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This is a 2006 diagnosis. The histology code is 9691/3 [follicular lymphoma, grade 2]. Do not code differentiation for hematopoietic cases.

For diagnoses 2010 and forward, a small number of cases of follicular lymphoma do have marginal zone differentiation. However, there is no code for this variant of follicular lymphoma. It would simply be coded as a follicular lymphoma because that is the most accurate histology code available. The marginal zone differentiation is not to be coded as a second primary (marginal zone lymphoma).

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

This case should be accessioned as one primary. Per Rule PH26, code the primary site to bone marrow (C421) when lymphoma is present only in the bone marrow. (We assumed all available physical exams, scans, and other work-up were negative for lymph node, tissue, or organ involvement.) Histology is coded to 9680/3 [Diffuse large B-cell lymphoma (DLBCL)]. Under the Alternate Names section of the Heme DB, a synonym for DLBCL is B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt 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.

Code histology to 8310/3 [clear cell adenocarcinoma]. The Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology for cases diagnosed 2007 or later.

The following steps are used to determine the histology code:

Open the Multiple Primary and Histology Coding Rules manual. For an endometrial primary, use the Other Sites Histo rules to determine the histology code because endometrium does not have site specific rules.

Go to the SINGLE TUMOR: INVASIVE ONLY module, which starts at Rule H8.

. Code clear cell adenocarcinoma [8310/3] because only one histologic type is identified.

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

Abstract the diffuse large B-cell lymphoma (Richter transformation) as a second primary per Rule M10. Rule M10 states to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (CLL) AND there is a second diagnosis of an acute neoplasm (the diffuse large B-cell lymphoma (Richter transformation)) more than 21 days after the chronic diagnosis.

"Richter transformation," also known as "Richter syndrome," is a term that indicates CLL has transformed to DLBCL. Richter syndrome is listed under the Alternate Names section in the Heme DB for DLBCL (9680/3).

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 2007 or later: The 2011 diagnosis of adenocarcinoma, NOS in the left lower lobe lung is a separate primary.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules manual. For a lung primary, use the Lung Multiple Primary rules to determine the number of primaries.

The 2010 right lung bi-lobectomy showed two separate tumors that were determined to be two primaries: clear cell adenocarcinoma [8310/3] and adenocarcinoma, NOS [8140/3]. The histology of the new left lung mass is adenocarcinoma, NOS [8140/3].

Start at Rule M3 using the MULTIPLE TUMORS module because this patient has more than one tumor. The rules are intended to be reviewed in consecutive order within the module (i.e., from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient has two tumors in each lung with ICD-O-3 histology codes that are different at the second (xxxx) digit. Abstract the LLL adenocarcinoma as a new primary [C343, 8140/3].

The patient has two tumors in each lung. The right lung showed adenocarcinoma and clear cell adenocarcinoma. The two tumors in the left lung were both adenocarcinomas. Clear cell adenocarcinoma [8310] on the right is different at the second digit from adenocarcinoma [8140] on the left. Rule M12 cannot be applied to this case, because Rule M7 is the first rule that applies to this case when processing the rules in consecutive order.

For cases diagnosed 2007 to 2017 ONLY: Code the histology as papillary urothelial carcinoma [8130].NOTE: In the CAP checklist, the statement that the tumor has a papillary configuration is a further description of this tumor. This is supported by the pathologist's stage of pTa [noninvasive papillary carcinoma]. Use the information from the CAP checklist when available. The MP/H Rules will be revised to include the term "configuration" in the specific histology terms for in situ tumors.

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 (i.e., flowchart, matrix or text) and go to the Urinary Histo rules. The module you use depends on the behavior and number of tumors identified in the primary site. In this case, the patient has a single bladder tumor per the submitted information.

Step 2: Start at Rule H1 in the Single Tumor module. The rules are intended to be reviewed in consecutive order from Rule H1 to Rule H15. Stop at the first rule that applies to the case you are processing. Stop at Rule H7. Code the histology as 8130/2 (noninvasive papillary urothelial carcinoma) when the urothelial carcinoma is stated to have a papillary configuration.

For cases diagnosed 2018 or later, refer to the Solid Tumor Rules, https://seer.cancer.gov/tools/solidtumor/

Code the behavior to /3 [malignant, invasive].

"Stromal invasion" means the cancer is invasive. "Stroma" is the supporting connective tissue around and between ducts. It is outside the duct basement membrane. If the tumor cells extend into the stroma, the proper behavior designation for the tumor is invasive.

After consultation with our expert pathologist, the decision is report this case as a single primary. There was some confusion about how to apply the current MP/H rules to this pathology report given 1) the definition of M16 and M17 and 2) the likelihood for a single endometrial primary to present with several differentiations. According to our expert pathologist, "I would regard this case as a single endometrial primary with extensive endometrial involvement and several types of differentiation, all of which are seen in endometrial carcinomas."

Next, the Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology for cases diagnosed 2007 or later.

The following steps are used to determine the histology code.

Open the Multiple Primary and Histology Coding Rules manual. For an endometrial primary, use the Other Sites Histo rules to determine the histology code because endometrium does not have site specific rules.

Start with the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY module, Rule H18. The rules are intended to be reviewed in consecutive order within the module from Rule H18 to Rule H31. You stop at the first rule that applies to the case you are processing.

Code the appropriate combination/mixed code (Table 2) when there are multiple specific histologies. GYN malignancies with multiple types of adenocarcinoma have histology coded to 8323/3 [mixed cell adenocarcinoma] per rule H30.

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.