Report | Question ID | Question | Discussion | Answer | Year |
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20120012 | Histology--Heme & Lymphoid Neoplasms: How is histology coded if the pathology report shows diffuse large B-cell lymphoma arising in a small cell lymphoma - Richter's transformation, also compatible with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL)? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the histology to 9680/3 [diffuse large B-cell lymphoma (DLBCL)].
For CLL (and CLL/SLL), Richter's transformation represents when CLL changes into DLBCL. In this case, there was a biopsy that demonstrated a diagnosis of the chronic disease (CLL/SLL) transforming (Richter's transformation) into an acute disease DLBCL.
Per Rule M8, one is instructed to abstract the acute neoplasm as a single primary when both a chronic (CLL/SLL) and an acute neoplasm (diffuse large B-cell lymphoma (DLBCL)) are diagnosed simultaneously there is documentation of only one positive bone marrow biopsy, lymph node biopsy or tissue biopsy.
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. |
2012 | |
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20120011 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: Is there a timing rule used to recode histology should a more specific diagnosis of refractory anemia with excess blasts (RAEB) be confirmed after an initial diagnosis of myelodysplastic syndrome (MDS)? How many primaries are abstracted if RAEB subsequently evolves toward an acute myeloid leukemia? See Discussion. |
Facility A: 4/8/2010 Bone Marrow biopsy: Features most compatible with MDS. (No treatment administered.) 7/2/2010 Peripherial Blood: Transforming Myelodysplastic Syndrome (MDS). COMMENT: Clonal abnormality compatible with MDS/acute myeloid leukemia (AML) in all metaphases examined. (Still no treatment administered.) Facility B: 10/6/2010 Patient now presents for evaluation and treatment. Patient started on Vidaza. 10/07/10 Bone Marrow biopsy: Refractory anemia with excess blasts (RAEB-2) COMMENT: Evolution towards AML with myelodysplasia related changes considered; cytogenetic analysis reveals abnormalities most compatible with MDS and/or AML. Based on the Heme Manual and DB, the 4/8/2010 diagnosis of MDS, NOS (9989/3) is the first primary. Should the 7/2/2010 diagnosis of transforming MDS to AML (9861/3) be a new, second primary? Based on the Abstractor Note for MDS in the Heme DB for MDS, "If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific diagnosis." Based on this note, should the MDS histology code [9989/3] be changed to refractory anemia with excess blasts (RAEB-2) [9983/3] from the biopsy taken on 10/7/2010 (one day after treatment began) that revealed RAEB-2 with evolution towards AML? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. There is no time limit set to update histology to a more specific disease process if a patient has an initial NOS histology identified. Unlike solid tumors, hematopoietic and lymphoid neoplasms may take a year or more to manifest the specific disease. This is simply a part of the "disease characteristics." Abstract a single primary per M2, a single histology represents a single primary. Code the histology to 9983/3 [MDS/RAEB-2.] The Heme DB guidelines were interpreted correctly. MDS/RAEB can transform to AML and would be two separate primaries there had also been a reportable diagnosis of AML. The 7/2/2010 peripheral blood showed MDS and a clonal abnormality that was "compatible with MDS/AML." The 10/7/2010 bone marrow biopsy showed only RAEB-2 with "evolution towards AML with myelodysplasia related changes." Ambiguous terminology is only used to help determine reportability; it not used to code a more specific histology. In this case, there was only ambiguous terminology used to describe the AML. It is important to understand the implication of incorrectly assigning histology codes for hematopoietic and lymphoid neoplasm using ambiguous terminology. Using this case as an example, the patient was not treated until three months after the 7/2/2010 peripheral blood diagnosis of MDS compatible with MDS/AML. The medical literature indicates that AML, if left untreated, is usually fatal within 1-3 months. The treatment given 10/6/2010, 3 months after the "compatible with" diagnosis, was a drug used to treat MDS and not AML. The other issue with this case is that the bone marrow examination, which is more reliable than peripheral blood, showed only "evolution towards AML." This means that the bone marrow is exhibiting the changes seen in the final stages of MDS prior to progression to AML. Wait for a definitive diagnosis of AML and/or treatment for AML before abstracting the second primary. 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. |
2012 |
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20120010 | Multiple primaries/Behavior--Ovary: What is the diagnosis date and histology for the primary(ies) abstracted for a patient with a mucinous cystic borderline tumor of the ovary in 2003 and a metastatic ovarian adenocarcinoma in 2011? See Discussion. | The 2011 pathology report: Spine at L3 biopsy: metastatic adenocarcinoma. Per addendum: Prior total abdominal hysterectomy specimen from 2003 was reviewed and showed an ovarian mucinous cystic tumor of borderline malignancy which has a similar morphology to the invasive adenocarcinoma seen on current specimen.
Abdominal tissue and omental biopsy: invasive and non-invasive glandular implants compatible with origin from ovarian mucinous borderline tumor.
The final diagnosis per radiation oncologist was, "recurrent ovarian cancer." |
This is a single primary. The diagnosis date is coded to 2003 and the histology is mucinous cystadenocarcinoma [8470/3]. The bone, abdominal tissue and omentum are metastatic sites. The MP/H Rules do not apply to metastases.
This is a case where an invasive or microinvasive element was missed in the original pathology. Because the entire tumor was not sectioned and placed on slides, the pathologist used their expertise when sectioning and selecting tissue to be examined. It is not a matter of poor judgment, just a fact that it is impossible to review the tissue from the entire tumor. The behavior must be changed to malignant [/3]. |
2012 |
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20120009 | Histology--Heme & Lymphoid Neoplasms: How is the histology coded when the pathology report states the morphologic features and immunophenotype of a low grade B-cell lymphoma are most compatible with lymphoplasmacytic lymphoma or marginal zone lymphoma? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the histology to 9591/3 [B-cell lymphoma, NOS] per Rule PH28 which states that one is to code the histology when the diagnosis is
There is only one non-specific histology code mentioned, low grade B-cell lymphoma. This term is synonymous with B-cell lymphoma, NOS.
Per the Multiple Primaries Calculator, when comparing the histology 9591/3 [B-cell lymphoma, NOS] and 9671/3 [lymphoplasmacytic lymphoma], it is the same primary. When comparing the histology 9591/3 [B-cell lymphoma, NOS] and 9699/3 [marginal zone lymphoma], it is the same primary.
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. |
2012 | |
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20120008 | MP/H Rules/Recurrence--Ovary: How many primaries are accessioned if a patient was diagnosed with ovarian serous carcinoma four years ago and currently has sacral and pelvic masses positive for serous carcinoma on biopsy? Should this be disease progression or a new primary? See Discussion. |
Should this be a new primary per the MP/H Rules (Other Sites, Rule M10) because the diagnoses were made more than one year apart? Or is the new disease metastasis? The pathologist did not compare the subsequent mass biopsies with the original pathology. Is a pathologist's comparison of slides the only criteria for determining recurrent disease? This case seems to fit the definition of metastatic disease rather than a recurrence, and therefore would not be a new primary. |
Accession a single primary, the original ovarian serous carcinoma. The MP/H Rules do not apply to metastases. Metastases: When cancer cells appear in other nodes or organs that are not the primary site they are metastatic cells. Discontinuous (separate from the primary tumor) masses or cells in regional lymph nodes, distant lymph nodes, or distant sites are always metastases. In this case, the sacral and pelvic masses are distant metastases. The pathologist does not have to compare cells to the original tumor slides; the discontinuous tumor mass/cells in any site other than the primary site are metastases. Recurrence: For a disease to recur there are several criteria that must be met. First and most important, the patient must have had a disease-free interval (a tumor cannot recur if it has always been present). The other criteria are: the "new tumor" has to occur in the original primary site, it must be the same histology as the original tumor, AND must meet the timing requirements in the MPH rules for that organ/site. |
2012 |
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20120004 | Grade--Heme & Lymphoid Neoplasms: How is grade coded for a malignant non-Hodgkin lymphoma, large B-cell type, with features consistent with T-cell rich variant? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code grade to 6 [B-cell] for the histology malignant non-Hodgkin lymphoma, large B-cell type, with features consistent with T-cell rich variant [9680/3]. Under the Definition section for histology code 9680/3 it states there are morphologic variants of the disease: centroblastic, immunoblastic, plasmablastic, T-cell/histiocyte-rich, anaplastic.
Rule G3 in the Heme Manual confirms the grade listed in the Heme DB under its Grade section for the histology 9680/3. While the patient presented with a variant of DLBCL that is T-cell/histiocyte rich, it is still a B-cell phenotype. The grade is coded accordingly.
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. |
2012 | |
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20120002 | Histology/Diagnostic confirmation--Heme & Lymphoid Neoplasms: How are histology and diagnostic confirmation coded when a patient has a clinical diagnosis of lymphoma but a pathologic diagnosis of malignant neoplasm, NOS? See Discussion. |
This patient had CT scans showing extensive bilateral retroperitoneal lymphadenopathy suspicious for lymphoma and left axillary lymphadenopathy. Thin core biopsies were done of the left axillary lymph nodes and immunohistology pathology was read as malignant neoplasm with extensive necrosis. Flow cytometry analysis of the sample shows no definitive or sufficient CD45+ events for informative analysis. Karyotype analysis could not be performed on this specimen due to inadequate sample. FISH analysis using IGH break apart probe showed no evidence of clonal rearrangement in limited number of cells available for analysis. The physician's diagnosis is probable lymphoma, no further workup felt necessary because patient would not tolerate chemotherapy anyway and hospice was felt most appropriate care for patient.
The definitive diagnostic method for lymphoma, NOS is histologic confirmation, but the only histologic confirmation was of "malignant neoplasm with extensive necrosis." Should the histology and diagnostic confirmation be coded as lymphoma, NOS [9590/3] and imaging without microscopic confirmation [7] or malignancy, NOS [8000/3] and positive histology [1]? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the histology to 9590/3 [malignant lymphoma, NOS] and the diagnostic confirmation to 7 [radiology and other imaging techniques without microscopic confirmation]. Per the Diagnostic Confirmation Coding Instructions for Heme and Lymphatic Neoplasms, use code 1 when ONLY the biopsy was used to diagnose the specific histology. The biopsy only confirmed a malignancy; the scan confirmed the specific diagnosis of lymphoma.
Note that a clinical diagnosis can be a definitive diagnostic method for malignant lymphoma, NOS. In this case, the biopsy was inadequate and a more specific diagnosis could not be made by histology. Because no further work-up was pursued, this NOS diagnosis of malignant lymphoma was a clinical diagnosis only.
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. |
2012 |
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20120001 | Multiple primaries/Recurrence--Heme & Lymphoid Neoplasms: How many primaries are abstracted if a patient was diagnosed with diffuse large B-cell lymphoma in 2001 and was diagnosed with diffuse large B-cell lymphoma involving the larynx in 2011? See Discussion. | Does the medical oncologist's statement that this is a second malignancy, rather than a recurrence, given the length of the disease-free interval, affect the number of primaries abstracted? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Abstract a single primary per Rule M2; a single histology is a single primary diagnosed. The histology code for both the 2001 and 2011 diagnoses is 9680/3[diffuse large B-cell lymphoma]. Case is coded as diagnosed in 2001.
The hematopoietic physician experts say that the issue with lymphomas is that the patient may be disease-free then recur years later. Even though years have passed, this is still a recurrence or relapse. Currently, there are no molecular markers that are able to distinguish "new primaries" from recurrences. There are also no established criteria for timing rules that could be used to determine a new primary from a recurrence.
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. |
2012 |
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20110155 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient shows evidence of "MDS as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera" 18 years after a diagnosis of "thrombocytosis and probable polycythemia that progressed to probable myelofibrosis"? See Discussion | Per consultation: an 83 year old patient started on hydroxurea 18 years ago following a diagnosis of thrombocytosis and probable polycythemia. It appears the polycythemia progressed to probable myelofibrosis. The possibility of an MDS needs to be considered.
Problem list: Polycythemia with probable progression to myelofibrosis or MDS.
Bone marrow biopsy two weeks later shows some progression of dysmegakaryocytopoiesis. Patient has evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera.
On follow-up visit six weeks later: Continue to manage patient with hydroxyurea.
An additional six months later: Diagnosis is polycythemia with thrombocytosis. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case should be accessioned as a single primary. Code the histology to 9920/3 [therapy-related myelodysplastic syndrome].
The reportable diagnoses must first be separated from the non-reportable diagnoses mentioned in the consult. Thrombocytosis (NOS), polycythemia (NOS), and myelofibrosis (NOS) are not reportable terms. To verify this, look up each term in the Heme DB. No database matches list the preferred name or the alternative names as any of these NOS terms.
The reportable diagnoses are all from the post-bone marrow biopsy consult, "evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera." The subsequent notes in the consult again only refer to this as non-reportable polycythemia (NOS) or thrombocytosis (NOS). Keep in mind that this patient has been undergoing treatment with chemotherapy (hydroxyurea) for many years for polycythemia (NOS); the patient was diagnosed with polycythemia, "about 18 years ago."
According to the Subject Matter Experts, as MDS progresses, it may manifest as several different subtypes, this is a part of the disease process and abstracting each subtype would result in over-reporting this disease. This patient has a complicated history. The consult information does not adequately document whether this patient's initial diagnosis of "polycythemia" was primary polycythemia (reportable) or a secondary polycythemia (not reportable). If the patient was initially diagnosed with a primary polycythemia 18 years ago the current diagnosis of "JAK2 mutation positive polycythemia vera" would not be a new primary. The manifestation of ET may be due to the progression of MDS. In either case, this patient does have a therapy-related myelodysplastic syndrome which is the same primary as both PV and ET.
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 |
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20110154 | Behavior--Breast: Is a breast biopsy diagnosis of "ductal carcinoma in situ with focal and very early stromal invasion" an invasive tumor with a behavior code 3? |
Code the behavior to /3 [malignant, invasive]. "Stromal invasion" means the cancer is invasive. "Stroma" is the supporting connective tissue around and between ducts. It is outside the duct basement membrane. If the tumor cells extend into the stroma, the proper behavior designation for the tumor is invasive. |
2011 |