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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.

The site/type edit identifies unlikely combinations of primary site and histologic type.

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 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.

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.

For cases diagnosed prior to 2021

According to our expert pathologist consultant, code using the terms in the pathology report. Do not try to second guess the pathologist.

1. Thymomas are /1 and not reportable unless the pathologist states "malignant". This includes A, AB, B1, B2, and B3. Any one of these types of tumors may exhibit invasion but do not code /3 based only on invasion.

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

This case should be accessioned as two primaries. The first is refractory anemia with excess blasts in 2008, and the second is AML June 2, 2010.

As for the disease occurring in 2010, granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously.

In this case, when the physician gave a provisional diagnosis of "transformation to acute leukemia" it indicated he saw the solid deposits of myeloid cells on the vocal cord. Per Rule M3, AML and myeloid (granulocytic) sarcoma appearing simultaneously are a single primary coded to AML. When the patient has AML, solid myeloid deposits (myeloid sarcoma) may appear. This is a manifestation of the AML rather than a new primary. Rule PH10 states to code the histology to AML.

Under the Transformation section in the Heme DB for refractory anemia with excess blasts (a chronic neoplasm), it indicates this disease process does transform to acute myeloid leukemia, NOS (an acute neoplasm). In this case, the chronic and acute disease processes were diagnosed at different times. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic diagnosis.

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.