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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 a single primary. The histology code is 9596/3 [composite Hodgkin and non-Hodgkin lymphoma].

According to the Single Versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table, 9596/3 followed by 9670/3 is one primary.

For cases diagnosed 1/1/10 and later, 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.

We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3).

The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern.

In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis.

We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns.

Use Rule M2. Abstract as a single primary when there is a single histology.

When you have questions about how to code the primary site, start with the abstractor notes. If the answer isn't found there go to Module 7 (a specific module to help code primary site for lymphomas).

The abstractor notes for DLBCL in this case do not provide information you can use for this case. Go to Module 7 in the PH rules.

Use Rule PH25 Code the primary site to the organ when lymphoma is present in an organ and that organ’s regional lymph nodes. Code the primary site to colon (organ and regional lymph nodes involved). The fallopian tube is secondary involvement. As is common with lymphomas, there can be more than one organ involved. You can differentiate the primary site from the secondary site(s) because of the large colon mass with regional lymph node involvement.

Code Multiplicity Counter to 02 [Two tumors present] for an epithelial ovarian primary involving both ovaries. Do not count the peritoneal implants; they are regional metastasis and not included in the multiplicity counter. An example like this will be added to the manual in the next revision.

For cases diagnosed prior to 2018

Code the grade/differentiation field from the biopsy for this situation. According to experts consulted, hormone therapy does not alter the grade in this case and grade should be coded based on information after hormone therapy when that is the only grade information available.

Assign C211 for anal verge. Anal verge is defined as the lower (distal) end of the anal canal, junction between the skin of the anal canal and the perianal skin, http://www.seer.cancer.gov/manuals/2015/AppendixC/rectosigmoid/coding_guidelines.pdf

Code Urinary System NOS C689 for this case since there are two separate urinary sites involved. Apply instruction #4 when there is a mix of in situ and invasive urothelial tumors.

Based on the information provided, code the primary site to urachus (C677). Primary adenocarcinoma of the bladder accounts for less than 1% of all bladder malignancies. Of these, 20–39% are urachal in origin.

Code the primary site to cranial meninges [C700].