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For cases diagnosed prior to 1/1/2022

A low-grade appendiceal mucinous neoplasm (LAMN) is not reportable. The WHO classification designates LAMN with the behavior code /1 [uncertain whether benign or malignant].

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

This is a reportable diagnosis and should be accessioned with the histology coded to 9975/3 [myelodysplastic/myeloproliferative neoplasm, unclassifiable].

The term is a reportable ambiguous term per the Hematopoietic Coding Manual (Case Reportability Instructions, Rule 4). Also, the patient was treated for a myeloproliferative disorder, making this a reportable clinical diagnosis per the SEER Manual (Reportability, Pg 4, Exception 1).

Myeloproliferative disorder is synonymous with myeloproliferative disease. Myeloproliferative disease is listed as an alternate name for myelodysplastic/myeloproliferative neoplasm, unclassifiable.

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.

Hemophagocytic lymphohistiocytosis (HLH) is not a reportable disease because it is not listed in the Heme DB.

Per our expert pathologist consultant, "HLH is a lymphocyte driven hemophagocytic syndrome which may be either genetically based or caused by over-activated lymphoid cells, often in response to a viral infection. It is an abnormal immune response and is not considered a malignant disease, and is, therefore, not reportable. It is not synonymous with EBV-positive T-cell lymphoproliferative disease of childhood (9724/3)."

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 2007 and later, accession two primaries for each patient, signet ring cell carcinoma of the bladder and invasive urothelial carcinoma of the bladder for patient 1 and mucinous carcinoma of the bladder and non-invasive papillary urothelial carcinoma of the bladder for patient 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 Urinary MP rules because site specific rules exist for this primary.

Start at the MULTIPLE TUMORS module, rule M3. The rules are intended to be reviewed in consecutive order within a module. For both patients, rule M9 applies because the tumors have histology codes that are different at the second (xxx) number.

This guideline will be reviewed for the next version of the MP/H Rules.

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

Change the histology of the original 2010 diagnosis to 9823/3 [chronic lymphocytic leukemia/small lymphocytic lymphoma] based on the review of the 2010 slides. The 2010 diagnosis was revised based on the review of slides and the histology should be changed accordingly. The closest example of this is located in the SEER Manual, Changing Information on the Abstract, instruction 3, example 4.

Histology code 9670/3 [SLL] is obsolete for cases diagnosed 2010 and later. All diagnoses of CLL/SLL, CLL, and SLL are now coded to histology code 9823/3 [CLL/SLL].

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 C774 [inguinal lymph nodes] per Rule PH18. Code the primary site to inguinal lymph nodes [C774] when the site of lymphoma is described only as an inguinal mass. Groin lymph nodes are inguinal lymph nodes.

The diffuse large B-cell lymphoma diagnosed by right tibia biopsy is not a new primary per rule M7 because the histology of the history only case would be coded as 9590/3 [lymphoma, NOS]. No more specific histology is known for the initial diagnosis. Accession a single primary when a more specific histology [DLBCL] is diagnosed after the NOS ONLY histology when the Heme DB Multiple Primaries Calculator confirms the NOS and the more specific histology are the same primary. The right tibial involvement is not used to code the primary site because the patient had chemotherapy for this groin lymphoma prior to diagnosis of DLBCL.

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.

There has been a change in coding practice based on a change in clinical classification of leukemia/lymphomas. In the past, we did, indeed, default to lymphoma when both tissue and bone marrow were involved. The problem was that when only bone marrow was involved, the case was coded to leukemia with a primary site of bone marrow. When lymphoma symptoms developed later, there was a lot of inconsistency in how registries handled these cases. Some coded a new primary "lymphoma;" while others ignored the lymphoma calling it progression.

The clinical world, including the hematopoietic experts in the World Health Organization and the Inter-Lymph Consortium, agreed that for certain neoplasms (CLL/SLL being one of them) it was not useful or practical to code the leukemia and lymphoma separately OR to capture only one of the neoplasms (because these neoplasms almost always progress to lymphoma); so new codes for the leukemia/lymphoma were developed. According to the experts, 9823/3 most accurately portrays the neoplastic process for the neoplasms assigned to a lymphoma/leukemia code.

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 histology to 9950/3 [polycythemia vera], the more specific histology, per Rule PH29. Myeloproliferative syndrome is a non-specific (NOS) histology and polycythemia vera is a specific type of myeloproliferative disease.

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 to 8413/3 [eccrine adenocarcinoma]. This is the most specific code available for this diagnosis.

According to our expert pathologist advisor, "The adnexal glands in the skin, sweat (eccrine) glands and apocrine glands, all have ducts which connect the business portion of each gland to the skin surface. Some of the adnexal tumors have features of differentiation which appear to be duct-like, hence the designation 'ductal.'"

In addition, "The 'squamoid' simply indicates some degree of squamous differentiation, but doesn't alter the usefulness of [code 8413/3] because we have no way of coding anything more specific in this case anyway."

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

Code the histology to diffuse large B-cell lymphoma [9680/3] per Rule PH11.

Code the histology to 9680/3 [diffuse large B-cell lymphoma] when DLBCL and any other non-Hodgkin lymphoma (follicular lymphoma, grade 3 in this case) are present in the same anatomic location. DLBCL is coded because it is the more aggressive histology.

NOTE: This answer assumes these two histologies are present simultaneously in the same anatomic location. Per Rule M4, this 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.