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Report Produced: 06/16/2026 10:50 AM

Report Question ID Question Discussion Answer Year
20120073 Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a 2011 diagnosis of systemic mastocytosis? See Discusson. Patient presented with leukopenia, anemia and monoclonal gammopathy. A bone marrow biopsy in 2011 showed systemic mastocytosis [9741/3]. A subsequent shave biopsy of abdominal skin showed histologic features that were consistent with a diagnosis of mastocytosis. A later bone marrow biopsy was subsequently performed that showed progressive systemic mastocytosis.

Updated May 2026

The Heme DB indicates the primary site for systemic mastocytosis is always coded to C421 [bone marrow].

2012
20110056 Primary site--Heme & Lymphoid Neoplasms: What is the primary site for a post-transplant lymphoproliferative disorder (PTLD) diagnosed on a brain biopsy? See Discussion. A patient was diagnosed in 6/2010 with PTLD by a brain biopsy. PTLD typically involves lymph nodes. Can the primary site for PTLD be coded to the brain?

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)]

2011
20110108 Primary site--Heme & Lymphoid Neoplasms: What is the primary site for a bone marrow biopsy positive for systemic mastocytosis that also involves the spleen and lymph nodes with associated leukocytosis, mild anemia and thrombocytopenia?

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.

2011
20100048 Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a patient diagnosed with Langerhans cell histocytosis/eosinophilic granuloma involving both the seventh rib and the right temporal bone? See Discussion. Patient was diagnosed with Langerhans cell histiocytosis/eosinophilic granuloma following a biopsy of the seventh rib on 3/22/10. On 4/13/10 the patient had a right external ear canal mass (right temporal bone) biopsy with same diagnosis. Should the primary site be coded to bone, NOS [C419]?

Updated May 2026

Per the Hematopoietic database, if a primary site of LCH is not able to be determined, default to C419. So, assign C419 for this case.

Langerhans cell histiocytosis can occur as a solitary lesion, multifocal lesions, or multisystem disease. In this case, the patient has multifocal disease of the bone.

For cases diagnosed 2010-2020, LCH is reported as 9751/3 for all diagnoses.

For cases diagnosed 2021+, only Langerhans, disseminated (multifocal, multiorgan, multisystem) is reportable as 9751/3.

2010
20120045 Primary site--Heme & Lymphoid Neoplasms: What is the primary site of a diffuse large B-cell lymphoma described on a PET and an abdominal CT scan as a large pelvic mass displacing bladder and uterus, inseparable from anus, right pelvic sidewall, cervix and bilateral ovaries and per the clinician as stage IIE? See Discussion.

PET: large pelvic mass displacing bladder and uterus, inseparable from anus, right pelvic sidewall, cervix and bilateral ovaries. Diffuse abnormal uptake within this mass as well as the adjacent structures. No regional hypermetabolic adenopathy is noted and no imaging evidence of distant metastatic disease. The PET also demonstrated diffuse abnormal uptake within the pelvic mass as well as the adjacent structures.

CT abdomen: large pelvic mass invading vagina and inseparable from the anus, right pelvic sidewall, cervix and bilateral ovaries.

MD states: "stage IIE with invasion of vagina."

Updated May 2026

Per Rule PH18, code the primary site to the specified lymph node region when the site of lymphoma is described only as a mass. This rule also indicates that the Code pelvic lymph nodes [C775] when the lymph nodes are described as a pelvic mass.

This rule has been effect for SEER for over 20 years. It is based on the fact that a number of lymphomas that originate in nodes are not diagnosed until those nodes become matted or fixed. The presentation is then one of a "mass" in those nodal regions.

 2012
20110053 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient with a several month history of refractory anemia with excess blasts (RAEB), that may or may not have been treated, who now presents with a bone marrow biopsy that is compatible with acute myeloid leukemia?

Updated May 2026

Per Rule M10, abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm AND there is a second diagnosis of an acute neoplasm after the initial clinical workup for the chronic neoplasm is completed. Two primaries should be accessioned for this case: refractory anemia with excess blasts (RAEB) [9983/3] (a chronic neoplasm), and acute myeloid leukemia [9861/3] (an acute neoplasm).

2011
20110023 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported in a patient with a November 2009 diagnosis of refractory anemia and a 10/25/2010 biopsy diagnosis of refractory anemia with excess blasts type 2 with ringed sideroblasts that the clinician indicates actually demonstrates progression to AML? See Discussion.

Refractory anemia, NOS diagnosed in November 2009. The diagnosis on a bone marrow biopsy performed on 10/25/10 is myelodysplastic syndrome - refractory anemia with excess blasts type 2 with ringed sideroblasts. Per the medical oncologist, in the 12/16/10 clinic note it states, "Pt underwent bone marrow biopsy on 10/25/10 and ultimately this marrow demonstrates progression to AML.

When applying the Hematopoietic Rules, the refractory anemia, NOS and the myelodysplastic syndrome - refractory anemia with excess blasts type 2 with ringed sideroblasts is the same primary. However, the refractory anemia NOS and the AML are multiple primaries.

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.

 2011
20110035 Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded when both a lymph node biopsy and peripheral blood are positive for CLL/SLL? See Discussion. Per Module 3, Rules PH5 and PH6 in the Hematopoietic Manual, it states that CLL has peripheral blood involvement and SLL does not.

Updated May 2026

Code the primary site to C421 [bone marrow] and histology to 9823/3 [CLL/SLL]. Per Rule PH5 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).

2011
20130014 Reportability--Heme & Lymphoid Neoplasms: Is Castleman disease reportable when diagnosed 2010 and later? When checking Castleman disease in the Hematopoietic Database, the result is a reportable histology code 9738/3. However, per an online search, Castleman disease is a very rare disorder characterized by non-cancerous growths (tumors).

Updated May 2026

Castleman disease, NOS, is not reportable for any year of diagnosis. However, when Castleman disease is diagnosed in connection with large B-cell lymphoma [9738/3], it is reportable.

2013
20100087 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for one patient with history of marginal zone lymphoma initially diagnosed in 1994, followed by a 2010 diagnosis of large B-cell lymphoma and another patient with both B-cell chronic lymphocytic leukemia/small cell lymphoma (CLL/SLL) and diffuse large B-cell (DLBCL) in 2009? See Discussion.

Case 1 - Patient has a history of marginal zone lymphoma diagnosed in 1994 with recurrences in 2007 and 2009. The patient now presents for a bone marrow biopsy in May 2010 and is found to have large B-cell lymphoma, transformation. The first primary, marginal zone lymphoma, falls under the 2009 rules and the second primary, large B-cell lymphoma, falls under the 2010 and forward rules?

Case 2 - Patient was diagnosed with B-cell CLL/SLL and a DLBCL in 2009. If the 2009 rules only apply, these are a single primary. If the patient is admitted and treated in 2010 are the rules still based on the diagnosis date?

Updated May 2026

Case 1: Accession two primaries per Rule M10 when a neoplasm is originally diagnosed as a chronic neoplasm AND there is a second diagnosis of an acute neoplasm diagnosed after the initial clinical workup of the chronic neoplasm is completed. The histology for the first primary is 9699/3 [marginal zone lymphoma] represents a chronic neoplasm and the second primary is 9680/3 [diffuse large B-cell lymphoma] is an acute neoplasm which was diagnosed more than 21 days after the first primary.

Case 2: Do not use the Heme DB and Manual rules for this case. Both diagnoses were made prior to 2010. The Heme DB and Manual are only effective for cases diagnosed 1/1/2010 and forward. Use the ICD-O-3 Hematopoietic Primaries Table to determine the number of primaries for this case. Per the Table, a second diagnosis of DLBCL [9680/3] following a diagnosis of CLL/SLL [9823/3] is one primary

 2010