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20120050 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what histology codes apply if a patient has a 1998 diagnosis of essential thrombocythemia and a recent clinical diagnosis of secondary myelofibrosis? See Discussion. | The patient has a history of essential thrombocythemia (ET) since 1998. This has been treated daily with aspirin. A recent bone marrow biopsy was consistent with myeloproliferative disorder with excess blasts, marked extensive reticulin marrow fibrosis with osteosclerosis, excess blasts (11%) in the marrow aspirate and peripheral blood. JAK2 mutation was present in a small minority of cells. The physician stated patient was, "considered to have secondary myelofibrosis and was started on Jakafi." | 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. |
2012 |
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20120073 | Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a 2011 diagnosis of systemic mastocytosis? See Discusson. | Patient presented with leukopenia, anemia and monoclonal gammopathy. A bone marrow biopsy in 2011 showed systemic mastocytosis [9741/3]. A subsequent shave biopsy of abdominal skin showed histologic features that were consistent with a diagnosis of mastocytosis. A later bone marrow biopsy was subsequently performed that showed progressive systemic mastocytosis. | 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. |
2012 |
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20120004 | Grade--Heme & Lymphoid Neoplasms: How is grade coded for a malignant non-Hodgkin lymphoma, large B-cell type, with features consistent with T-cell rich variant? | 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. |
2012 | |
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20120068 | Histology--Heme & Lymphoid Neoplasms: What is the correct histology code for a diagnosis of mature B cell leukemia/lymphoma diagnosed only on a peripheral blood smear? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the histology to 9591/3 [B-cell lymphoma, NOS].
After searching the Heme DB for the term , no B-cell leukemia/lymphoma NOS code is found. However, the diagnosis of B-cell lymphoblastic leukemia/lymphoma is found. This case scenario does not specify that this is a lymphoblastic leukemia/lymphoma; therefore, the histology code 9811/3 [B-cell lymphoblastic leukemia/lymphoma, NOS] cannot be applied.
A subsequent search of the Heme DB for the term returns "Non-Hodgkin lymphoma, NOS" [9591/3]. Under the Alternative Names section of the Heme DB, B-cell lymphoma, NOS, is a synonym for Non-Hodgkin lymphoma, NOS. Therefore, the B-cell lymphoma NOS code [9591/3] is the most appropriate histology code to use for this case.
This will be added to the next revision of the Heme DB and Manual.
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. |
2012 | |
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20120060 | Primary Site/Reportability: What is the primary site and reportability status of a "pancreatic endocrine neoplasm" that arises in the heterotopic pancreas of the splenic hilum that is stated to be a "well-differentiated endocrine tumor, uncertain behavior per the WHO classification"? See Discussion. | SINQ 20120035 states that well differentiated pancreatic endocrine neoplasms should be reported with histology code 8240/3. However, the pathology report provides the WHO Classification which states "uncertain behavior." Should this tumor still be reported as 8240/3?
If reportable, how is the primary site coded? The tumor arose in heterotopic pancreas (in the splenic hilum), which is pancreatic tissue found outside the usual anatomical location of the pancreas. Per the pathology report, the tumor did not invade the spleen. Should the primary site be coded to C48.1 [mesentery]? The patient is female and the coding schema for "Peritoneum for Females" would apply to the case. However, none of those CS extension codes seem to apply to this localized case.
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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. |
2012 |
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20120065 | MP/H Rules/Primary site: What is the primary site and histology for a focus of papillary thyroid cancinoma, follicular variant, arising in thyroid tissue of mature cystic teratoma of the ovary? | 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]. |
2012 | |
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20120033 | Multiple Primaries--Hematopoietic: How many primaries are abstracted when a patient is diagnosed with essential thrombocythemia in 2007 and a bone marrow biopsy performed on 12/4/2009 shows primary myelofibrosis? See Discussion. |
The patient was diagnosed with essential thrombocythemia in 2007 and was treated with Hydrea. The 2009 bone marrow biopsy showed primary myelofibrosis which the physician states is a transition from the essential thrombocythemia. The Heme DB calls this two primaries. |
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. |
2012 |
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20120081 | Reportability: Is VIN II-III reportable? |
For cases diagnosed 2021 and later VIN II-III is reportable based on ICD-O-3.2 which lists squamous intraepithelial neoplasia, grade II as 8077/2 making it reportable. Also see SINQ 20120094. |
2012 | |
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20120018 | MP/H Rules/Histology--Breast: How is histology coded if a lumpectomy reveals multifocal ductal carcinoma in situ spanning an area of 0.9-1.2 cm with close margins and a subsequent mastectomy reveals only a single focus of lobular carcinoma in situ measuring 0.2 cm in the UOQ, remote from all surgical margins? See Discussion. | Does the general instruction apply in this case that indicates the histology is coded from the most representative tumor specimen resulting in the histology coded to 8500/2 [DCIS]? Or is the histology coded to 8522/2 [duct and lobular carcinoma in situ] per Rule H28 because there is any combination of lobular [8520] and duct carcinoma [8500]? | 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. |
2012 |
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20120008 | MP/H Rules/Recurrence--Ovary: How many primaries are accessioned if a patient was diagnosed with ovarian serous carcinoma four years ago and currently has sacral and pelvic masses positive for serous carcinoma on biopsy? Should this be disease progression or a new primary? See Discussion. |
Should this be a new primary per the MP/H Rules (Other Sites, Rule M10) because the diagnoses were made more than one year apart? Or is the new disease metastasis? The pathologist did not compare the subsequent mass biopsies with the original pathology. Is a pathologist's comparison of slides the only criteria for determining recurrent disease? This case seems to fit the definition of metastatic disease rather than a recurrence, and therefore would not be a new primary. |
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. |
2012 |
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