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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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20120050 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what histology codes apply if a patient has a 1998 diagnosis of essential thrombocythemia and a recent clinical diagnosis of secondary myelofibrosis? See Discussion. | The patient has a history of essential thrombocythemia (ET) since 1998. This has been treated daily with aspirin. A recent bone marrow biopsy was consistent with myeloproliferative disorder with excess blasts, marked extensive reticulin marrow fibrosis with osteosclerosis, excess blasts (11%) in the marrow aspirate and peripheral blood. JAK2 mutation was present in a small minority of cells. The physician stated patient was, "considered to have secondary myelofibrosis and was started on Jakafi." | Updated April 2026 For cases diagnosed 2010 and forward, access the Hematopoietic Database (Heme DB) at http://seer.cancer.gov/seertools/hemelymph.
Per the Heme DB, entry Myelofibrosis, a secondary myelofibrosis is not a reportable case. Secondary myelofibrosis is not listed as a synonym for primary myelofibrosis in the Heme DB. The term "secondary myelofibrosis" means that the myelofibrosis was caused by, in this case, the essential thrombocythemia.
The diagnosis "consistent with myeloproliferative disorder" is also not a new reportable diagnosis. "Myeloproliferative disorder" refers to a group of diseases (an NOS category) that includes essential thrombocythemia, which was originally diagnosed in 1998, prior to reportability for this disease type.
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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20120063 | Reportability--Pancreas: Are neuroendocrine "tumors" reportable and are they synonymous with neuroendocrine "carcinoma"? See Discussion. | Example: Pancreatic mass that probably represents a neuroendocrine tumor is staged as cT2N0M0. | According to the World Health Organization (WHO) pancreatic neuroendocrine tumors (NET) are malignant. They are reportable.
For pancreas primaries, code NET, G1 (well differentiated) to 8240/3; NET G2 (moderately differentiated) to 8249/3; and nonfunctional NET, GI or G2 to 8150/3. The histology code for neuroendocrine carcinoma (NEC) is 8246/3, large cell NEC is 8013/3 and small cell NEC is 8041/3. |
2012 |
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20120089 | MP/H Rules/Histology--Colon: The final diagnosis on a path report for a colon specimen says: Is a colon specimen final diagnosis of carcinoma in situ in a serrated adenoma coded to 8010/2, 8210/2 or 8213/2? | For cases diagnosed 2007 or later, code the histology as 8213/2 [carcinoma in situ in a serrated adenoma].
The steps used to arrive at this decision are:
: Apply ICD-O-3 rule F (Matrix principle) and assign the behavior code /2 when the behavior assigned by the pathologist differs from the usual behavior as given in the ICD-O-3.
: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text) and go to the Colon Histology rules.
: Start at the SINGLE TUMOR module, Rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at rule H4. Code the histology as 8213/2.
Note: The histology 8213 (adenocarcinoma in serrated adenoma) will be added to rule H4 in the next revision. |
2012 | |
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20130092 | Reportability--Head & Neck: What are the correct site and histology codes if a glomus tympanicum tumor of the middle ear is reportable? |
Glomus tympanicum tumors of the middle ear are not reportable. The 2005 WHO Classification of Head and Neck Tumors classified these tumors as a borderline [/1] behavior and recorded them in the ICD-O-3 with histology code 8690 [glomus jugulare tumor, NOS]. According to WHO, "the distinction between jugular and tympanic paragangliomas can easily be made in the patient by modern imaging methods ... the jugular neoplasm is identified as arising from the jugular bulb region ... while the tympanic neoplasm is confined to the middle ear." Benign and borderline neoplasms of the middle ear [C301] are not reportable. The middle ear is not a reportable CNS site for benign and borderline tumors. |
2013 | |
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20130040 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what rule applies when a patient has a history of chronic myeloid leukemia diagnosed in 1993 followed by a diagnosis of acute myeloid leukemia arising in chronic myelogenous leukemia, blast phase? See Discussion. |
12/1993 Bone marrow biopsy: Chronic myeloid leukemia t(9;22) (q34;q11).
09/2011 Bone marrow biopsy: Abnormal cytogenetic & FISH support persistent involvement by chronic myelogenous leukemia.
12/2011 Peripheral blood, flow cytometry: Involvement by acute myeloid leukemia arising in chronic myelogenous leukemia (CML, blast phase, 30% blasts by manual diff.).
Is the 12/2011 diagnosis a new primary? If not, why don't Rules M8-M13 apply when the Heme DB Abstractor Notes section for CML indicates that when there is a chronic, accelerated and blast phase that develops later in the course of the disease, change the histology code to the more specific diagnosis?
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For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case should be accessioned as a multiple primary: chronic myelogenous leukemia t(9;22) (q34;q11) [9863/3] diagnosed in 1993 and acute myeloid leukemia [9861/3] diagnosed in 2011 per Rule M15.
Use the diagnosis date to determine the appropriate manual and rules to follow to determine the histologies for this case. To determine the histology of the 1993 diagnosis, use the ICD-O-2. The Heme Manual & DB will be used to determine the number of primaries and the histology of the 2011 diagnosis of AML.
Rules M8-M13 in the Heme Manual cannot be applied to this case because no transformation occurred. CML does not transform to another neoplasm.
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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20130021 | Histology--Heme & Lymphoid Neoplasms: When will the follicular lymphoma, grade 1 code [9695/3] ever be used? See Discussion. | The Abstractor Notes currently do not explain the histologic classification of follicular lymphoma [FL]. Frequently, FL grade 1 and 2 are not being separated and are described as "low grade" or "grade 1-2" in the pathology final diagnosis. The correct histology code would be 9691/3 [FL, grade 2] for these cases. Apparently, per the 2008 WHO Classification, grade 1 and grade 2 are being grouped together as grade 1-2 due to the minimal difference in patient outcome. If these histologies are grouped together, will histology code 9695/3 [FL, grade 1] ever be used? Should the Heme Database explain the classifications of follicular lymphoma grade 1, 2, and 3? | When the latest WHO classification for heme neoplasms was written in 2008, there was a lot of controversy about whether or not the FL grading system was useful or not. A number of papers have been written stating that grades 1 and 2 do not have a statistically different survival or transformation rate. Given that the controversy had not been settled by those in the clinical world, the WHO recommended analyzing grades 1 and 2 together. They did not, however, remove either grade 1 or 2 from their classification. When the WHO intend to change their classification (have both grades classified under one histology number), they omit one code from their book (make it obsolete) and change the definition for the other code. The 2008 WHO book did not make either ICD-O-3 code obsolete. Therefore, we continue to collect the cases as designated by the pathologist. If the controversy is settled before the next WHO classification, you may see changes in the codes.
Additionally, since the 2008 WHO book was written, there have been some clinical papers challenging the designation of grade 3. They contend that grade 3 can be mistaken for low-grade.
The grades for follicular lymphoma are based on the number of centroblasts per high powered field (HPF). The number of centroblasts for grade 1 is 0-5; for grade 2 is 6-15, for grade 3a and 3b is >15 centroblasts. 3a has centrocytes and 3b has no centrocytes. |
2013 |
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20130173 | Histology/Primary site--Heme & Lymphoid Neoplasms: How is the primary site and histology coded when a bladder biopsy reveals myeloid sarcoma and a simultaneously performed bone marrow biopsy demonstrates acute myeloid leukemia? See Discussion. | 12/22/11 Bladder biopsy: myeloid sarcoma,
12/22/11 Bone marrow biopsy: acute myeloid leukemia.
Presenting symptoms were urological with three month history of painful hematuria and hydronephrosis with solid mass of bladder.
Prior to biopsy hem/onc states bladder mass of unknown pathology. CBC revealed peripheral blasts and Auer rods -- presumed diagnosis of acute myeloid leukemia (AML). No statement from physician as to where disease originated. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule M3, abstract a single primary when a sarcoma (myeloid sarcoma) is diagnosed either simultaneously or after a leukemia of the same lineage (acute myeloid leukemia). Per the notes for Rule M3, the sarcoma is a solid manifestation of the associate leukemia.
Per PH10, code the histology to 9861/3 [acute myeloid leukemia] and the primary site to C421 [bone marrow]. PH10 states one is to code the primary site bone marrow (C421) 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. |
2013 |
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20130162 | Reportability--Heme & Lymphoid Neoplasms: Is erythrocytosis of an unknown cause a reportable disease? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
No. Per Appendix F, erythrocytosis of an unknown cause is not reportable.
The diagnosis must state "erythrocytosis megalosplenic" to be reportable (9950/3).
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 | |
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20130020 | Reportability--Heme & Lymphoid Neoplasms: Is aplastic anemia reportable and is it an alternate name for refractory anemia? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Aplastic anemia is not reportable and it is not an alternative name for refractory anemia.
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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