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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 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.

The expert pathologist recommends coding histology to 8980/3 [Carcinosarcoma] for this combination histology.

Expert consultation: The designation "carcinosarcoma" is given when the pathology shows differentiation in both the sarcomatous (rhabdomyoblastic) and carcinomatous (adenoca) elements. This is emphasized in the path for this case with the term "biphasic." The term "heterologous" mean that the sarcomatous component is of a type not normal to lung. Rhabdomyoblastic means skeletal muscle differentiation. Because skeletal muscle is not normally found in lung it is heterologous. If it were smooth muscle, it would be homologous because smooth muscle is found in lung (as a part of the bronchi).

The date of diagnosis is 2/25/10 and diagnostic confirmation is coded to 8 [clinical diagnosis only].

As the cytogenetics state, you need clinicopathologic correlation to get confirm a reportable diagnosis. There is no reportable diagnosis from the bone marrow biopsy. The cytogenetics were done (the pathologic part) and then the physician confirmed refractory cytopenia with multilineage dysplasia [9985/3] (the clinical part). The diagnostic process and the determination of a reportable diagnosis were completed when the clinician made the statement that this is refractory cytopenia with multilineage dysplasia.

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 hard-to-treat diseases such as DLBCL, the treatment plan outlined prior to treatment beginning may indicate, "The first course of treatment will be radiation and R-CHOP. If the R-CHOP does not achieve remission, we will use RICE." In other words, the first course treatment plan includes a second round of chemotherapy if the patient has not achieved a complete response after the R-CHOP and radiation. If the treatment plan was documented like this for the patient, the first course treatment includes R-CHOP, involved field radiation and RICE.

However, if there is no initial treatment plan in the medical record, all treatment provided after the date when "residual disease" or "failed to achieve remission" is documented in the medical record is either second or a subsequent course of therapy.

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.

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]."

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.

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.

This is a single primary, epithelioid sarcoma of the left upper limb, diagnosed in 2003. The sarcoma progressed over the years and the patient was never free of disease -- positive margins were documented at each surgical event. Per the 2004 SEER Manual coding rules in place at the time of pre-2007 recurrences, they would not be multiple primaries according to Rule 5, exception 1.

The occurrence in 2010 is also not a new primary. The steps used to arrive at this decision are as follows.

Open the Multiple Primary and Histology Coding Rules manual. For a soft tissue primary, use one of the three formats (i.e., flowchart, matrix or text) under the Other Sites MP rules to determine the number of primaries because soft tissue primaries do not have site specific rules.

Start with the UNKNOWN IF SINGLE OR MULTIPLE TUMORS module, Rule M1. The rules are intended to be reviewed in consecutive order within the module that applies for this case. In this module there is only one rule.

. This patient was never disease free and it is unknown if this tumor was the same tumor (single tumor) or multiple tumors. Abstract a single primary for this patient.