SEER Inquiry System - Search

Updated May 2026

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)) after the initial clinical workup for the chronic neoplasm is completed.

"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).

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 mentioned. The histology is coded to 9699/3 [MALT lymphoma]. Code the primary site to C509 [breast] per Rule PH24 which states to code the primary site to the organ when lymphoma is present only in an organ.

Unless your software has edits that prevent coding laterality for lymphomas, code the laterality as bilateral. Up to half of extranodal, extragastric MALT lymphomas occur in multiple sites, particularly in paired sites (breast is an example).

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.

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].

Code Surgery of Primary Site to 30 [Subcutaneous mastectomy] for this case.

Assign code 30 when the nipple and areolar complex are NOT removed. Assign code 40 (or higher) when the nipple and areolar complex ARE removed.

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.

Updated May 2026

First, note that myelodysplastic syndrome (MDS) is a term that includes a number of diseases. Refractory anemia, NOS and refractory anemia with ringed sideroblasts are types of MDS. These two diseases are an NOS and a more specific disease, which is accessioned as one primary per Rule M7.

Next, assess the change from refractory anemia to AML. In checking the Heme DB, AML is listed under transformations for refractory anemia with ringed sideroblasts. This patient has a chronic disease (refractory anemia with ringed sideroblasts) and an acute disease (AML). 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 diagnosed after the initial clinical workup is completed for the chronic neoplasm.

Updated May 2026

Per the Heme DB, code the primary site to C421 [bone marrow] because that is the only site listed under the Primary Site section of the Heme DB.

Under the Abstractor Notes section in the Heme DB, it indicates that the bone marrow is always involved, and the white and red pulp of the spleen may be involved with systemic mastocytosis. This is how this patient presented; therefore, the bone marrow is the primary site. The spleen is secondarily involved because the spleen cleanses the blood and the neoplastic cells have infiltrated the red and white pulp of the spleen. The same is true for the lymph nodes. Although the lymph nodes are rarely involved, they may be involved when the patient has systemic mastocytosis.

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.

This is a single primary per Rule M2 which indicates to abstract a single primary when there is a single histology. Code the histology to 9590/3 [lymphoma] and the primary site to C629 [testes. Unless your software has edits that prevent coding laterality for lymphomas, code the laterality as bilateral. Up to half of extranodal lymphomas occur in multiple sites, particularly in paired sites.

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.

Updated May 2026

Per the Heme DB, PTLD commonly involves lymph nodes, GI tract, lungs, and liver. Although CNS involvement is rare, in solid organ recipients the CNS may be the only site of involvement or may be associated with multi-organ involvement. Code the primary site to C719 [brain, NOS] and the histology to 9971/3 [post-transplant lymphoproliferative disorder (PTLD)]

For cases diagnosed 2007 or later, code one primary with histology and behavior coded to 8522/2 [intraductal carcinoma and lobular carcinoma in situ].

The steps used to arrive at this decision are as follows

Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three formats (i.e., flowchart, matrix or text) under the Breast Histology rules. The module you use depends on the behavior and number of tumors identified in the primary site. The information provided does not specify whether this was a single tumor with DCIS and LCIS or multiple tumors with DCIS and LCIS. In this case, the number of tumors does not change the histology code for this patient. For this example, assume this disease process was a single tumor.

Start at the SINGLE TUMOR: In Situ Carcinoma Only module. The rules are intended to be reviewed in consecutive order from Rule H1 to Rule H8. Stop at the first rule that applies to the case you are processing. Code the histology as 8522/2 (intraductal carcinoma and lobular carcinoma in situ) when there is a combination of in situ lobular (LCIS) [8520] and intraductal carcinoma (DCIS).

Do not code the behavior as invasive in this case. The pathologist indicated that these findings were "suspicious" but not definite in the microscopic examination. If the pathologist decided that this was truly an invasive tubular element, it would have been included in the final diagnosis.