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This is a single primary, essential thrombocythemia [9962/3] diagnosed in 2007. The 2010 Heme DB and Manual should not have been used to determine the number of primaries in this case. The Heme DB applies only to cases diagnosed 2010 and later.

In order to determine the number of primaries, use the rules in place at the time of the subsequent 2009 diagnosis of primary myelofibrosis. Per the Single Versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table, a diagnosis of essential thrombocythemia [9962/3] followed by a diagnosis of primary myelofibrosis [9961/3] is a single primary.

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

Accession a single primary per Rule M2 which indicates you are to abstract a single primary when there is a single histology. Code the histology to 9983/3 [refractory anemia with excess blasts type 2 (RAEB-2)].

Per Appendix F, myelofibrosis, NOS, is NOT a synonym for primary myelofibrosis. Myelofibrosis, NOS, if not specified to be myelofibrosis, therefore, is not reportable.

Per PH29, code the specific histology when the diagnosis is one non-specific (NOS) histology (MDS) and one specific histology (RAEB-2) AND the Multiple Primary Calculator confirms the specific histology and NOS histology are the same primary (which it does).

Myelodysplastic syndrome, NOS is a generic disease description. In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. The more specific myelodysplastic syndromes are: refractory anemia; refractory neutropenia; refractory thrombocytopenia; refractory anemia with ring sideroblasts; refractory cytopenia with multilineage dysplasia; refractory anemia with excess blasts; and refractory cytopenia of childhood. If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific 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.

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

Code the primary site to C119 [nasopharynx] per Rule PH25.

Per our subject matter expert, use Module 7 Rule PH25 to code the primary site to an organ (nasopharynx and maxillary sinus) because an organ(s) and its regional lymph nodes are involved. The distant lymph nodes are simply part of the staging (the lymphoma has progressed to another lymph node region).

Diffuse large B cell lymphoma originating in the oral cavity and maxillofacial region is rare, but documented. The most common sites for this rare neoplasm are Waldeyer ring, tonsils, nasopharynx, base of tongue, and palatine tonsil. There are also rare cases of diffuse large B cell lymphoma originating in the maxillary sinus. The percentage of cases arising in the nasopharynx is greater than those originating in the maxillary sinus.

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.

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 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.

For cases diagnosed 2007 or later, accession two primaries, serous carcinoma of the right ovary and serous tubal intraepithelial carcinoma of the left fallopian tube based on the information provided.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text) and go to the Other Sites MP rules because neither the ovary nor fallopian tube have site specific rules developed.

Start at the MULTIPLE TUMORS module, Rule M3. The rules are intended to be reviewed in consecutive order within a module. The patient has multiple tumors with ICD-O-3 topography codes that are different at the third character (Cxx) and therefore this case should be accessioned as a multiple primary.

It could be helpful to know the extent of involvement noted prior to neoadjuvant therapy and debulking surgery. For example, if the patient had widely metastatic disease throughout the entire pelvis prior to the initiation of treatment, the answer may have been different.

The following answers depend on what this pathologist means by "ductal (tubular)." According to the WHO classification, tubular is not a duct subtype. Check with the pathologist if possible to determine if the intended meaning is "tubular carcinoma" or "duct carcinoma".

If the pathologist uses the expression "ductal (tubular)" as an equivalent of "tubular carcinoma": Accession two primaries, a tubular carcinoma [8211/3] and a ductal carcinoma in situ, solid type [8230/2].

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

Determine the provisional histologies of these tumors in order to apply the Multiple Primary rules. Open the Multiple Primary and Histology Coding Rules manual. For a breast primary, use the Breast Histology rules to determine the histology codes because there are site specific rules for breast primaries.

Determine the histology of in situ carcinoma, solid type ductal carcinoma in situ. Start at Rule H1. The rules are intended to be reviewed in consecutive order within the applicable Module. Code the more specific histologic term when the diagnosis is intraductal carcinoma and a type of intraductal carcinoma. Solid is a specific type of DCIS. The histology is 8230/2.

Determine the histology of the invasive carcinoma, tubular carcinoma. Start at Rule H10. Code the histology when only one histologic type is identified, Tubular carcinoma was the only type identified. The histology is 8211/3.

Go to the Breast MP rules found in the Multiple Primary and Histology Coding Rules Manual after determining the histology of each tumor. Start at the MULTIPLE TUMORS Module, Rule M4, because the patient has a single invasive tumor and separate foci of DCIS. These tumors have ICD-O-3 histology codes that are different at the third (xxx) number and are, therefore, multiple primaries.

If the pathologist uses the expression "ductal (tubular)" as an equivalent of "duct carcinoma": Accession a single primary, a duct carcinoma [8500/3].

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 has a single invasive duct carcinoma and separate foci of solid type ductal carcinoma in situ. Multiple intraductal and/or duct carcinomas are a single primary. Table 1 identifies solid type as a specific type of intraductal carcinoma.

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. Code the invasive histology when both invasive and in situ tumors are present. Code the histology as 8500/3 [duct carcinoma].

Mature teratomas in the ovary are benign [9080/0].

For testis, mature teratoma in an adult is malignant (9080/3); however, mature teratoma in a child is benign (9080/0).

With regard to the thyroid issue, from the information above, the medullary carcinoma in the patient's thyroid is clearly a separate event. According to our expert pathologist consultant, "thyroid tissue is one of the many tissue types that may be seen in teratomas. When the teratoma has exclusively or predominantly thyroid tissue the term struma ovarii is used Adenoma or carcinoma of the thyroid type may be seen in this thyroid tissue. If medullary carcinoma were present in the thyroid tissue in the ovary/teratoma, there would be mention of it in the path report."