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For cases diagnosed 2021 or later, use ICD-O-3.2 to determine reportability. Use the Solid Tumor Rules to determine the number of primaries to report and the histology to code for tumors that are reportable. Do not use the Solid Tumor Rules to determine reportability.

ICD-O-3.2 was implemented by the North American standard setters as of 1/1/2021 and it is the basis for reportability for cases diagnosed as of 1/1/21. See 1.a on page 6 in the 2021 SEER manual, https://seer.cancer.gov/manuals/2021/SPCSM_2021_MainDoc.pdf

WHO 4th edition Tumors of the Urinary System has proposed ICD-O code 8323/1 for clear cell papillary renal cell carcinoma. This has not been approved for implementation by the standard setters. Continue assigning 8323/3 for clear cell papillary renal cell carcinoma.

Sarcomatoid RCC is listed as a synonym for RCC 8312/3. This is correct per WHO and our SME. Do NOT code sarcomatoid RCC to 8318/3. 

Code the primary site to vulva. Use text fields to record the details.

According to the WHO classification, several types of primary vulvar mammary-like carcinoma have been reported. It is rare and is thought to arise from specialized anogenital mammary-like glands within the vulva. It does not arise from ectopic breast tissue and is does not represent metastatic breast carcinoma.

For cases diagnosed prior to 1/1/2010:

This is one primary. Code as 9699 [Marginal zone B-cell lymphoma, NOS].

The first biopsy was not conclusive. The biopsy one week later was more definitive. The reports are describing a difference between specimens, not a difference in disease.

According to the WHO classification, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is an extranodal lymphoma with B-cells, cells resembling monocytoid cells, small lymphocytes and scattered immunoblast and centroblast-like cells.

For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

For cases diagnosed prior to 1/1/2010:

For the hematologic diseases, do not accession the case unless there is a definite positive diagnosis. A positive diagnosis, such as "Refractory anemia" must be stated in order to code that diagnosis. Other words associated with the positive diagnosis, such as "sideroblasts" are NOT to be used alone to assume a diagnosis.

Decreased sideroblasts does not make a diagnosis of Refractory anemia with sideroblasts. The sideroblasts for 9982/3 [Refractory anemia with sideroblasts] are characteristic in rings, and are INCREASED to make the diagnosis.

Based on the information provided, this case is not reportable. The final path diagnosis is not a reportable disease. The comment further states that the overall picture is not diagnostic of a specific entity. Therefore, it should not be reported at this point.

For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

Do not use FIGO grade to code the grade field. See the sentence below the table in Instruction #6 in the Grade Coding Instructions for cases diagnosed 2014 and later, http://seer.cancer.gov/tools/grade/

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

There is no time limit set to update histology to a more specific disease process if a patient has an initial NOS histology identified. Unlike solid tumors, hematopoietic and lymphoid neoplasms may take a year or more to manifest the specific disease. This is simply a part of the "disease characteristics."

Abstract a single primary per M2, a single histology represents a single primary. Code the histology to 9983/3 [MDS/RAEB-2.]

The Heme DB guidelines were interpreted correctly. MDS/RAEB can transform to AML and would be two separate primaries there had also been a reportable diagnosis of AML. The 7/2/2010 peripheral blood showed MDS and a clonal abnormality that was "compatible with MDS/AML." The 10/7/2010 bone marrow biopsy showed only RAEB-2 with "evolution towards AML with myelodysplasia related changes." Ambiguous terminology is only used to help determine reportability; it not used to code a more specific histology. In this case, there was only ambiguous terminology used to describe the AML.

It is important to understand the implication of incorrectly assigning histology codes for hematopoietic and lymphoid neoplasm using ambiguous terminology. Using this case as an example, the patient was not treated until three months after the 7/2/2010 peripheral blood diagnosis of MDS compatible with MDS/AML. The medical literature indicates that AML, if left untreated, is usually fatal within 1-3 months. The treatment given 10/6/2010, 3 months after the "compatible with" diagnosis, was a drug used to treat MDS and not AML.

The other issue with this case is that the bone marrow examination, which is more reliable than peripheral blood, showed only "evolution towards AML." This means that the bone marrow is exhibiting the changes seen in the final stages of MDS prior to progression to AML. Wait for a definitive diagnosis of AML and/or treatment for AML before abstracting the second 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 tumors diagnosed prior to 2007:

This is a single primary. According to Exception 3 of Multiple Primary Rule 6 for multiple tumors, combinations of Paget disease and ductal carcinoma are a single primary. The histology code for this case is 8541 [Paget disease and infiltrating duct carcinoma]. Assign CS extension code 10 [confined to breast tissue] based on the information above.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.

The piece of bone was likely removed to access the maxillary sinus and would not be a separate organ. Use the "All Other Sites" surgery coding schemes to code this primary. For cases diagnosed 1/1/2003 and after: Code the Surgery of Primary Site field to 40 [Total surgical removal of primary site]. Code the Surgical Procedure of Other Site field to 2 [Non-primary surgical procedure to other regional sites]. The maxillectomy was not performed in continuity to the turbinectomy and should be coded in this field rather than the Surgery of Primary Site field.

The code in Surgical Procedure of Primary Site should correspond to the most invasive, extensive, or definitive surgery when the patient has multiple surgical procedures of the primary site even if there is no residual tumor found in the pathologic specimen from the more extensive surgery. The timing of the procedures does not affect the code choice.

Assign code 27 for the first example. Assign code 24 for the second example.

The lung H rules and tables have been updated to include histologies that CAP identifies using the term "predominant" in the diagnosis. Example: Code adenocarcinoma, lepidic predominant, to 8250/3 rather than 8140/3. When the final pathology diagnosis includes more than one "predominant" adenocarcinoma subtype such as acinar, solid, or lepidic, then code the type with the greatest percentage according to Lung Solid Tumor Rule H7.