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

Code the histology as Myelodysplastic syndrome, unclassifiable [9989/3].

Ambiguous terminology is used to accession cases (determine reportability). While ambiguous terminology is generally not used to code a specific histology, it can be used to code histology if it is the .

The statement that you do not use ambiguous terms to code histology is intended for those NOS histologies with an ambiguous term being used to describe the subtype. For example, if the physician states this is a myelodysplastic syndrome, NOS, refractory thrombocytopenia. The correct histology would be MDS, NOS [9989/3] and not refractory thrombocytopenia [9992/3].

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.

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: follicular lymphoma [9690/3] and diffuse large B-cell lymphoma [9680/3] per Rule M10. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (follicular lymphoma) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) more than 21 days after the chronic diagnosis.

Record the CHOP as the first course of treatment for the follicular lymphoma because this was the only treatment given for the chronic neoplasm (follicular lymphoma) prior to the transformation to the acute neoplasm (DLBCL). Record the chemotherapy and bone marrow transplant as first course treatment for the DLBCL.

As noted above, follicular lymphoma does transform to DLBCL. This "transformation" is actually a new disease. Follicular lymphoma is a disease in which the lymph nodes have a prominent follicular pattern; DLBCL is a disease with diffuse proliferation of large lymphoid cells. While it is true that follicular lymphoma will transform to DLBCL, this transformation indicates it becomes a different entity.

The DLBCL is coded as a second primary for several reasons: to determine the incidence of follicular lymphomas transforming to DLBCL; survival time can be calculated for the diagnosis of the more aggressive DLBCL; death will be attributed to the DLBCL (for mortality statistics) and not the follicular lymphoma

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.

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

This case should be accessioned as a single primary: malignant lymphoma, mixed cell type, follicular [9691/3] diagnosed in 2003. The following describes how this determination was made.

This case is one in which the terminology for follicular lymphoma has changed over time. In 2003, follicular lymphoma was classified as small cleaved cell, large cell, or mixed cell (both small cleaved and large cell). Those designations are no longer used. This disease process is currently classified as follicular lymphoma NOS, grade 1, grade 2 or grade 3. The change was simply a change in classification/terminology.

Appendix A, Table A3 (Obsolete Terms as Defined in ICD-O-3, Lymphoid Neoplasm Obsolete Terms) should be used to determine the current term when an obsolete term is known/given. Per the Table, "Mixed cell type follicular lymphoma" is currently known as "Follicular lymphoma, grade 2" and the correct histology code is 9691/3. This is the correct histology for the 2003 primary.

Per Rule M15, the histologies must be check in the Multiple Primaries Calculator to determine the number of primaries. Enter [follicular lymphoma, grade 2 (malignant lymphoma, mixed cell type, follicular)] for Histology Code 1 and [follicular lymphoma, NOS] for Histology Code 2. The result is "Same Primary." As a result, accession a single 2003 diagnosed primary with the histology follicular lymphoma, grade 2 [9691/3] when the patient is subsequently diagnosed with follicular lymphoma, NOS.

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.

Abstract as a single primary: dermatofibrosarcoma protuberans [8832/3] of the left upper inner arm [C446] diagnosed in August 2008.

The rationale for this answer was provided by subject matter experts. The physician specialists for soft tissue and bone replied as follows:

Low-grade sarcomas tend to recur locally. Because this tumor recurred in same area, i.e. scar of prior surgery, and recurred in this period of time, this is a local recurrence. Dermatofibrosarcoma Protuberans is a low grade tumor which can recur many years following tumor excision.

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

Idiopathic thrombocytic purpura (ITP) is not a synonym for refractory thrombocytopenia (RT). ITP is not a reportable disease. See Appendix F.

Under the Alternate Names section in the Heme DB, the only synonym for refractory thrombocytopenia is "RT." ITP is not listed as a synonym for refractory thrombocytopenia.

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.

When there is a question of whether the SEER MP/H Rules or Hematopoietic and Lymphoid Neoplasm Rules apply, check the histology and refer to the Case Reportability Instructions in the Hematopoietic and Lymphoid Neoplasm Manual. All ICD-O-3 morphology codes in the range 9590 - 9992 are included in the Hematopoietic Rules. Mycosis Fungoides [9700/3] is included in this range. Therefore, the SEER MP/H Rules do not apply to mycosis fungoides.

This case should be accessioned as a single primary: mycosis fungoides [9700/3] of the skin, NOS [C449]. Per Rule M2 abstract a single primary when there is a single histology.

Note that in the Primary Site(s) section of the Heme DB, it states the primary site must always be coded to skin (C440 - C449) for mycosis fungoides. Because the primary site is stated in this section of the Heme DB, it is not necessary to use the Primary Site Rules to determine the primary site. Code the primary site to C449 [skin, NOS] because the patient has multiple sites of skin involvement and there is no documentation indicating which subsite of skin was the origin of the mycosis fungoides.

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 not be accessioned. In situ lymphoma is not reportable for any of the standard setters (CoC, NPCR, or SEER). In the Case Reportability Instructions, the NOTE under Rule 3 states, "Do report in situ (/2) lymphomas."

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 patient only has two primaries: cutaneous T-cell lymphoma diagnosed in the 1980s and diffuse large B-cell lymphoma of the bowel diagnosed in 1991.

The DLBCL of the brain does not represent a new primary. It is progression of the 1991 disease process with the same histology. Under the Alternate Names section in the Heme DB, one synonym for DLBCL is "Primary DLBCL of the CNS." The histology code for both the 1991 bowel neoplasm and the current CNS neoplasm is 9680/3. Per Rule M2, a single histology is a single 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.