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

Do not accession the 2011 diagnosis of Langerhans cell histiocytosis. In the Abstractor Notes section of the Heme DB is indicates this is reportable for cases diagnosed 2010 and later. However, this patient was initially diagnosed prior to 2010 when it was not a reportable disease process.

The histology code for Langerhans cell histiocytosis has not changed over time. The histology code for cases of Langerhans cell histiocytosis diagnosed prior to 2010 was also 9751 per the ICD-O-3. The only change since 2010 was in the behavior code for this disease. It changed from borderline [/1] to malignant [/3]. The current disease represents a recurrence of the previous Langerhans cell histiocytosis. Per the Multiple Primary rules, Rule M2, a single histology is a single primary. The original diagnosis was made before the disease was reportable; do not report the disease recurrence or progression as a new 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.

This case should be accessioned as two primaries per Rule M11. Code the histology of one primary to 9680/3 [diffuse large B-cell lymphoma], the acute neoplasm. Code the histology for the second primary to 9823/3 [chronic lymphocytic leukemia/small lymphocytic lymphoma], the chronic neoplasm.

Per Rule M11, abstract as multiple primaries when both a chronic and acute neoplasm are diagnosed simultaneously or less than or equal to 21 days apart AND there is documentation of two pathology specimens, one confirming the chronic neoplasm (bone marrow biopsy) and one confirming the acute neoplasm (stomach 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 2007 or later, code the histology as lobular carcinoma, in situ [8520/2].

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). Go to the Breast Histo rules because site specific rules exist for this primary.

Start at the SINGLE TUMOR: IN SITU CARCINOMA ONLY module, Rule H1. The rules are intended to be reviewed in consecutive order. Stop at the first rule that applies to the case you are processing. Code the histology to lobular carcinoma in situ [8520/2] because this is the only histologic type identified.

Pleomorphic lobular carcinoma is a variant of lobular carcinoma which does not have an ICD-O-3 code. It is still a lobular carcinoma. The identification of the variants of lobular carcinoma was a relatively recent discovery and the information was not available when the 2007 MP/H Rules were written. All of the lobular variants will be included in the next revision of the MP/H Rules.

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."

Hyalinizing trabecular neoplasm is not reportable.

Hyalinizing trabecular neoplasm, or hyalinizing trabecular tumor, is a synonym for hyalinizing trabecular adenoma [8336/0] in the ICD-O-3. The 2004 WHO classification states that "fine needle aspiration biopsy is often interpreted as papillary carcinoma because of the nuclear features in the tumor."

Accession two primaries per Rule M17, neuroendocrine carcinoma [8246/3] of the rectosigmoid junction [C199], and adenocarcinoma in a villous adenoma [8261/3] of the rectosigmoid junction [C199]. There are two tumors with ICD-O-3 histology codes that differ at the third number.

Rule M13 does not apply to neuroendocrine carcinoma. Rule M16 does not apply to this case because there are two specific histologies.

Based on the information provided, this case of adult granulosa cell tumor is malignant and reportable. According to our expert pathologist consultant, "though granulosa cell tumor NOS/ adult NOS is 8620/1, the presence of peritoneal implants or metastases, and/or lymph node metastases indicates the tumor is malignant, and it should be coded /3."

Note that the presence of implants or metastases does not indicate malignancy in the case of low malignant potential ovarian epithelial tumors. Our path expert explains "in contrast, by convention the behavior of borderline/LMP ovarian epithelial tumors is determined by the ovarian primary, and is /1, even though there may be peritoneal implants/metastases, or metastatic disease in lymph nodes. The treatment may vary in these circumstances, but to my knowledge the decision as to the tumor designation remains based on the primary tumor."

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

Chronic myeloproliferative neoplasm is reportable. The preferred term is myelodysplastic/myeloproliferative neoplasm, unclassifiable (MPN). Chronic myeloproliferative neoplasm is listed in the Heme DB under the Alternate Names section for this neoplasm.

The term chronic does not affect the reportability of this neoplasm. The newer terms are myeloproliferative neoplasm or myeloproliferative disorder and chronic is not used in most diagnoses.

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.

Without further information as to the type of acute myeloid leukemia, code the histology to 9861/3 [acute myeloid leukemia, NOS]. If further information on the specific acute myeloid leukemia becomes available, update the histology code.

Document that the pathology report states the acute myeloid leukemia is a "non-M3 type" in a text field. This documentation will help explain the choice of 9861/3 for this case. M3 refers to one of the eight FAB subtypes described by a group of French, American, and British leukemia experts in the 1970's who divided acute myeloid leukemias into subtypes, M0 through M7. They classified the disease based on the type of cell from which the leukemia developed and how mature the cells were. This was based largely on how the leukemia cells looked under the microscope after routine staining.

In this case, all we know is that the histology does not pathologically represent the M3 (acute promyelocytic leukemia (APL)) form of acute myeloid leukemia. We do not know which type of acute myeloid leukemia it does represent.

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.