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20100064 | Histology--Heme & Lymphoid Neoplasms: How is histology to be coded for acute lymphoblastic leukemia (ALL) and/or precursor B acute lymphoblastic leukemia (Pre-B ALL) for cases diagnosed 2010 and later? The Heme Database has two histology codes for this disease, both 9811/3 and 9836/3, which is the correct histology code? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code histology to 9811/3 [B lymphoblastic leukemia/lymphoma, NOS].
See the Abstractor Notes section in the Heme DB, when determining how to code histology for a case. It indicates the code 9811/3 is effective for cases diagnosed 2010 and forward. The 9836/3 is listed as obsolete and refers you to code 9811/3. Make sure to check for a specific subtype of B lymphoblastic leukemia/lymphoma [9812/3 - 9818/3] before assigning the NOS code [9811/3].
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. |
2010 | |
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20190070 | Histology--Heme & Lymphoid Neoplasms: How is the histology coded for a when the pathologist notes the low grade B-cell lymphoma raises the possibilities of extranodal marginal zone lymphoma of mucosa associated tissue (MALT lymphoma) and lymphoplasmacytic lymphoma (LPL)? See Discussion. |
Rule PH28 confirms the more specific histologies are ignored if this is truly a low grade B-cell lymphoma (i.e., non-Hodgkin lymphoma, NOS) since both MALT lymphoma and LPL are more specific types of low grade B-cell lymphomas. This leaves only a diagnosis of low grade B-cell lymphoma with plasmacytic differentiation to consider. SINQ 20130033 states a low grade B-cell lymphoma with plasmacytic differentiation should be coded as 9680/3 (diffuse large B-cell lymphoma (DLBCL)). However, DLBCL is a high grade B-cell lymphoma, not a low grade B-cell lymphoma. If the pathologist classifies this as a non-specific low grade B-cell lymphoma, and clarifies that this may represent a more specific type of low grade B-cell lymphoma (MALT lymphoma or LPL), should the histology be coded to a high-grade lymphoma (DLBCL) or non-Hodgkin lymphoma, NOS? |
Code low grade B-cell lymphoma with plasmacytic differentiation as 9591/3 (Non-Hodgkin lymphoma, NOS). Plasmacytic differentiation is commonly seen with B-cell neoplasms. If further information identifies a more specific histology, the abstract can be updated to reflect the more specific histology. In the latest WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, 4th ed., there is confirmation that DLBCL is a high grade B-cell neoplasm. We will update the SINQ question. |
2019 |
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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 | |
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20100033 | Histology--Heme & Lymphoid Neoplasms: How is this field coded for a case described as follicular lymphoma, grade 3a/3 [9698/3], with focal areas of diffuse large B cell lymphoma [9680/3] (approximately 10%)? Does the term "focal" have the same significance in Heme cases as it does for solid tumors? See Discussion. |
Per rule PH11, "Code the primary site to the site of origin (lymph node region(s), tissue, or organ) and code the histology 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."
Should the focal diffuse large B cell lymphoma be ignored in this case and rule PH11 not be applied? To apply rule PH11, does the follicular lymphoma have to be NOS [9690/3] or does PH11 include all grades of follicular lymphoma [9695/3, 9691/3, 9698/3]? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
First, you need to determine how many primaries are to be accessioned. Per Rule M4, abstract 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).
Code the histology to 9680/3 [Diffuse large B cell lymphoma] per rule PH11 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. Follicular lymphoma (FL), which is a non-Hodgkin lymphoma, includes FL, NOS, FL grade 1, FL grade 2 and FL grade 3.
Focal, foci, and focus are not used in the hematopoietic rules, meaning that you DO NOT ignore histology terms described as focal, foci, or focus.
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. |
2010 |
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20170039 | Histology--Heme & Lymphoid Neoplasms: How should histology be coded for final bone marrow diagnosis of myelodysplastic syndrome with excess blasts? See Discussion. |
This terminology is not specifically included in either alternate names list for myelodysplastic syndrome, NOS (9989/3) or refractory anemia with excess blasts (9983/3). Example: Bone Marrow Biopsy, Final Diagnosis: Consistent with involvement by myelodysplastic syndrome with excess blasts-2 (MDS EB-2). |
Assign code 9983/3 refractory anemia with excess blasts. Refractory anemia is a type of myelodyplastic syndrome. We will add this to the Heme & Lymphoid database during the next update. |
2017 |
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20200088 | Histology--Heme & Lymphoid Neoplasms: Is there an inconsistency between the histologies listed as deleted in the ICD-O-3.2 Implementation Guidelines and the obsolete histologies in the Hematopoietic and Lymphoid Neoplasms Database (Heme DB)? See Discussion. |
While we recognize the Heme DB has been the correct source for histology coding for heme and lymphoid neoplasms dating back to 2010, the ICD-O-3.2 Implementation Guidelines appear to provide incorrect coding instructions. Histologies 9670/3, 9728/3, 9729/3 and 9836/3 are listed in Table 3 - Deleted ICD-O codes in ICD-O-3.2. While we recognize these histologies have been included in this Table because they have now been deleted, it is unclear whether the Comments regarding their use listed in the 4th column of the Table is correct. For each of these histologies, the comment states the histology listed in the 1st column (ICD-O-3/3.1) should be used prior to 2021. For example, for histology 9670/3, the comment states: Cases diagnosed prior to 1/1/2021 use code 9670/3. Cases diagnosed 1/1/2021 forward use code 9823/3. However, each of these histology codes have been obsolete for cases diagnosed 1/1/2010 and later. If registrars were following the Heme DB and Heme Manual instructions (the appropriate coding source for these neoplasms), these histologies would not have been used in a decade. Should the Comments column in Table 3 be updated? Or should a Note follow the Table indicating registrars should not use these histology codes for cases diagnosed after 1/1/2010, and these histology codes have been deleted for cases diagnosed 1/1/2021? It seems misleading to indicate any of these are valid histology codes for a 2010-2020 diagnosis when the Heme DB confirms these histology codes only apply to cases diagnosed prior to 2010. |
Follow the Heme DB to determine which codes are obsolete as of 2010. These histologies were made obsolete based on the 2010 WHO Hematopoietic book and confirmation with physicians. The official changes from ICD-O-3 were not implemented until ICD-O-3.2 Also, edits will not allow these histologies to be used for cases diagnosed 2010 and later. The ICD-O tables were based on documentation from IARC ICD-O committee and may differ from practices in North America. |
2020 |
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20140084 | Histology--Heme & Lymphoid Neoplasms: Should the 1995 diagnosis be changed to plasmacytoma? A 1995 case on the central registry database indicates that MRI and bone surveys revealed a pubic ramus lesion that was biopsied. There are no other bone lesions. A bone marrow biopsy was negative. The pathologist's diagnosis at that time was "Plasma Cell Myeloma". In 2013 there was a positive bone marrow biopsy and a diagnosis of Plasma Cell Myeloma. In 2013, a history of "sequential plasmacytomas since 1995" was mentioned. Since the 1995 diagnosis was only a solitary bone lesion with no marrow involvement, it certainly seems to fit a diagnosis of plasmacytoma better than myeloma. |
Do not change the 1995 diagnosis in this case. It is best to code the histology according to information from the time of the diagnosis. Using information obtained many years later is less reliable. |
2014 | |
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20240066 | Histology--Heme & Lymphoid Neoplasms: How should histology be coded for a pathologic diagnosis of “Follicular lymphoma, diffuse pattern grade 3A of 3, equivalent to diffuse large B cell lymphoma (germinal center cell type)” when later referenced clinically as follicular lymphoma grade 3A? See Discussion. |
The WHO Classification of Hematopoietic Tumors (Blue Book), 5th edition states: “Rare cases of classic follicular lymphoma with cytological features of follicular lymphoma (FL) grade 3A can present with a prominent diffuse pattern. In the previous edition, such cases were defined as diffuse large B-cell lymphoma (DLBCL). Currently, it is uncertain whether such cases should be classified as FL or diffuse large B-cell lymphoma; and in such cases, individual treatment choices should be made in multidisciplinary conference settings taking into consideration clinical, laboratory, and imaging parameters. The presence of diffuse areas composed entirely or predominantly of large cells, however, warrants a diagnosis of diffuse large B-cell lymphoma.” Our concern is that the Hematopoietic (Heme) Manual and Database do not provide instruction for coding this scenario. We hesitate to interpret the terms “equivalent to” as ambiguous because one could argue it is unambiguous. Barring this argument, the M and H rules would indicate this is a diagnosis of diffuse large B-cell lymphoma. However, the physician does not seem to agree with the pathologist. |
Assign histology as DLBCL (9680/3) as supported by the WHO Classification of Hematolymphoid Tumors, 5th edition. It is consistent with how it would have been coded in the 4th edition. The Heme Manual and Database currently are based on the 4th edition. Physicians are using the 5th edition blue book, whereas the cancer registry field is not yet at this time. Regarding the Heme Manual and Database, this type of scenario is not covered because it is part of the 5th edition WHO Blue Book. The database cannot be updated until the 5th edition is approved for implementation (2026). |
2024 |
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20230059 | Histology--Heme and Lymphoid Neoplasms: How is histology coded for a diagnosis stated as MDS/AML (myelodysplastic syndrome/acute myeloid leukemia) per the international consensus classification (ICC)? See Discussion. |
The final diagnosis on bone marrow biopsy was high grade myeloid stem cell neoplasm, 17% blasts by differential count. The pathologist further states that this could be classified as “MDS with increased blasts (MDS-IB2) per the WHO 5th edition classification, or MDS/AML per the international consensus classification (ICC).” FISH and cytogenetics revealed a loss of 7q, but no other AML-related genetic abnormalities. The physician confirms the patient has MDS/AML. |
Updated Answer July 2024 Code histology as myelodysplastic neoplasm with increased blasts (9983/3) based on the WHO Classification of Hematolymphoid Tumors, 5th edition, Beta version 2. WHO lists MDS with increased blasts-2 (MDS-IB2) as a subtype of 9983/3. Terms coded to 9983/3 include
When differences exist between WHO and ICC, assign the histology based on the WHO Classification. |
2023 |
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20230033 | Histology--Heme and Lymphoid Neoplasms: What is the histology code for a final diagnosis of features compatible with EBV-positive nodal T-/NK-cell lymphoma? See Discussion. |
This patient has only nodal involvement for this EBV positive NK/T cell lymphoma. There is no extranodal involvement, specifically no nasal involvement. Additionally, the immunophenotyping does not match the immunophenotyping listed for histology 9719. Therefore, histology 9719 (Extranodal NK/T cell lymphoma, nasal type) does not seem applicable to this case. This patient is an adult, so histology 9724 (Systemic EBV-positive T-cell lymphoma of childhood) also does not seem to be a match. The most recent WHO Classification seems to classify these as 9702. Is this the appropriate histology for an EBV-positive nodal NK/T-cell lymphoma in an adult? |
Assign code 9702/3 for EBV+ nodal T- and NK-cell lymphoma. Primary nodal EBV-positive T- or NK-cell lymphoma is a rare lymphoma type introduced in the 2017 WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (4th Ed.) as a variant of peripheral T-cell lymphoma (PTCL). It presents more commonly in elderly and/or immunodeficient patients, lacks nasal involvement, and is more often of T-cell rather than NK-cell lineage. |
2023 |
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