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

First, note that myelodysplastic syndrome (MDS) is a term that includes a number of diseases. Refractory anemia, NOS and refractory anemia with ringed sideroblasts are types of MDS. These two diseases are an NOS and a more specific disease, which is accessioned as one primary per Rule M7.

Next, assess the change from refractory anemia to AML. In checking the Heme DB, AML is listed under transformations for refractory anemia with ringed sideroblasts. This patient has a chronic disease (refractory anemia with ringed sideroblasts) and an acute disease (AML). Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic 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.

Use the Multiple Primary and Histology Coding Rules Manual for cases diagnosed 2007 or later to determine the number of primaries. This is a single primary.

For thyroid cancer only, the term micropapillary does not refer to a specific histologic type. It means that the papillary portion of the tumor is minimal or occult. The histology is the same in both lobes of the thyroid.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules manual. For a thyroid primary, use the Other Sites MP rules under one of the three formats (i.e., flowchart, matrix or text) to determine the number of primaries because the thyroid does not have site specific rules.

Start with the MULTIPLE TUMORS module, Rule M3. The rules are intended to be reviewed in consecutive order within the module from Rule M3 to Rule M18. You stop at the first rule that applies to the case you are processing.

. This patient has multiple papillary carcinomas of the thyroid diagnosed simultaneously; no other rule applies, so this is a single primary. Abstract a single primary for this patient.

Determine the histology code. For a thyroid, use one of the three formats (i.e., flowchart, matrix or text) under the Other Sites Histo rules to determine histology because thyroid primaries do not have site specific rules.

Start with the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY module, Rule H18. The rules are intended to be reviewed in consecutive order within the module from Rule H18 to Rule H31. You stop at the first rule that applies to the case you are processing.

. Code papillary carcinoma of the thyroid to papillary adenocarcinoma, NOS [8260].

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

Use Rule PH21 to code the primary site to C778 [lymph nodes of multiple regions]. The spleen is not listed under the Primary Site(s) section in the Heme DB for follicular lymphoma. Per Rule PH21 code the primary site to multiple lymph node regions, NOS (C778) when multiple lymph node regions, as defined by ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated. The spleen is a primary site for only a few lymphomas (noted in the Heme DB). Because the spleen filters blood, it is often reactive (splenomegaly) or frankly involved with the lymphoma. That reaction or involvement, however, does not affect the primary site coding. Only the involved nodes are used in coding primary site.

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.

The histology is coded as Plasma cell myeloma [9732/3]. The diagnostic confirmation is coded to 1 [positive histology].

Under the Definitive Diagnostic Methods section in the Heme DB it indicates that a bone marrow aspiration and bone marrow biopsy are procedures used to diagnose this disease process. This patient's diagnosis was based on the pathology (presumably from a bone marrow biopsy).

NOTE: This is a reportable case. Ambiguous terminology is used to accession cases (determine reportability) because it has been used for over 30 years to do so. Any deviation from using ambiguous terminology to determine case reportability would cause the reporting of incidence counts to vary. In this case, there was a reportable, ambiguous terminology diagnosis of plasma cell myeloma on the pathology report; as well as a reportable physician's statement/diagnosis of plasma cell myeloma.

Ambiguous terminology, however, is not used to report a more specific diagnosis for the Heme & Lymphoid neoplasms. For example, if the pathology report final diagnosis was "Myeloproliferative neoplasm, probably Polycythemia Vera" the histology would be coded as myeloproliferative neoplasm, unclassifiable [9975/3]. The ambiguous terminology indicates that the genetic testing, immunophenotyping, etc., probably are not complete or are not diagnostic of the more specific disease. Wait to code the histology until there is a definite diagnosis given.

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.

The expert pathologist recommends coding histology to 8980/3 [Carcinosarcoma] for this combination histology.

Expert consultation: The designation "carcinosarcoma" is given when the pathology shows differentiation in both the sarcomatous (rhabdomyoblastic) and carcinomatous (adenoca) elements. This is emphasized in the path for this case with the term "biphasic." The term "heterologous" mean that the sarcomatous component is of a type not normal to lung. Rhabdomyoblastic means skeletal muscle differentiation. Because skeletal muscle is not normally found in lung it is heterologous. If it were smooth muscle, it would be homologous because smooth muscle is found in lung (as a part of the bronchi).

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 PH1-PH29 do not apply. Per the Abstractor Notes section in the Heme DB, PTLD commonly involves lymph nodes, GI tract, lungs, and liver. Although CNS involvement is rare, in solid organ recipients the CNS may be the only site of involvement or may be associated with multi-organ involvement. Code the primary site to C719 [brain, NOS] and the histology to 9971/3 [post-transplant lymphoproliferative disorder (PTLD)]

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 2007-2014:

Code the Primary Site field to C210 [Anus, NOS]. This is an unusual and rare presentation. According to our expert pathologist, "There is no ideal site code [for] this case. I would code to C210. In this location it can at least be located by anyone who wants to get a look at such lesions. Because of the unusual location of this tumor, I would like to be able to code it to perineum, but it will be totally lost in those site codes as they represent extensive areas beyond perianal (skin of trunk, soft tissue of pelvis, and pelvis, respectively)... I would not code to rectum [because it would be] lost among too many primary rectal carcinomas."

The behavior code for hemangioblastoma can be coded to /3 when a pathologist indicates that the behavior is malignant. The behavior code should be based on a pathologist's opinion. It is usually not possible for a radiologist or patient care physician to make this determination clinically.

The histologic appearance of hemangioblastoma may resemble metastatic renal cell carcinoma; therefore, one will often see renal cell carcinoma listed as a possible diagnosis. This does not indicate that the hemangioblastoma is malignant. Do not code the behavior as /3 based on a differential diagnosis of renal cell carcinoma.

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

Code the primary site to C421 [bone marrow] and histology to 9823/3 [CLL/SLL]. Per Rule there may be involvement of bone marrow AND lymph node(s), lymph node region(s), organ(s), or tissue(s) but as long as the peripheral blood and/or bone marrow are involved, the primary site is bone marrow (C421).

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 is a single primary and the primary site and histology is coded as bone marrow [C421] and CLL/SLL [9823/3]. The code 9670/3 [malignant lymphoma, small B lymphocytes, NOS] used for SLL is now obsolete.

Per the Abstractor Notes section in the Heme DB indicates that SLL is, "usually associated with CLL and coded CLL/SLL 9823/3. Small lymphocytic lymphoma (SLL) is almost identical to CLL. A somewhat arbitrary distinction is drawn between them based on the relative degree of marrow and nodal involvement and the numbers of circulating cells."

Per the Definition section in the Heme DB it states that, "CLL by definition involves blood and bone marrow at time of diagnosis." Check the PRIMARY SITE and MODULE RULE sections that indicate the primary site is C421, Rule PH5. Per this rule, code the primary site bone marrow (C421) and code the histology B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) [9823/3] when the diagnosis is B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) AND peripheral blood is involved (the bone marrow may also be involved).

This may appear to contradict the physician's diagnosis, but the 2008 WHO no longer codes CLL and SLL as separate neoplasms, rather one neoplasm, CLL/SLL, which reflects the actual neoplastic process. Those patients with SLL usually manifest CLL during the neoplastic process and those patients with CLL usually manifest SLL during the neoplastic process. WHO recommends coding to CLL/SLL rather than coding two primaries when the other neoplasm manifests.

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.