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

Code the histology to 9680/3 [diffuse large B-cell lymphoma (DLBCL)].

For CLL (and CLL/SLL), Richter's transformation represents when CLL changes into DLBCL. In this case, there was a biopsy that demonstrated a diagnosis of the chronic disease (CLL/SLL) transforming (Richter's transformation) into an acute disease DLBCL.

Per Rule M8, one is instructed to abstract the acute neoplasm as a single primary when both a chronic (CLL/SLL) and an acute neoplasm (diffuse large B-cell lymphoma (DLBCL)) are diagnosed simultaneously there is documentation of only one positive bone marrow biopsy, lymph node biopsy or tissue biopsy.

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 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 18, code the primary site to C775 [pelvic lymph nodes]. Per Rule PH18, code the primary site to the specified lymph node region when the site of lymphoma is described only as a mass. This rule also indicates that the Code pelvic lymph nodes [C775] when the lymph nodes are described as a pelvic mass.

This rule has been effect for SEER for over 20 years. It is based on the fact that a number of lymphomas that originate in nodes are not diagnosed until those nodes become matted or fixed. The presentation is then one of a "mass" in those nodal regions.

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, accession two primaries, right breast cancer diagnosed in June 2006 and a subsequent right breast primary diagnosed in December 2011.

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 exist for this primary.

Start at the MULTIPLE TUMORS module, rule M4. The rules are intended to be reviewed in consecutive order within a module. Accession two primaries, tumors diagnosed more than five (5) years apart are multiple primaries.

If the pathology report stated the tumor originated in residual breast tissue, then this is a new tumor and, therefore, a new primary per rule M5. If the pathology report stated the tumor arose in the chest wall and/or there is no designation of residual breast tissue, then this is a regional metastasis and not a new primary.

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.

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.

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

Abstract a single primary per Rule M2; a single histology is a single primary diagnosed. The histology code for both the 2001 and 2011 diagnoses is 9680/3[diffuse large B-cell lymphoma]. Case is coded as diagnosed in 2001.

The hematopoietic physician experts say that the issue with lymphomas is that the patient may be disease-free then recur years later. Even though years have passed, this is still a recurrence or relapse. Currently, there are no molecular markers that are able to distinguish "new primaries" from recurrences. There are also no established criteria for timing rules that could be used to determine a new primary from a recurrence.

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, accession a single primary.

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 Head and Neck MP rules after determining the histology of each tumor - (8070/3 [squamous cell carcinoma] and 8010/3 [carcinoma, NOS]) because site specific rules have been developed for this primary.

Start at the MULTIPLE TUMORS module, Rule M3. The rules are intended to be reviewed in consecutive order within a module. Abstract a single when one tumor is carcinoma, NOS [8010] and another tumor is a specific carcinoma, squamous cell carcinoma [8070] because the ethmoid sinus (site of origin) is not a paired site per the MP/H rules.

We will review the list of paired organs for the next edition of the MP/H Rules.

For cases diagnosed 2007 or later, accession two primaries, serous carcinoma of the right ovary and serous tubal intraepithelial carcinoma of the left fallopian tube based on the information provided.

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) and go to the Other Sites MP rules because neither the ovary nor fallopian tube have site specific rules developed.

Start at the MULTIPLE TUMORS module, Rule M3. The rules are intended to be reviewed in consecutive order within a module. The patient has multiple tumors with ICD-O-3 topography codes that are different at the third character (Cxx) and therefore this case should be accessioned as a multiple primary.

It could be helpful to know the extent of involvement noted prior to neoadjuvant therapy and debulking surgery. For example, if the patient had widely metastatic disease throughout the entire pelvis prior to the initiation of treatment, the answer may have been different.