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For cases diagnosed 2007 or later, accession a single primary, papillary transitional cell carcinoma of the bladder and ureter [C689, urinary system, NOS] diagnosed in 2010. 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 Urinary MP Rules because site specific rules exist for this primary.

Start at the MULTIPLE TUMORS module, rule M3. The rules are intended to be reviewed in consecutive order within a module. This patient has urothelial tumors in two or more of the listed sites (bladder, ureter and renal pelvis) diagnosed within 3 years.

When C689 is assigned because tumors of the bladder and tumors of the ureter were determined to be a single primary and the site of origin is not known (as in this example), rule M8 is applied when a subsequent tumor is diagnosed in one of the listed sites. However, if site C689 [urinary system, NOS] was assigned for other unknown urinary primary site situations, rule M8 would not be used.

Rule M8 was written specifically for urothelial tumors in the renal pelvis, ureter, bladder and urethra. Paraurethral gland [C681] and overlapping lesions of urinary organs [C688] do not belong in rule M8.

We will add this issue to the list of possible revisions for the next edition of the MP/H Rules.

For cases diagnosed 2007 or later, the correct histology code is 8244/3 [composite carcinoid]. The steps used to arrive at this decision 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 Colon Histology rules because site specific rules have been developed for this primary.

Step 2: Start at the SINGLE TUMOR module, rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at rule H9. Code the histology as 8244/3 [composite carcinoid] when the diagnosis is adenocarcinoma and carcinoid tumor.

Neuroendocrine tumor, grade 1 (G1) is synonymous with carcinoid tumor [8240/3] for the purpose of rule H9.

Accession two primaries per Rule M17, neuroendocrine carcinoma [8246/3] of the rectosigmoid junction [C199], and adenocarcinoma in a villous adenoma [8261/3] of the rectosigmoid junction [C199]. There are two tumors with ICD-O-3 histology codes that differ at the third number.

Rule M13 does not apply to neuroendocrine carcinoma. Rule M16 does not apply to this case because there are two specific histologies.

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas with low grade dysplasia, also referred to as IPMN adenoma, is not reportable.

IPMN of the pancreas is reportable when stated as "IPMN with high-grade dysplasia," or "IPMN with an associated invasive carcinoma," or  "IPMN with an associated in situ carcinoma."

The case in SINQ 20021099 is stated to have "multifocal high grade dysplasia (so-called borderline tumor and carcinoma in-situ)" and is reportable because there is an explicit statement of carcinoma in situ, not because of the reference to the presence of high grade dysplasia. It is coded 8453/2 [Intraductal papillary-mucinous carcinoma, non-invasive].

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, acute myeloid leukemia [9861/3].

MDS diagnosed prior to 1/1/2001 is not a reportable disease process. However, because MDS is currently a reportable disease process, it must be considered when trying to determine whether the AML represents a separate primary.

• If the Heme DB indicates MDS and AML represent different (separate) disease processes, only one primary is reported (i.e., AML) because the 1999 diagnosed MDS is not reportable.

• If the Heme DB indicates MDS and AML represent the same disease process, then no primaries are reported because MDS was not reportable in 1999.

Rule M2 does not apply to this case because more than one histology is mentioned in the scenario. According to the Heme DB, MDS can transform to AML. Rules M8-M13 apply to cases involving transformation. In this case, Rule M10 applies because the patient was diagnosed with a chronic neoplasm (myelodysplastic syndrome) followed greater than 21 days later by an acute neoplasm (AML).

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 lymphohistiocytosis (HLH) is not a reportable disease because it is not listed in the Heme DB.

Per our expert pathologist consultant, "HLH is a lymphocyte driven hemophagocytic syndrome which may be either genetically based or caused by over-activated lymphoid cells, often in response to a viral infection. It is an abnormal immune response and is not considered a malignant disease, and is, therefore, not reportable. It is not synonymous with EBV-positive T-cell lymphoproliferative disease of childhood (9724/3)."

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 9690/3 [follicular lymphoma, NOS].

Low grade for follicular lymphoma are not listed in the Heme DB or Manual. Because low grade can mean grade 1 or grade 2, default to follicular lymphoma, NOS [9690/3].

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 PH24, code primary site to orbit. According to Rule PH24, one is to code the primary site to the organ when lymphoma is present only in an organ. Note 2 under this rule also instructs one to capture the secondary involvement of distant lymph nodes and/or bone marrow in CS extension fields.

If the physician had not confirmed the primary site as orbit, you would have used Rule PH22 when the primary site is not indicated.

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.