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Report Produced: 02/21/2026 22:22 PM

Report Question ID Question Discussion Answer Year
20130147 Primary site--Heme & Lymphoid Neoplasms: What the primary site for a diagnosis of Langerhans cell histiocytosis with multifocal multisystem involvement of the skin, chest, CNS and thyroid, but no evidence of involvement on a bone scan?

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

Code the primary site to C809 [unknown].

Langerhans Cell Histiocytosis (LCH) includes three major groups:

  • Unifocal: Slowly progressing disease, involves one organ.

  • Multifocal unisystem: Involves several organs in one system.

  • Multifocal multisystem: Involves several organs in two or more systems.

    • When the disease is both multifocal and multisystem, code the primary site to unknown [C809] because there is no way to identify the origin of the neoplasm in this situation.

    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.

    		2013
    		20130023

    Reportability--Brain and CNS: Why has reportability changed for "intradural extramedullary schwannomas"? Are all "spinal" schwannomas reportable or only those stated to be "intradural"? See Discussion.

    If intradural schwannomas are to be collected for cases diagnosed 2011 and later, why were they not included in the 2012 SEER Manual? Should collection of spinal schwannomas be postponed until the next revision of the MP/H Rules?

    The reportability of schwannomas was not initially agreed upon by the standard setters. After the issue was discussed by the CoC, NPCR and SEER Technical Workgroup and an agreement was reached. See #2 under Reportability in the Data Collection Answers from the CoC, NPCR, SEER Technical Workgroup http://www.seer.cancer.gov/registrars/data-collection.html#reportability.

    The most accurate and most current instruction is to report these spinal tumors when they arise within the spinal dura or spinal nerve roots, or when they are stated to be "intradural" or "of the nerve root." Do not report these tumors when they arise in the peripheral nerves. The peripheral nerves are the portion of nerve extending beyond the spinal dura.

    Spinal cord intradural schwannomas originate in spinal nerve roots. Spinal nerve root is best classified as spinal cord, C720.

    		2013
    		20130188 Reportability--Heme & Lymphoid Neoplasms: Is plasma cell neoplasm reportable? See Discussion. A previously submitted question in 2012 stated this was reportable, but recent answers seem to indicate this is not reportable. Please clarify whether this is reportable or not.

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

    Plasma cell neoplasm is not reportable.

    We apologize for the confusion that this has caused. The term "plasma cell neoplasm" was not included in the 2010 Heme DB and Manual. It was added to the 2012 Heme DB and Manual after repeated questions were received regarding this diagnosis. After further investigation, this term is being removed from the Manual and DB.

    According to WHO, 'Plasma cell neoplasm' is an umbrella term that includes MGUS, plasma cell myeloma, solitary plasmacytoma of bone, immunoglobulin deposition diseases, extraosseous plasmacytoma, and osteosclerotic myeloma. Of these, only plasma cell myeloma, solitary plasmacytoma of bone, and extraosseous plasmacytoma are reportable. Physicians may use the term 'plasma cell neoplasm' when they are not sure what the specific disease is. Plasma cell neoplasm is not reportable; however, follow up on these types of patients is recommended because continued evaluation is likely to determine a more specific disease. A reportable neoplasm may be diagnosed at a later date.

    Cases of plasma cell neoplasm diagnosed 2010 or later are not reportable. This change should not have taken place as a result of the update in the 2012 Manual. At this time SEER is not requiring registries to go back and review plasmacytoma or multiple myeloma cases that were collected based on this terminology.

    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.

    		2013
    		20130134

    Reportability--Heme & Lymphoid Neoplasms: According to the hematopoietic database, systemic mastocytosis is reportable; does that include INDOLENT systemic mastocytosis (which is not listed in the list of alternative names)?

    For cases diagnosed 2018 and forward, indolent systemic mastocytosis is not reportable (9741/1). Smoldering systemic mastocytosis is reportable (9741/3).

    		 2013
    		20130045 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if subsequent to a bone marrow biopsy diagnosis of acute myeloid leukemia there is an oncology consult note that indicates the pathology finding is suggestive of an underlying myelodysplastic syndrome? See Discussion

    5/14/12 Bone marrow biopsy: Acute myeloid leukemia (AML).

    5/21/12 Oncology consult: AML with 30-40% blasts and evidence of del(20q) and del(5q), is suggestive of an underlying myelodysplastic syndrome (MDS). Hence the patient has secondary AML.

    If these are two primaries, how are the diagnosis dates coded?

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

    This case is accessioned as a single primary diagnosed on 5/14/12 as acute myeloid leukemia with myelodysplasia-related (e.g., del(5q)) changes [9895/3] per Rule M2. The patient was diagnosed with a single histology, acute myeloid leukemia with myelodysplasia-related changes per the submitted information.

    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.

    		2013
    		20130031

    Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a plasmacytoma of the intervertebral disc is diagnosed in 2010 followed by a diagnosis of immature plasma cell myeloma by a right hip biopsy in 2011? See Discussion.

    The patient was diagnosed with intervertebral disc plasmacytoma and had radiation therapy to the pelvic bones in 2010. In 2011 (more than 21 days later) a right hip biopsy revealed immature plasma cell myeloma. There is clinical documentation that this is progression into myeloma. Per the Heme DB (Primary Site(s) and Definition sections) and Rule PH30, in the Heme Manual, the primary site is coded to C421 [bone marrow] and the histology is coded 9732/3 [plasma cell myeloma] when there is a clinical diagnosis of multiple myeloma and/or there is no documentation of a bone marrow biopsy or the results are unknown. This patient did have a bone marrow biopsy that indicates there are an increased plasma cells present; plasma cells represent less than 10%. The skeletal survey and bone scan did not reveal any further lesions.

    Is this progression of disease because there is only one lesion in the right hip 8 months after the diagnosis of plasmacytoma? Or is this a second primary based on the right hip biopsy that showed plasma cell myeloma and the physician's documentation of disease progression? Plasmacytomas are usually single lesions. Would this disease process have multiple lesions if they are diagnosed at different times?

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

    This case is accessioned as two primaries: Plasmacytoma diagnosed in 2010 and plasma cell myeloma diagnosed in 2011 per Rule M10. The patient has a diagnosis of a solitary plasmacytoma (chronic neoplasm) followed by a diagnosis of plasma cell myeloma (acute neoplasm) diagnosed greater than 21 days later.

    The physician is calling this a progression to plasma cell myeloma even though the bone marrow has less than 10% plasma cells, take this statement as progression or a clinical diagnosis of plasma cell myeloma.

    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.

    		 2013
    		20130033

    Histology--Heme & Lymphoid Neoplasms: How is the histology coded for a low grade B-cell lymphoma with plasmacytic differentiation?

    This answer has been corrected. Previous answer is shown below under "History."

    Assign 9591/3 for this case.

    See also SINQ 20190070.

    		 2013
    		20130007

    MP/H Rules/Histology--Colon: What rule applies and how is histology coded if a colon tumor is composed of moderately differentiated adenocarcinoma and neuroendocrine tumor, grade 1 (G1)? See Discussion.

    Intestine, large -- moderately differentiated adenocarcinoma

    Pathological stage: IIIA (T2 N1a Mx) -- Neuroendocrine tumor, G1

    Addendum comment: The results of the immunochemical study are compatible with a neuroendocrine tumor, G1.

    For cases diagnosed 2007 or later, the correct histology code is 8244/3 [composite carcinoid]. 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 formats (i.e., flowchart, matrix or text). Go to the Colon Histology rules because site specific rules have been developed for this primary.

    Step 2: Start at the SINGLE TUMOR module, rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at rule H9. Code the histology as 8244/3 [composite carcinoid] when the diagnosis is adenocarcinoma and carcinoid tumor.

    Neuroendocrine tumor, grade 1 (G1) is synonymous with carcinoid tumor [8240/3] for the purpose of rule H9.

    		2013
    		20130064 Primary site--Heme & Lymphoid Neoplasms: Are hematopoietic primaries coded to C421 [bone marrow] or C420 [blood]?

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

    Refer to the Hematopoietic Database and Manual to determine the primary site.

    Leukemias are coded to C421 [bone marrow]. The ONLY neoplasm that is coded to C420 [blood] is Waldenstrom's macroglobulinemia [9761/3].

    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.

    		2013
    		20130057 Histology--Heme & Lymphoid Neoplasms: How is the histology coded if the bone marrow biopsy favors lymphoplasmacytoid lymphoma and the physician states the diagnosis is lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia? See Discussion.

    Bone marrow biopsy: Focal bone marrow involvement with B-cell lymphoproliferative disorder. Comment: This patient has 2 monoclonal proteins in serum, IgM kappa and IgG kappa clones. The marrow does have focal involvement with a small cell lymphoproliferative disorder. A lymphoplasmacytoid lymphoma is favored.

    Flow Cytometry: Bone marrow reveals a low level, kappa-bearing-B-lymphoproliferative population that has an immunophenotype compatible with mantle cell lymphoma or related small, mature non-Hodgkin lymphoproliferative disorder.

    Physician statement: lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia.

    Per the Heme DB, the criteria to diagnosis WM is the serum paraprotein IgM. This patient's IgM was 6020 mg/dL. It was described as elevated per the physician. The physician also states the patient's IgG is elevated. According to the Heme DB, when both IgG and IgM are elevated it is indicative of LPL.

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

    Code the histology to 9671/3 [lymphoplasmactyic lymphoma (LPL)] per the Heme DB Abstractor Notes and Rule PH17. When IgG and IgM are elevated, code to lymphoplasmacytic lymphoma. Waldenstrom's macroglobulinemia is caused by increased lymphocytes which causes an increase in IgM. LPL has mixed abnormalities, both the lymphocytes and plasma cells are increased which results in an abnormally high IgM and IgG.

    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.

    		2013