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

Code the primary site to C77.8 [multiple lymph node regions, NOS].

Per Rule PH6, code the primary site to the involved lymph node region(s) when there is no bone marrow involvement or when it is unknown whether the bone marrow is involved. To determine the more specific lymph node subsite to code, use Rule PH21. It indicates one is to code the primary site to C778 [multiple lymph node regions, NOS] when multiple lymph node regions, as defined by the ICD-O-3 (see Table C1: Lymph Node/Lymph Node Chain Reference Table in Appendix C), are involved and it is not possible to identify the lymph node region where the lymphoma originated.

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.

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.

Because the site is specifically stated to "skin" of nipple [C44.5], this case is not reportable.

If possible, you may wish to confirm the type of biopsy performed. If the biopsy was done by FNA or needle biopsy, the biopsy tissue should contain a full-thickness of skin and subcutaneous breast (nipple) tissue. If that is the case, this tumor would likely be a reportable squamous cell carcinoma of nipple [C50.0]. If, however, this was a punch biopsy it is more likely a non-reportable squamous cell carcinoma of the skin [C44.5].

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.

For the purpose of applying the MP/H rules, the term "synchronous" means that the two diagnoses occurred at the same time or less than or equal to 60 days apart.

The case in SINQ 20100025 was not synchronous. The first lesion in the renal pelvis [C65.9] occurred in 1/08 and the subsequent tumors were diagnosed in 5/09, more than one year apart. In this case, you do not go back to change the primary site code on the original abstract.

The case in SINQ 20110119 was diagnosed synchronously, the first lesion in the bladder [C67.9] was diagnosed in 11/09 and the second lesion in the renal pelvis [C65.9] was diagnosed in 12/09, less than 60 days apart. Because the lesions were synchronous, the primary site is coded urinary system, NOS [C68.9].

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

Myelodysplastic disorder is a synonym for myelodysplastic syndrome (MDS). If no further workup is done or no additional information can be found, code the histology of myelodysplastic disorder to 9989/3 [MDS] for cases diagnosed 1/1/2010 and later.

Refer to the Abstractor Notes section in the Heme DB, Abstractor Notes for MDS. Myelodysplastic (disorder) syndrome is a NOS term. Usually when this diagnosis is made, the physician will conduct further tests to determine a more specific disease in the Myeloproliferative Neoplasms group. Other specific histologies include: refractory anemia with unilineage dysplasia, refractory anemia with ring sideroblasts, refractory cytopenia with multilineage dysplasia, refractory anemia with excess blasts, myelodysplastic syndrome with del(5q), childhood myelodysplastic syndrome. If a more specific disease is diagnosed, code to that specific 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.

For cases diagnosed 2021 or later, VIN II-III is reportable. Similarly, AIN II-III, VAIN II-III, etc. are reportable. For cases diagnosed 2021 or later, the primary resource for reportability is ICD-O-3.2. Squamous intraepithelial neoplasia, grade II is listed in ICD-O-3.2 as 8077/2 making it reportable. This applies to the various sites of intraepithelial neoplasia grade II including anus, vulva, and vagina.

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.

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.

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

Do not accession the 2011 diagnosis of Langerhans cell histiocytosis. In the Abstractor Notes section of the Heme DB is indicates this is reportable for cases diagnosed 2010 and later. However, this patient was initially diagnosed prior to 2010 when it was not a reportable disease process.

The histology code for Langerhans cell histiocytosis has not changed over time. The histology code for cases of Langerhans cell histiocytosis diagnosed prior to 2010 was also 9751 per the ICD-O-3. The only change since 2010 was in the behavior code for this disease. It changed from borderline [/1] to malignant [/3]. The current disease represents a recurrence of the previous Langerhans cell histiocytosis. Per the Multiple Primary rules, Rule M2, a single histology is a single primary. The original diagnosis was made before the disease was reportable; do not report the disease recurrence or progression as a new 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.