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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.

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.

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

Ambiguous terms are not used to code a specific histology. This includes ambiguous terminology used as a result of immunophenotyping or genetic studies. However, a definitive clinical diagnosis can be used to code a more specific histology.

In this example, the histology is coded to non-Hodgkin lymphoma, NOS [9591/3] because the pathology final diagnosis was non-Hodgkin lymphoma, NOS even though it was followed by further genetic and immunohistochemistry studies that were "compatible with" (ambiguous terminology) follicular lymphoma.

However, if there was a subsequent non-ambiguous clinical diagnosis, the histology would be coded to the more specific diagnosis. For example, if the pathology final diagnosis was non-Hodgkin lymphoma, NOS, and there was a subsequent clinical diagnosis of follicular lymphoma or the patient was treated for follicular lymphoma, then the histology should be coded to 9690/3 [follicular lymphoma, NOS]. Document either of these in a text field to support the histology code chosen. Follicular lymphoma is a specific type of non-Hodgkin lymphoma. If you do have a confirmed diagnosis of follicular lymphoma, code that specific cell type per rule PH29.

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 Appendix F, 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.

Code the histology as superficial spreading melanoma [8743/3]. For cases diagnosed 2007-2014, the steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules manual. For a melanoma primary, use the Melanoma Histology rules to determine the histology code because there are site specific rules for cutaneous melanomas.

Start at Rule H1. The rules are intended to be reviewed in consecutive order within the applicable Module. Code the most specific histologic term when the diagnosis is melanoma, NOS [8720] with a single specific type, superficial spreading in this case. The subtype of this invasive melanoma is "superficial spreading."

A change will be made to Rule 9 in next update to indicate "growth pattern" can be used to describe an invasive histology.

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 2007 or later, code the primary site to ovary [C56.9] and the histology to papillary carcinoma, follicular variant [8340/3].

The steps used to arrive at this decision are:

Refer to the 2012 SEER Manual for help to determine the primary site. This neoplasm is arising in a teratoma of the ovary. Per the 2012 SEER Manual, in this case the site is coded to ovary [56.9] because that is where the tumor originated. Although the teratoma contains thyroid tissue, it arose in the ovary. Teratomas are unusual in that they contain all three germ cell layers from which an embryo forms. It is not unusual to have malignancies that are usually primary to the thyroid, liver, brain, lung, etc., originate in a teratoma.

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Other Sites Histology rules because site specific rules have not been developed for this primary.

Start with the SINGLE TUMOR: INVASIVE ONLY module, rule H8. The rules are intended to be reviewed in consecutive order within a module. Code the histology as papillary carcinoma, follicular variant [8340/3].

This case is reportable. Code the primary site to C25.9 [pancreas, NOS] and the histology to 8240/3 [neuroendocrine tumor (NET), Grade 1].

Per the 2012 SEER Manual, code the site in which the primary tumor originated. This neoplasm arose in pancreatic tissue and will behave accordingly, even though this pancreatic tissue is not located in the usual place.

Pancreatic endocrine and neuroendocrine neoplasms are essentially the same thing. However, they are described in two different WHO classifications; the endocrine classification and the digestive system classification. The digestive system classification is more recent, and is preferred by our expert pathologist consultant. The term "neuroendocrine" is to be used now, rather than "endocrine." In the pancreas, "well differentiated endocrine tumor" is synonymous with "neuroendocrine tumor (NET) Grade 1" and is coded 8240/3.