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For cases diagnosed 2010 and later

Please see the Hematopoietic database, https://seer.cancer.gov/seertools/hemelymph/

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

Code the primary site to C421 [bone marrow] and the histology to 9680/3 [diffuse large B-cell lymphoma] per Rule PH26. B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma is listed under Alternative Names section of the Heme BD for DLBCL [9680/3]. This patient has bone marrow involvement only. The Note for Rule PH26 instructs one to code the primary site to the bone marrow when all physical exams or work-up were negative for lymph node, tissue, or organ involvement OR no other work-up was done.

The histology is not coded 9684/3 [malignant lymphoma, large B-cell, diffuse, immunoblastic, NOS]. This histology code became obsolete in 1/1/2010. Diffuse large B-cell lymphoma, immunoblastic variant is also listed under Alternative Names section of the Heme BD for 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.

The histology is coded to lobular carcinoma, NOS [8520/3] because the mastectomy (the most representative specimen) showed only lobular carcinoma.

The MP/H Rules state to code the histology from the most representative tumor specimen examined. Although this patient underwent neoadjuvant treatment, there is no indication that the ultrasound-guided biopsy contained more tumor than the mastectomy. The mastectomy is the most representative specimen and should be used to code the histology.

For cases diagnosed 2007 or later, the correct histology code is 8244/3 [composite carcinoid]. The steps used to arrive at this decision are:

Step 1: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Colon Histology rules because site specific rules have been developed for this primary.

Step 2: Start at the SINGLE TUMOR module, rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at rule H9. Code the histology as 8244/3 [composite carcinoid] when the diagnosis is adenocarcinoma and carcinoid tumor.

Neuroendocrine tumor, grade 1 (G1) is synonymous with carcinoid tumor [8240/3] for the purpose of rule H9.

Mast Cell Activation Syndrome (MCAS) is not a reportable neoplasm unless it is specifically stated to be a result of a mast cell proliferative disorder that is reportable.

Per our expert pathologist, MCAS is a relatively new term used for conditions in which patients experience the symptoms of mast cell mediators in the absence of an increase/proliferation of mast cells. The diagnosis of this group of disorders is based in part on a complex of symptoms and on the demonstration of no increase in mast cells. Some of these diseases are difficult to separate from mastocytosis (which is reportable). Currently, this group of disorders is not part of the systemic mastocytosis/mast cell leukemia/mast cell sarcoma spectrum.

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

Without further information as to the type of acute myeloid leukemia, code the histology to 9861/3 [acute myeloid leukemia, NOS]. If further information on the specific acute myeloid leukemia becomes available, update the histology code.

Document that the pathology report states the acute myeloid leukemia is a "non-M3 type" in a text field. This documentation will help explain the choice of 9861/3 for this case. M3 refers to one of the eight FAB subtypes described by a group of French, American, and British leukemia experts in the 1970's who divided acute myeloid leukemias into subtypes, M0 through M7. They classified the disease based on the type of cell from which the leukemia developed and how mature the cells were. This was based largely on how the leukemia cells looked under the microscope after routine staining.

In this case, all we know is that the histology does not pathologically represent the M3 (acute promyelocytic leukemia (APL)) form of acute myeloid leukemia. We do not know which type of acute myeloid leukemia it does represent.

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 1/1/2022

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.

Code the grade to 6 [B-cell] for all cases of 9811/3 [B lymphoblastic leukemia/lymphoma] per Rule G3 in the Heme Manual.

Acute lymphoblastic leukemia, NOS [9835/3] is an obsolete code and cannot be used for cases diagnosed 2010 and later. The Heme DB indicates the correct histology code is 9811/3 and grade 6 [B-cell] for cases diagnosed 2010 and later.

For cases of acute lymphoblastic lymphoma, NOS [9835/3] diagnosed prior to 2010, use the pathology report information to code the grade. Code the grade as 9 [unknown] if the pathology report does not specify the grade.

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.