Report | Question ID | Question | Discussion | Answer | Year |
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20120044 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient is diagnosed with acute monocytic leukemia in 2009 and in 2011 has biopsy confirmed granulocytic sarcoma of the cerebellum? See Discussion. |
Is this a recurrence of the patient's leukemia? In 2011, the patient is found to have several masses in the cerebellum, biopsy confirmed granulocytic sarcoma. The physician stated this is an "extramedullary relapse of leukemia." The bone marrow biopsy in 2011 was negative.
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For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Accession a single primary per Rule M3. Code histology to 9891/3 [acute monocytic leukemia] diagnosed in 2009 and primary site to C421 [bone marrow].
Per Rule M3 a single primary is reported when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. Histology 9891/3 [acute monocytic leukemia] is listed as one of the histologies in the "same lineage." Myeloid sarcoma (9930/3) diagnosed simultaneously with or after acute myeloid leukemia (9861/3) or another leukemia of the myeloid lineage (9840/3, 9865/3-9867/3, 9869/3-9874/3, 9891/3, 9895/3-9898/3, 9910/3, 9911/3 and 9931/3).
NOTE: Under the Alternate Names section of the Heme DB, granulocytic sarcoma is a synonym for myeloid sarcoma.
Per PH10, code the primary site C421 [bone marrow] and code the histology acute myeloid leukemia, NOS (9861/3) or any of the specific AML histologies (9840/3, 9865/3-9867/3, 9869/3-9874/3, 9891/3, 9895/3-9898/3, 9910/3, 9911/3 and 9931/3) when the diagnosis is myeloid sarcoma (9930/3) AND there is a simultaneous or previous diagnosis of acute myeloid leukemia.
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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20120024 | MP/H Rules/Histology--Breast: How many primaries are abstracted and what histology codes are used when a patient has two tumors, one reported as duct and lobular carcinoma and another reported as pleomorphic lobular and duct carcinoma? See Discussion. |
The pathology report indicated two tumors in the upper outer quadrant of the breast. One tumor has duct and lobular carcinoma and the other tumor has pleomorphic lobular and duct carcinoma. Per a web search, pleomorphic lobular carcinoma is a recently recognized subtype of lobular cancer. According to the MP/H Rules, Breast Equivalent Terms, Definitions, Tables and Illustrations, "pleomorphic carcinoma" is a specific type of duct carcinoma [8022/3]. This is not listed as a combined histology in Table 3. Should this be abstracted as a single primary per Rule M10, with the histology coded 8523/3 [infiltrating duct mixed with other types of carcinoma]? Or should this be abstracted as two primaries per Rule M12, with the histologies coded as 8022/3 [pleomorphic carcinoma] and 8522/3 [infiltrating duct and infiltrating lobular carcinoma]? |
This is a single primary with the histology coded as infiltrating duct and infiltrating lobular carcinoma [8522/3]. For cases diagnosed 2007 or later, the steps used to arrive at this decision are: Open the Multiple Primary and Histology Coding Rules manual. For a breast primary, start with the Breast Multiple Primary Rules because there are site specific rules for breast primaries. Start at Rule M4 because this patient has multiple tumors in the same breast. The rules are intended to be reviewed in consecutive order within the applicable Module. Abstract a single primary as tumors that are lobular [8520] and intraductal or duct are a single primary. Use the Breast Histology Coding Rules to determine the correct histology for these multiple tumors abstracted as a single primary. Start at Rule H20 as there were multiple tumors present but it is a single primary. Code the histology to 8522 [duct and lobular] when there is any combination of lobular [8520] and duct carcinoma. The Note for Rule M10 indicates Table 1 and Table 2 are used to identify specific intraductal and duct carcinomas. Referring to Table 2 (Duct 8500/3 and Specific Duct Carcinomas) note that pleomorphic carcinoma is listed as a specific type of duct carcinoma. Pleomorphic is a word that describes the cellular appearance rather than a specific histology. It is coded when that is the only description/diagnosis given (pleomorphic carcinoma/pleomorphic duct carcinoma). In this case, both duct and lobular are describing the actual histologic types. Ignore the term "pleomorphic" and code the actual histologic descriptors, ductal and lobular. We will make appropriate changes to the breast rules in the MP/H revisions so this distinction is clear. |
2012 |
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20120015 | Diagnostic confirmation--Heme & Lymphoid Neoplasms: How does one determine and code a clinical diagnosis for the diagnostic confirmation in patient diagnosed with essential thrombocythemia? See Discussion. |
The Heme DB originally stated the Definitive Diagnostic Method is coded to 8 [clinical diagnosis only] while an updated version stated it can coded as a clinical diagnosis or it can be based on the results of a bone marrow biopsy or a genetic test. The Abstractor Note section specifies this is a diagnosis of exclusion. According to a recent Web-based training seminar, the JAK-2 diagnosis would be coded 5 [positive laboratory test/marker study]. Doesn't the Definitive Diagnostic Method of a clinical diagnosis/diagnosis of exclusion mean that the diagnostic confirmation of essential thrombocythemia will always be coded as 8 [clinical diagnosis only]? Many people use code 3 for positive bone marrow biopsy and genetics (JAK-2), but the bone marrow is usually reported as only borderline or is stated to be abnormal for a person's age.
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For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the diagnostic confirmation to 8 [clinical diagnosis only] in this case.
Per the Heme DB, JAK-2 is only positive in about 50% of essential thrombocythemia (ET) patients. In addition, a positive JAK-2 test does not identify the type of myeloproliferative disease (MPN) the patient has, only the presence or absence of the JAK-2 mutation.
The WHO guidelines for diagnosing ET are: elevated platelet count over months and the elimination of other causes for an elevated platelet count (such as polycythemia vera (PV), chronic myelogenous leukemia (CML), idiopathic myelofibrosis, or myelodysplastic syndrome (MDS)); the absence of Philadelphia chromosome, BCR/ABL fusion gene; and del(5q), t(3;3)(q21;26),inv(3)(q21q26)).
Subsequently, the physician rules out any underlying causes of thrombocytosis such as an inflammation or infection, other neoplasms, and prior splenectomy.
Ultimately, there is a diagnosis of exclusion. In other words, all other causes for the elevated platelet count have been excluded. The physician assembles the information from the blood counts, bone marrow and JAK-2 testing along with the information that excludes all other diseases and makes a clinical diagnosis of 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. |
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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20120080 | MP/H Rules/Multiple primaries--Kidney, renal pelvis/Bladder: How many primaries are accessioned if the patient was diagnosed with transitional cell carcinoma in situ of the renal pelvis in October 2006, TCC in situ of the bladder in July 2008 and TCC in situ of the ureter in November 2009?. See Discussion. | Per MP/H rule M8, the TCC in situ of the bladder diagnosed in July 2008 is the same primary as the TCC in situ of the renal pelvis diagnosed in October 2006. Should the new TCC in situ of the ureter diagnosed in November 2009 be a new primary per rule M7 because the renal pelvis TCC in situ was diagnosed in 2006? Or does the 3 year time frame for rule M7 start from the date of the last recurrence (July 2008)? | Abstract two primaries for this scenario per Rule M7. The first primary is the renal pelvis in Oct. 2006; the second primary is the ureter in Nov. 2009. The bladder tumor in July 2008 is not a new primary per Rule M8.
Compare the diagnosis date of the current (most recent) tumor to the diagnosis date of the original tumor. This applies even if the patient had six occurrences in-between these dates; you still compare the current tumor to the diagnosis date of the original tumor and ignore recurrences in this process. See slide 6 of the Beyond the Basics presentation, http://www.seer.cancer.gov/tools/mphrules/training_adv/SEER_MPH_Gen_Instruc_06152007.pdf. |
2012 |
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20120034 | Primary site--Brain and CNS: How is the primary site to be coded if a clinician used an MRI to diagnose a left cerebellar venous angioma? See Discussion. |
According to the WHO Classification of Brain/CNS Tumors, code 9122/0 (venous angioma) does not appear under tumors of the cerebellum (C716). |
Venous angiomas (9122/0) are not reportable wherever they arise. The primary site for venous angioma arising in the cerebellum is C490. The combination of 9122/0 and C490 is not reportable. Venous angioma is a venous abnormality, currently referred to as a developmental venous anomaly (DVA). |
2012 |
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20130185 | Reportability/Behavior: Is HGSIL (high grade squamous intraepithelial lesion) of the vulva or vagina reportable and is it a synonym for histology code 8077/2 [squamous intraepithelial neoplasia, grade III]? |
For cases diagnosed 2018 and later HGSIL of the vulva or vagina is reportable. HGSIL is a synonym for squamous intraepithelial neoplasia, grade III. |
2013 | |
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20130013 | Reportability--Heme & Lymphoid Neoplasms: Is Mast Cell Activation Syndrome (MCAS) reportable? |
MCAS has been given an ICD-9 code of 202.60 by our medical record coders. In the Progress Notes, the physicians state this is not the same as systemic mastocytosis. There is no listing for MCAS in the Hematopoietic and Lymphoid Database. |
Mast Cell Activation Syndrome (MCAS) is not a reportable neoplasm unless it is specifically stated to be a result of a mast cell proliferative disorder that is reportable. Per our expert pathologist, MCAS is a relatively new term used for conditions in which patients experience the symptoms of mast cell mediators in the absence of an increase/proliferation of mast cells. The diagnosis of this group of disorders is based in part on a complex of symptoms and on the demonstration of no increase in mast cells. Some of these diseases are difficult to separate from mastocytosis (which is reportable). Currently, this group of disorders is not part of the systemic mastocytosis/mast cell leukemia/mast cell sarcoma spectrum. |
2013 |
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20130083 | Ambiguous terminology/Histology--Heme & Lymphoid Neoplasms: How is the histology coded if an FNA reveals high grade B-cell lymphoma, compatible with diffuse large B-cell lymphoma, and the treating physician states this is diffuse large B-cell lymphoma? See Discussion. | The FNA showed high grade B-cell lymphoma, morphologically compatible with diffuse large B cell lymphoma. Special studies state: Tumor cells are positive for Vimentin, CD45, and CD20, focally weakly positive for CD43; negative for Myeloperoxidase, CD99, AE1/AE3, CK7, CK20, S100, CD3, cyclin D1, CD34, CD5 and TTF1. The cellular findings and immunophenotype are compatible with large B-cell lymphoma.
The treating physician refers to this disease process and is treating the patient for diffuse large B-cell lymphoma. Should the histology be coded as B-cell lymphoma, NOS (9591/3) because both the FNA and the immunophenotyping use ambiguous terminology? Does the physician reference to the disease process as diffuse large B-cell lymphoma, Stage II-AE impact the histology used? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the histology to diffuse large B-cell lymphoma [9680/3] because the physician states this is a DLBCL and is treating the patient accordingly. Although the pathology report was only compatible with DLBCL, there was a subsequent clinical diagnosis that confirmed a diagnosis of DLBCL. In addition, the patient was treated for DLBCL.
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 |
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20130035 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what rule applies when a subsequent diagnosis of diffuse large B-cell lymphoma (95%) and follicular lymphoma, grade 3 (5%) is made following an original diagnosis of low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL) ? See Discussion. |
In 2011, patient presented with a large mesenteric mass, numerous other smaller mesenteric lymph nodes, moderate retroperitoneal and extensive iliac chain adenopathy greater on right; small inguinal nodes are also present mostly on right side and splenomegaly per the CT scan. Abdominal pelvic mass needle biopsies showed low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL). The patient was treated with R-CVP with unknown response. In June 2012, patient presented again for laparoscopy and lymph node biopsy for stated recurrence of lymphoma found on CT scan. A large mass was seen in mesentery of bowel. Abdominal mass biopsy showed diffuse large B-cell lymphoma (DLBCL). Abdominal mass #2 excisional biopsy showed diffuse large B-cell lymphoma, 95%, and follicular lymphoma grade 3, 5%. The majority of the tumor is now DLBCL. |
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, diffuse large B-cell lymphoma diagnosed in 2011 per Rule M7. Note 4 for Rule M7 states to change the histology code on the original abstract to the more specific histology, diffuse large B-cell lymphoma in this case. There is no time restriction for rule M7. Apply rule PH11 and code the histology as 9680/3 [DLBCL] when both DLBCL and follicular lymphoma are present in the same lymph node(s). Ambiguous terminology is not used to code a more specific histologic type per the Heme Manual. The information submitted states only that this low grade B-cell lymphoma was "most consistent with follicular lymphoma." The term "consistent with" is an ambiguous term per SEER and cannot be used to code the histology of the 2011 neoplasm as follicular lymphoma. There was no subsequent clinical statement that this patient was diagnosed with follicular lymphoma in 2011. 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. Although the ambiguous terminology on the pathology report is not used to code the histology to follicular lymphoma, had there been a subsequent clinical statement that this patient had follicular lymphoma, the histology would be coded to follicular lymphoma [9690/3]. A diagnosis of follicular lymphoma followed by a diagnosis of DLBCL more than 21 days later is a new primary per rule M12. |
2013 |