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

Use Rule PH21 to code the primary site to C778 [lymph nodes of multiple regions]. The spleen is not listed under the Primary Site(s) section in the Heme DB for follicular lymphoma. Per Rule PH21 code the primary site to 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. The spleen is a primary site for only a few lymphomas (noted in the Heme DB). Because the spleen filters blood, it is often reactive (splenomegaly) or frankly involved with the lymphoma. That reaction or involvement, however, does not affect the primary site coding. Only the involved nodes are used in coding primary site.

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 histology to 9680/3 [diffuse large B-cell lymphoma (DLBCL)]. Per the Alternate Names section in the Heme DB, B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma is one of the synonyms for for DLBCL.

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 the involved lymph nodes [C770-C779]. Per Rule PH 8, it indicates you are to code the primary site to the site of origin when lymph node(s) or lymph node region(s), tissue(s) or organs are involved. Note 2 further states that the bone marrow may or may not be involved. If the bone marrow is involved, code this information in the CS Extension field.

Per the Abstractor Notes section in the Heme DB, this is a systemic disease with widespread lymph node involvement as well as involvement of the peripheral blood. In addition, systemic involvement of extranodal sites (including lung) are often involved.

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 tumors diagnosed prior to 2007:

Yes. This case is reportable as a second primary. The rule in the SEER Program Code Manual says that invasive bladder cancers with histology codes 8120-8130 [papillary, transitional] are always coded as a recurrence and are an exception to the multiple primary rule. Squamous cell carcinoma [8070] is not a part of that exception.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.

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

Per Rule M2, this is a single primary because there is a single histology. Code histology to 9823/3 [CLL/SLL]/

The distinction of CLL vs. SLL cannot be made on bone marrow biopsy in isolation. The pathologist cannot make a diagnosis of CLL vs SLL without having peripheral blood counts available for review. If the patient was treated for CLL in the past, that may alter the peripheral counts seen in 2010 (e.g., lymphocytosis). The distinguishing feature is peripheral lymphocytosis in CLL (not seen in SLL). The disease looks the same and both will often have bone marrow involvement and lymph node involvement. If the patient had true CLL in 2005, then any subsequent lymph node (or other) biopsy consistent with CLL/SLL remains consistent with the original diagnosis of CLL. I would not change the original CLL code.

I agree with the previous response. We have to assume the 2005 diagnosis included a peripheral blood supporting that diagnosis. Otherwise, CLL and SLL look the same in nodes and marrow. The interplay between the two "diseases" is expected. This is why they are considered a single disease.

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.

Rule H8 applies, code the histology with the numerically higher ICD-O-3 code which is papillary transitional cell carcinoma, 8130.

Based on the information provided, there is a single bladder tumor, papillary urothelial carcinoma with squamous cell differentiation. Urinary sites rule H12 does not apply because this is a single tumor, not multiple tumors. In the single tumor H rules, H3 does not apply as this rule does not include papillary transitional cell carcinoma. Rule H4 is papillary carcinoma or papillary transitional cell carcinoma and refers you to Table 1. Table 1 does not list papillary urothelial carcinoma with squamous cell differentiation because there is no ICD-O-3 code for this histology. Table 1 does list transitional cell carcinoma with squamous differentiation as code 8120, however, the papillary transitional cell carcinoma is the higher code, 8130. We will review this situation for the next version of the rules.

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.

Code to mucinous (8480) since that is the only clearly invasive component of this diagnosis.

According to our pathologist consultant, "Encysted papillary carcinoma is the same thing as intracystic papillry carcinoma, which I think of as an intraductal papillary carcinoma which has greatly expanded the duct to form a cyst-like structure. It generally behaves in an in-situ rather than an invasive fashion. The only clearly invasive component is the mucinous carcinoma, which is what I would code."

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

Myeloproliferative syndrome and the myeloproliferative diseases were used in the past to describe myeloproliferative neoplasms. For cases diagnosed 2010 and forward, although the term "myeloproliferative syndrome" is not currently used to describe this disease, the synonyms "myeloproliferative syndrome" and "myeloproliferative disease" were added to the database for myelodysplastic/myeloproliferative neoplasm, unclassified [9975/3].

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 prior to 1/1/2010:Do not change the histology to small lymphocytic lymphoma (9670/3). The chronic lymphocytic leukemia has advanced/progressed and disseminated into other tissues from the blood during the last six months. If the patient presents with disease in the blood and/or bone marrow only, code to CLL. If a lymph node or other solid tissue is involved initially, code to SLL. For the case cited, the tissue involvement occurred six months after the initial diagnosis and the histology code is not changed to reflect the progression of disease.

For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.