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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.

Per Rule PH30, use the Heme DB to determine the primary site and histology when rules PH1-PH29 do not apply. The Heme DB indicates the primary site for systemic mastocytosis is always coded to C421 [bone marrow].

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.

This case should be accessioned as two primaries per Rule M13. Code the histology for the 7/31/08 diagnosis to 9680/3 [diffuse large B-cell lymphoma] and the code the histology for the 10/14/2010 diagnosis to 9698/3 [follicular lymphoma, grade 3A of 3]. Rule M13 applies to this case because the neoplasm was originally diagnosed in the blast or acute phase (DLBCL) and reverted to a less aggressive or chronic phase (follicular lymphoma) after treatment.

Per the "Transformations to" section in the Heme DB for follicular lymphoma, grade 3 transforms to diffuse large B-cell lymphoma [9680/3]. This means that the follicular lymphoma is the chronic neoplasm and that DLBCL is the acute neoplasm. In this case, the chronic neoplasm was diagnosed after the acute neoplasm was diagnosed and treated (with chemotherapy).

Do not Stop at Rule M4 because diffuse large B-cell lymphoma and follicular lymphoma (both NHL's) were not present in the same node(s) AT THE SAME TIME.

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.

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.

Code the histology to duct and lobular carcinoma in situ [8522/2].

For cases diagnosed 2007 and later, the steps used to arrive at this decision are:

Go to the Breast MP rules found in the Multiple Primary and Histology Coding Rules Manual. Start at the MULTIPLE TUMORS Module Rule M4 because the patient had multiple foci of DCIS and a separate, single focus of LCIS. The rules are intended to be reviewed in consecutive order within the applicable Module. Tumors that are lobular and duct are a single primary.

Go to the Breast Histology rules found in the Multiple Primary and Histology Coding Rules Manual. Start at the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY Module Rule H20 because the patient has multiple foci of DCIS and LCIS. Code the histology as 8522/2 [duct and lobular carcinoma in situ] when there is any combination of lobular [8520] and duct carcinoma.

The DCIS and LCIS are separate tumors. The DCIS was removed by the lumpectomy and the LCIS by the mastectomy. The most representative specimen for the DCIS is the lumpectomy. The most representative specimen for the LCIS is the mastectomy. Both pathology reports must be used in this case to determine the histology.

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 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.

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.

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is not reportable. Per Appendix F, HLH is caused by an over stimulated immune system (infection, etc.). It is a clinical syndrome associated with a variety of underlying conditions. To be reportable, a child's diagnosis must state "fulminant hemophagocytic syndrome" to be reportable (9724/3). This is not the situation in this case.

"Hemophagocytic lymphohistiocytosis" is also listed in Appendix F: Non-Reportable List for Hematopoietic Diseases.

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.