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Report Produced: 04/21/2026 18:44 PM

Report Question ID Question Discussion Answer Year
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
20110088

Chemotherapy/Neoadjuvant treatment: Should neoadjuvant chemotherapy be coded for an incidental second primary discovered at the time of surgery? If so, how is the diagnosis date coded? See Discussion.

The patient had neoadjuvant chemotherapy for rectal carcinoma. An AP resection revealed an incidental second primary intramucosal carcinoma in adenomatous polyp in the descending colon. Is the chemotherapy coded as therapy for the intramucosal carcinoma of the descending colon?

Record the neoadjuvant therapy only for the first primary and do not record the neoadjuvant therapy for the incidental new primary found on surgery. 

 2011
20110005 Histology--Heme & Lymphoid Neoplasms: How is the pre-2010 histology coded for a "follicular grade 2, non-Hodgkin lymphoma with marginal zone B-cell differentiation"? See Discussion. This patient was seen in 2010 for the same primary as diagnosed in 2006. The histology was coded to marginal zone lymphoma [9699/3] in 2006. Is this correct? Or should this have been coded as a follicular lymphoma, ignoring the modifying expression "marginal zone B-cell differentiation"?

This is a 2006 diagnosis. The histology code is 9691/3 [follicular lymphoma, grade 2]. Do not code differentiation for hematopoietic cases.

For diagnoses 2010 and forward, a small number of cases of follicular lymphoma do have marginal zone differentiation. However, there is no code for this variant of follicular lymphoma. It would simply be coded as a follicular lymphoma because that is the most accurate histology code available. The marginal zone differentiation is not to be coded as a second primary (marginal zone lymphoma).

2011
20110150 Ambiguous Terminology--Heme & Lymphoid Neoplasms: As ambiguous terminology is not used to code histology for Heme & Lymphoid primaries, how is the histology coded when a patient has a clinical diagnosis of "consistent with a myelodysplastic syndrome"? See Discussion.

The physician states the "patient's clinical picture certainly is most consistent with MDS." Several FISH probes were performed on peripheral blood, specifically looking for the 5q minus syndrome as well as other molecular rearrangements to suggest or confirm MDS. These studies came back as normal. The initial bone marrow also came back negative. The physician then states, "The suspicion was that this represented a myelodysplastic syndrome despite the normal cytogenetics. Additional studies performed on the date of the clinic visit included the FISH for the 5q minus syndrome as well as CD59 to exclude PNH. Both of these were negative. Therefore, at this juncture, the patient has a macrocytic anemia not yet requiring transfusion support with a normal white count and an elevated platelet count and a hypercellular bone marrow. This is certainly consistent with a myelodysplastic syndrome."

Per coding guidelines, ambiguous terminology is not used to code histology, only for reportability. What is the histology code for this case?

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

Code the histology as Myelodysplastic syndrome, unclassifiable [9989/3].

Ambiguous terminology is used to accession cases (determine reportability). While ambiguous terminology is generally not used to code a specific histology, it can be used to code histology if it is the .

The statement that you do not use ambiguous terms to code histology is intended for those NOS histologies with an ambiguous term being used to describe the subtype. For example, if the physician states this is a myelodysplastic syndrome, NOS, refractory thrombocytopenia. The correct histology would be MDS, NOS [9989/3] and not refractory thrombocytopenia [9992/3].

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.

2011
20110053 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient with a several month history of refractory anemia with excess blasts (RAEB), that may or may not have been treated, who now presents with a bone marrow biopsy that is compatible with acute myeloid leukemia?

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

Per Rule M10, abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm AND there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. Two primaries should be accessioned for this case: refractory anemia with excess blasts (RAEB) [9983/3] (a chronic neoplasm), and acute myeloid leukemia [9861/3] (an acute 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.

2011
20110008 MP/H Rules/Histology--Vulva: How is histology coded for VIN III with focal invasion? See Discussion. Per SINQ 20000442, the histology for CIN III with microinvasion is coded to 8077 [squamous intraepithelial neoplasia, grade III] per the matrix system rules, with a behavior code of /3 [malignant]. Coding the histology to 8077/3 per the matrix principle causes IF25_3 and MorphICDO3_P1 edits to fail. Flagging the first error resolves any reporting issue. How is the MorphICDO3_P1 edit resolved? Assign 8076/3 [squamous cell carcinoma, microinvasive] for VIN III with focal invasion. This applies to all terminologies listed under 8077/2. The SINQ question from 2000 will be retired. 2011
20110079 MP/H Rules/Histology: In the MP/H Manual, where is the documentation indicating "focal" is not a term that can be used to code histology? See Discussion. Example: neuroendocrine carcinoma with focal squamous differentiation. For the purposes of the MP/H rules, the term "focal" is not used to indicate a more specific histology. Terms that may be used to indicate a more specific histology are listed in the relevant histology rules. For example, see Breast histology rule H3. Notice the terms listed in the note for this rule are "type, subtype, predominantly, with features of, major, with ___ differentiation, architecture or pattern." The term "focal" is not included. This concept will be clarified in future revisions to MP/H rules. 2011
20110080 Grade--Kidney, renal pelvis: How is this field coded for a non-invasive high grade papillary urothelial carcinoma of the renal pelvis? See Discussion. Per instructions in the 2010 SEER Manual, Appendix C, Coding Guidelines for Bladder, "Code grade 9 (unknown) for non-invasive urothelial (transitional) tumors." The Coding Guidelines listed under Renal Pelvis, Ureter are only for Kidney [C649]. Do the grade instructions under bladder apply to ALL non-invasive urothelial tumors, or are we to use the kidney grading instructions to code grade for renal pelvis and ureter malignancies? Code grade to 4 [high grade]. Follow the instructions in the main part of the 2010 SEER Manual under the data item Grade (pages 73 - 76). There are no specific instructions for coding grade for renal pelvis. Apply the general instructions in the absence of site-specific instructions. 2011
20110031 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if patient initially diagnosed with granulocytic sarcoma on a vocal cord biopsy is subsequently diagnosed with acute myeloid leukemia more than 21 days later? See Discussion.

The patient has a history of refractory anemia with excess blasts diagnosed in 2008. A vocal cord biopsy performed on 6/2/2010 stated, "in view of a previous history of myelodysplastic syndrome this is indicative of transformation to acute leukemia" and consistent with granulocytic sarcoma. A bone marrow biopsy done on 7/19/2010 stated this was compatible with refractory anemia with excess blasts in transformation.

Granulocytic sarcoma is a solid manifestation of AML. When these diagnoses occur more than 21 days apart, are they separate primaries?

According to the WHO definition, this is acute myeloid leukemia complicating myelodysplasia. Which rule applies for this case?

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

This case should be accessioned as two primaries. The first is refractory anemia with excess blasts in 2008, and the second is AML June 2, 2010.

As for the disease occurring in 2010, granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously.

In this case, when the physician gave a provisional diagnosis of "transformation to acute leukemia" it indicated he saw the solid deposits of myeloid cells on the vocal cord. Per Rule M3, AML and myeloid (granulocytic) sarcoma appearing simultaneously are a single primary coded to AML. When the patient has AML, solid myeloid deposits (myeloid sarcoma) may appear. This is a manifestation of the AML rather than a new primary. Rule PH10 states to code the histology to AML.

Under the Transformation section in the Heme DB for refractory anemia with excess blasts (a chronic neoplasm), it indicates this disease process does transform to acute myeloid leukemia, NOS (an acute neoplasm). In this case, the chronic and acute disease processes were diagnosed at different times. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic diagnosis.

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
20110147 Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How is the histology coded when no bone marrow examination is performed but the peripheral blood flow cytometry listed several differential diagnoses and the physician states the diagnosis is small lymphocytic lymphoma? See Discussion.

The peripheral blood flow cytometry results state, "findings consistent with a small mature B-cell neoplasm, differential - marginal zone lymphoma, lymphoplasmacytic lymphoma, and atypical CLL." The physician states the diagnosis is "SLL." No bone marrow examination or CT scan was done to assess whether the patient had lymphadenopathy.

Per Rule PH5, if the diagnosis is B-cell CLL/SLL and peripheral blood is involved, the histology is coded to B-CLL/SLL [9823/3]. Should the primary site and histology be coded to bone marrow [C421] and CLL/SLL [9823/3] per Rule PH5 despite the physician's diagnosis of SLL [9670/3]?

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

This is a single primary and the primary site and histology is coded as bone marrow [C421] and CLL/SLL [9823/3]. The code 9670/3 [malignant lymphoma, small B lymphocytes, NOS] used for SLL is now obsolete.

Per the Abstractor Notes section in the Heme DB indicates that SLL is, "usually associated with CLL and coded CLL/SLL 9823/3. Small lymphocytic lymphoma (SLL) is almost identical to CLL. A somewhat arbitrary distinction is drawn between them based on the relative degree of marrow and nodal involvement and the numbers of circulating cells."

Per the Definition section in the Heme DB it states that, "CLL by definition involves blood and bone marrow at time of diagnosis." Check the PRIMARY SITE and MODULE RULE sections that indicate the primary site is C421, Rule PH5. Per this rule, code the primary site bone marrow (C421) and code the histology B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) [9823/3] when the diagnosis is B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) AND peripheral blood is involved (the bone marrow may also be involved).

This may appear to contradict the physician's diagnosis, but the 2008 WHO no longer codes CLL and SLL as separate neoplasms, rather one neoplasm, CLL/SLL, which reflects the actual neoplastic process. Those patients with SLL usually manifest CLL during the neoplastic process and those patients with CLL usually manifest SLL during the neoplastic process. WHO recommends coding to CLL/SLL rather than coding two primaries when the other neoplasm manifests.

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