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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 PH1-29 do not apply, In this case, code the primary site to C449 [Skin]. According to the Abstractor Notes section in the Heme DB, the solitary form of Langerhans cell histiocytosis (LCH) [9751/3] occurs less commonly than the multisystem form of the disease; but can appear in nodes, skin and lung. This is a solitary form of LCH. Code the primary site to skin [C449].

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 M10, this case should be accessioned as two primaries when a neoplasm is originally diagnosed as a chronic neoplasm (is follicular lymphoma (FL), grade 2) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) more than 21 days after the chronic diagnosis.

Code the histology for the first primary to 9691/3 [follicular lymphoma (FL), grade 2] and the primary site to bilateral C509 [breast, NOS]. FL can start as an extranodal disease; breast is one of the sites in which it originates. It is unlikely that the lymphoma extended from the nodes to the breast, but highly likely that it extended from the breast to the nodes.

Per Rule M4, abstract the 2010 disease process as a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s). Per Rule PH11 the primary site is coded to C779 [lymph nodes, NOS] and the histology is coded to 9680/3 [diffuse large B-cell lymphoma (DLBCL)]. Rule PH11 states one is to code the primary site to the site of origin, lymph node(s), lymph node region(s), tissue(s) or organ(s) and histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.

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 9837/3 [adult T cell leukemia/lymphoma] and the primary site to C778 [lymph nodes, multiple regions]. Per Rule PH8, code the primary site to the site of origin when lymph node(s) or lymph node region(s), tissue(s) or organs are involved. A statement of "mediastinal mass" and lymphadenopathy for lymphoma primaries is equivalent to lymph node involvement. To identify the more specific primary site, you need to move to Rule PH21 that indicates you are to code the primary site as multiple lymph node regions, NOS [C778] when multiple lymph node regions, as defined by ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated.

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.

Use the Multiple Primary and Histology Coding Rules Manual for cases diagnosed 2007 or later to determine the number of primaries. This is a single primary. The 2010 diagnosis is urothelial carcinoma. The presence of "neuroendocrine features" does not change the histologic category.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules manual. Once in the manual, locate the Urinary MP rules under one of the three formats (i.e., flowchart, matrix or text). The rules are intended to be reviewed in consecutive order within the module. You stop at the first rule that applies to the case you are processing.

Start at the MULTIPLE TUMORS module start at rule M3.

. Bladder tumors with any combination of transitional cell carcinoma and papillary transitional carcinoma are a single primary.

Code as a single primary. The histology is carcinoid.

Our expert pathology consultant replied as follows: "This patient clearly has a condition driving the proliferation of neuroendocrine cells. Possibilities include hypergastrinemia from a gastrinoma or from response of antral gastrin cells due to achlorhydria from long standing chronic atrophic gastritis, or multiple endocrine neoplasia (MEN1) syndrome (genetically driven). How should these cases be coded given we do not have a way to code the inciting situation. (I suspect the gastroenterologist knows what it is, but we haven't obtained that information.) We do not have an ICD-O-3 code for the underlying condition, MEN1 or hypergastrinemia. Therefore, the only choice is to code the resulting tumor, carcinoid [8240/3]."

The histology codes for lung tumors are based on the World Health Organization Classification of Lung Tumors. Chart 1 in the MP/H Lung Equivalent Terms, Definitions, Charts, Tables and Illustrations (2007 MP/H Rules Manual) illustrates the WHO Classification of Lung Tumors.

Using Chart 1, note that papillary adenocarcinoma [8260] is located under the Adenocarcinoma (NOS) branch. The histology in question was stated to be "micropapillary adenocarcinoma" and not "papillary carcinoma." Papillary carcinoma, NOS [8050] is not actually located on the chart. However, papillary squamous cell carcinoma is listed under the Squamous Cell Carcinoma, NOS branch, histology code 8052.

Next, look up papillary carcinoma [8050] in the Morphology - Numerical listing section of the ICD-O-3. Papillary carcinoma, NOS is a Squamous Cell Neoplasm. (Refer also to SINQ 20091040.)

The key word used to determine the appropriate histology in this case is "adenocarcinoma." This is a papillary adenocarcinoma and not a papillary squamous neoplasm.

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: follicular lymphoma [9690/3] and diffuse large B-cell lymphoma [9680/3] per Rule M10. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (follicular lymphoma) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) more than 21 days after the chronic diagnosis.

Record the CHOP as the first course of treatment for the follicular lymphoma because this was the only treatment given for the chronic neoplasm (follicular lymphoma) prior to the transformation to the acute neoplasm (DLBCL). Record the chemotherapy and bone marrow transplant as first course treatment for the DLBCL.

As noted above, follicular lymphoma does transform to DLBCL. This "transformation" is actually a new disease. Follicular lymphoma is a disease in which the lymph nodes have a prominent follicular pattern; DLBCL is a disease with diffuse proliferation of large lymphoid cells. While it is true that follicular lymphoma will transform to DLBCL, this transformation indicates it becomes a different entity.

The DLBCL is coded as a second primary for several reasons: to determine the incidence of follicular lymphomas transforming to DLBCL; survival time can be calculated for the diagnosis of the more aggressive DLBCL; death will be attributed to the DLBCL (for mortality statistics) and not the follicular lymphoma

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.

The PH rules for coding lymphomas (Module 7) refer to a lymph node region as defined by the ICD-O-3. Per the Appendix C, , the ICD-O-3 lymph node region for "pelvic" is C775. In this case, there is one lymph node region involved (bilaterally). Per Rule PH20, code the specific lymph node region when multiple lymph nodes within the same lymph node region (as defined by the ICD-O-3) are involved, C775. Per Note 1 under Rule PH20, use this rule when there is bilateral involvement of lymph nodes.

This same table in Appendix C also provides information on how left and right pelvic lymph nodes are categorized by AJCC for purposes of coding stage. If the left and right pelvic lymph nodes are positive for lymphoma, it is involvement of two regions. The case is coded as Stage II.

Keep in mind that the ICD-O-3 definition of regions is used to code the primary site, while the AJCC definition of regions is used to code stage.

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:

Code 8523/2 [intraductal carcinoma mixed with other types of in situ carcinoma]. Rule H6 should apply to this case.

The wording in the Rule H6 needs to be clarified to handle a case of intraductal carcinoma with one or more subtypes that are not ductal. This will also require a modification to Table 3. A row needs to be added to the table labeled, "Intraductal and one or more of the histologies in Column 2." The Column 3 text for the newly added row would read, " Intraductal mixed with other types of carcinoma." The appropriate histology code to be reported per Column 4 would be 8523/2. This will be done in the next revision of the rules.

According to the MP/H rules, code histology to 8120/3 [urothelial carcinoma] for cases diagnosed 2007 or later. The term "glandular differentiation" is equivalent to adenocarcinoma differentiation. 8120/3 [urothelial carcinoma] would be the best way to code a "biphasic pattern with cautery-distorted urothelial carcinoma and adenocarcinoma" according to a pathologist consultant.

The steps used to arrive at this decision are as follows:

Go to the Urinary Histo rules found in the Multiple Primary and Histology Coding Rules Manual.

Start at the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY module, rule H9. Code the histology to 8120 [transitional cell/urothelial carcinoma] when there is transitional cell carcinoma with glandular differentiation.