to the diagnosis date of the original tumor and ignore recurrences in this process.

See slide 6 of the Beyond the Basics presentation,

http://www.seer.cancer.gov/tools/mphrules/training_adv/SEER_MPH_Gen_Instruc_06152007.pdf.

Code the histology to malignant melanoma, superficial spreading type [8743/3] based on the final diagnosis. 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 melanoma primary, use the Melanoma Histology rules to determine the histology code because there are site specific rules for cutaneous melanomas.

Start at Rule H1. The rules are intended to be reviewed in consecutive order from Rule H1 to Rule H10. The rules are intended to be reviewed in consecutive order within the applicable Module. Code the more specific histologic term when the diagnosis is melanoma, NOS [8720] with a single specific type (i.e., superficial spreading) mentioned in the final diagnosis.

The final diagnosis takes precedence over the CAP protocol. The CAP protocol may be used when it provides additional or noncontradictory information, but that does not apply in this case.

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].

Accession a single primary, the original ovarian serous carcinoma. The MP/H Rules do not apply to metastases.

Metastases: When cancer cells appear in other nodes or organs that are not the primary site they are metastatic cells. Discontinuous (separate from the primary tumor) masses or cells in regional lymph nodes, distant lymph nodes, or distant sites are always metastases. In this case, the sacral and pelvic masses are distant metastases. The pathologist does not have to compare cells to the original tumor slides; the discontinuous tumor mass/cells in any site other than the primary site are metastases.

Recurrence: For a disease to recur there are several criteria that must be met. First and most important, the patient must have had a disease-free interval (a tumor cannot recur if it has always been present). The other criteria are: the "new tumor" has to occur in the original primary site, it must be the same histology as the original tumor, AND must meet the timing requirements in the MPH rules for that organ/site.

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 per Rule M15. The histology is coded to 9698/3 [follicular lymphoma, grade 3] diagnosed in 2006. The 2011 diagnosis of follicular lymphoma, grade 2 [9691/3] is not a new primary.

Follicular lymphoma, grade 2 [9691/3] is listed under the Same Primaries section of the Heme DB for 9698/3 [follicular lymphoma, grade 3]. To confirm this, Rule M15 indicates we are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries because none of the rules from 1-14 apply. Per the calculator, these histologies represent the same 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.

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.

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.