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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 a single primary: malignant lymphoma, mixed cell type, follicular [9691/3] diagnosed in 2003. The following describes how this determination was made.

This case is one in which the terminology for follicular lymphoma has changed over time. In 2003, follicular lymphoma was classified as small cleaved cell, large cell, or mixed cell (both small cleaved and large cell). Those designations are no longer used. This disease process is currently classified as follicular lymphoma NOS, grade 1, grade 2 or grade 3. The change was simply a change in classification/terminology.

Appendix A, Table A3 (Obsolete Terms as Defined in ICD-O-3, Lymphoid Neoplasm Obsolete Terms) should be used to determine the current term when an obsolete term is known/given. Per the Table, "Mixed cell type follicular lymphoma" is currently known as "Follicular lymphoma, grade 2" and the correct histology code is 9691/3. This is the correct histology for the 2003 primary.

Per Rule M15, the histologies must be check in the Multiple Primaries Calculator to determine the number of primaries. Enter [follicular lymphoma, grade 2 (malignant lymphoma, mixed cell type, follicular)] for Histology Code 1 and [follicular lymphoma, NOS] for Histology Code 2. The result is "Same Primary." As a result, accession a single 2003 diagnosed primary with the histology follicular lymphoma, grade 2 [9691/3] when the patient is subsequently diagnosed with follicular lymphoma, NOS.

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 the histology to 8082/3 [urothelial carcinoma, plasmacytoid].

The Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology for cases diagnosed 2007 or later. Unfortunately, in this case there is no current rule that directs you appropriately to Table 1 from Rule H7 to find this histology combination. We need to add an example under Rule H7 that instructs you to "See Table 1" for an urothelial carcinoma diagnosis that mentions a more specific cell type (e.g., plasmacytoid). We will add a reference to Table 1 in Rule H7 in the updates to MP/H Rules.

Histology is sarcomatoid carcinoma [8033/3]. This case was sent to the lung physician experts because of the difficulty in trying to apply the current MP/H rules. Their rationale for the coding decision follows:

"This pathologist has diagnosed a sarcomatoid carcinoma, and then listed all of the subtypes associated with that diagnosis. I would go with the primary diagnosis, sarcomatoid carcinoma. The inclusion of squamous cell differentiation would exclude spindle cell and giant cell as diagnoses, so the pathologist is using them descriptively. We have no basis for picking one of the subtypes and sarcomatoid carcinoma covers all of the diagnoses given."

See the glossary in the Lung Equivalent Terms and Definitions for Sarcomatoid carcinoma: A group of tumors that are non-small cell in type and contain spindle cells and/or giant cells. Depending on the histologic features the tumor may be designated: pleomorphic carcinoma [8022/3]; spindle cell carcinoma [8032/3]; giant cell carcinoma [8031/3], carcinosarcoma [8980/3]; or pulmonary blastoma [8972/3].

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

Code the primary site to C421 [bone marrow] and histology to 9823/3 [CLL/SLL]. Per Rule there may be involvement of bone marrow AND lymph node(s), lymph node region(s), organ(s), or tissue(s) but as long as the peripheral blood and/or bone marrow are involved, the primary site is bone marrow (C421).

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.

RAEB I and RAEB II [9983/3] have the same histology code per the Heme DB. They are synonyms. Per Rule M2 one abstracts a single primary when there is a single histology. There is no change to report to the state regarding histology.

The I and II designators indicate the number of blasts in the bone marrow. In RAEB, the number of blasts measures the severity of the disease and is also a predictor of the chance of a genetic transformation to AML.

In this case, the patient's disease has progressed to a more severe phase - similar to a solid tumor progressing from Stage II to Stage III.

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.

When there is a question of whether the SEER MP/H Rules or Hematopoietic and Lymphoid Neoplasm Rules apply, check the histology and refer to the Case Reportability Instructions in the Hematopoietic and Lymphoid Neoplasm Manual. All ICD-O-3 morphology codes in the range 9590 - 9992 are included in the Hematopoietic Rules. Mycosis Fungoides [9700/3] is included in this range. Therefore, the SEER MP/H Rules do not apply to mycosis fungoides.

This case should be accessioned as a single primary: mycosis fungoides [9700/3] of the skin, NOS [C449]. Per Rule M2 abstract a single primary when there is a single histology.

Note that in the Primary Site(s) section of the Heme DB, it states the primary site must always be coded to skin (C440 - C449) for mycosis fungoides. Because the primary site is stated in this section of the Heme DB, it is not necessary to use the Primary Site Rules to determine the primary site. Code the primary site to C449 [skin, NOS] because the patient has multiple sites of skin involvement and there is no documentation indicating which subsite of skin was the origin of the mycosis fungoides.

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, low-grade mucinous tumors of the appendix are a /1, borderline/uncertain behavior, and not reportable. These tumors do spread to the peritoneal cavity (pseudomyxoma peritonei). This spread, or deposits, or implants are also borderline/uncertain behavior and do not make the appendiceal tumor reportable. By contrast, a high-grade mucinous tumor of the appendix may produce malignant/invasive pseudomyxoma peritonei. When the pseudomyxoma peritonei are diagnosed as invasive or malignant, the mucinous tumor in the appendix is reportable as a /3.

SEER will answer the question about the number of primaries to accession. Submit questions about stage to the CoC CAnswer Forum.

For cases diagnosed 2007 or later: Accession two primaries: a mixed adenocarcinoma, acinar and papillary types [8255/3] and a bronchioloalveolar carcinoma, non-mucinous [8252/3].

The steps used to arrive at this decision are:

Determine the histology code for each tumor prior to applying the Multiple Primary Rules to determine the number of primaries to accession. There are two non-small cell carcinomas, NOS; the histology code for these two tumors will be 8046/3. There is a single adenocarcinoma with acinar and papillary subtypes tumor, the histology for this tumor will be 8255. There is a single bronchioloalveolar carcinoma, non-mucinous subtype tumor; the histology for this tumor will be 8252/3.

Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three formats (i.e., flowchart, matrix or text) under the Lung Multiple Primary rules to determine the number of primaries.

Start at the MULTIPLE TUMORS module, Rule M3, because this patient has multiple tumors. The rules are intended to be reviewed in consecutive order within the module (from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient's adenocarcinoma with acinar and papillary subtypes [8255/3] and non-mucinous bronchioloalveolar carcinoma [8252/3] are multiple primaries.

Perform a second pass through the Multiple Primary rules to determine whether the two non-small cell carcinomas [8046/3] are multiple primaries or manifestations of the same primaries identified in Step 3.

Start at Rule M3 again because this patient has multiple tumors. Again, these rules are intended to be reviewed in consecutive order within the module (from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient's non-small cell carcinomas, NOS [8046/3] are a single primary when compared to the adenocarcinoma with acinar and papillary subtypes [8255/3] and non-mucinous bronchioloalveolar carcinoma [8252/3]. Both of these histologies are more specific types of non-small cell carcinoma per the Lung Histology Groups and Specific Types Chart (Chart 1).

You can also apply Rule M10 for both non-small cell carcinoma, NOS [8046/3] compared to adenocarcinoma with acinar and papillary subtypes [8255/3] and non-small cell carcinoma, NOS [8046/3] compared to non-mucinous bronchioloalveolar carcinoma [8252/3].

Donor lymphocyte infusion (DLI) is coded as immunotherapy. The lymphocytes are donated by the same person who donated the original stem cell transplant. The lymphocyte infusion creates an immune response in which the T-cells are activated to attack the cancer cells.

See "Treatments" for CLL/SLL (9823/3)