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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.

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

Per PH Rule22, code the primary site to C779 [lymph nodes, NOS].

Rule PH22 is a default rule for lymphomas that is used when there is no other information regarding the primary site and the Heme DB does not indicate a primary site under its Primary Site(s) section. Rule PH27, code the primary site to unknown [C809], does not apply. Only use C809 [unknown] as the primary site when there is no evidence of lymphoma in lymph nodes AND the physician documents that the lymphoma originates in an organ(s).

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.

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).

Urine cytology positive for malignancy is reportable. Code the primary site to C689 in the absence of any other information.

However, if a subsequent biopsy of a urinary site is negative, do not report the case.

For 2013 diagnoses and forward, report these cases when they are encountered. Do not implement new/additional casefinding methods to capture these cases.

As always, do not report cytology cases with ambiguous terminology.

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 per Rule M15. The histology is coded to 9698/3 [follicular lymphoma, grade 3] diagnosed in 2006. The 2011 diagnosis of follicular lymphoma, grade 2 [9691/3] is not a new primary.

Follicular lymphoma, grade 2 [9691/3] is listed under the Same Primaries section of the Heme DB for 9698/3 [follicular lymphoma, grade 3]. To confirm this, Rule M15 indicates we are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries because none of the rules from 1-14 apply. Per the calculator, these histologies represent the same 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 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.

Accession a single primary, the original ovarian serous carcinoma. The MP/H Rules do not apply to metastases.

Metastases: When cancer cells appear in other nodes or organs that are not the primary site they are metastatic cells. Discontinuous (separate from the primary tumor) masses or cells in regional lymph nodes, distant lymph nodes, or distant sites are always metastases. In this case, the sacral and pelvic masses are distant metastases. The pathologist does not have to compare cells to the original tumor slides; the discontinuous tumor mass/cells in any site other than the primary site are metastases.

Recurrence: For a disease to recur there are several criteria that must be met. First and most important, the patient must have had a disease-free interval (a tumor cannot recur if it has always been present). The other criteria are: the "new tumor" has to occur in the original primary site, it must be the same histology as the original tumor, AND must meet the timing requirements in the MPH rules for that organ/site.

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

Code the histology to 9695/3 [follicular lymphoma, grade 1] per PH29.

Under the Alternate Names section of the Heme DB, B-cell non-Hodgkin lymphoma is synonym for non-Hodgkin lymphoma, NOS and B-cell lymphoma, NOS.

Per PH29, one codes the histology when there is one non-specific histology (NHL, NOS) and one specific histology (FL, grade 1). You are also required to confirm the specific and the non-specific (NOS) histology represent the same primary using the Multiple Primaries Calculator. The calculator indicates these are the same 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].

Pancreatic (neuro)endocrine neoplasms (PanNETs) are reportable. The correct histology code is 8240/3. The grade is coded as 1 [well differentiated].