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Myxoinflammatory fibroblastic sarcoma (MIFS) (C49._), 8811/1, is not reportable for 2018 based on the 2018 ICD-O-3 New Codes, Behaviors, and Terms list. This is a new histology/behavior not previously listed in ICD-O-3. According to the WHO 4th Ed Tumors of Soft Tissue & Bone, this histology has been given a benign (/1) behavior; however, if the pathologist and/or physician state the tumor is malignant or metastatic, report the case and assign behavior code /3.

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 cases diagnosed prior to 1/1/2010:This is one primary. Assign code 9590 [Malignant lymphoma, NOS]. This is a composite lymphoma. Code to lymphoma when there is any solid tumor (in lymph nodes, tissue, etc.) Code to lymphoma, NOS since this is not purely follicular and there is no code for composite lymphoma.

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.

Assign 8523/3.

The 2013 revision to ICD-O-3 has a new code for mixed acinar ductal carcinoma; however, this new code will not be implemented in the U.S. until 2018 or later. Page 7 of the Guidelines for ICD-O-3 Update Implementation document released by NAACCR 1/1/2014 instructs us to use 8523/3 in the meantime.

For cases diagnosed prior to 1/1/2010:Code 9680/36 [Diffuse large B-cell lymphoma]. This post-transplant lymphoproliferative disorder was diffuse large B-cell lymphoma. According to the World Health Organization, there are two types of post-transplant lymphoproliferative disorder. "Regular" post transplant lymphoproliferative disorder is not a neoplasm and is therefore not reportable to a cancer registry. The second type (sometimes called Hodgkin-like PTLD) is classified as a B-cell lymphoma, which means that it IS reportable.

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 2007 or later, start with rule H1 in the Single Tumor module. Stop at rule H4. Assign code 8263 [adenocarcinoma in tubulovillous adenoma].

Stop at the first rule that applies. Code histology based on a specimen from the primary site whenever available.

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

Accession two primaries per Rule M15 which instructs you to use the Multiple Primaries Calculator to determine the number of reportable primaries. The result is that mycosis fungoides [9700/3] and peripheral T-cell lymphoma [9702/3] represents two primaries.

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.

According to our expert pathologist adviser, renal angiomyoadenomatous tumor ("RAT") is not reportable. He states "l would be reluctant to consider the entity malignant. The authors of the papers describing it do not seem ready to call it malignant either. I agree with calling it LMP, or in this case uncertain malignant potential."

For cases diagnosed 2007 or later:

Endometrioid adenocarcinoma with squamous differentiation is coded to 8570 [Adenocarcinoma with squamous metaplasia].

The following row needs to be added to Table 2 in order to be able to correctly use the MP/H rules to reach this conclusion.

Column 1:   Endometrioid adenocarcinoma

Column 2:   Squamous metaplasia

                    Squamous differentiation

Column 3:   Adenocarcinoma with squamous metaplasia

Column 4:   8570

The change will be made in the next revision of the rules.

Code to metaplastic carcinoma, 8575/3. According to our expert pathologist consultant, "The term 'metaplastic stroma' implies that at least a portion of the carcinoma has undergone a 'metaplastic' change from epithelial in appearance to 'stromal' in appearance. I assume this is what CAP means by 'Invasive mammary carcinoma with matrix production,' which the WHO equates to metaplastic carcinoma."