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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.

Well-differentiated neuroendocrine tumors and grade 1 neuroendocrine tumors of the appendix are reportable because these tumors have a morphology code 8240/3 per the WHO Classification of Tumors of the Digestive System. However, per the ICD-O-3, carcinoid tumors of the appendix have a behavior code of /1 [borderline].

The terminology of neuroendocrine tumors is evolving and current thinking at the international level is that carcinoid/WD NET of appendix is reportable. However, reportability in the United States is based on ICD-O-3. The histology code for "Carcinoid of appendix" is 8240/1; the histology code for a carcinoids of all other primary sites is 8240/3. Until the United States adopts the proposed changes for ICD-O-3, reportability of appendix cases is as follows:

• Carcinoid

• Well-differentiated neuroendocrine tumor

• Grade 1 neuroendocrine tumor

• Well-differentiated neuroendocrine tumor/carcinoid (Pathologist uses both terms in reporting the diagnosis and does not want to choose one diagnosis over the other.)

For cases diagnosed 2018 and forward, indolent systemic mastocytosis is not reportable (9741/1). Smoldering systemic mastocytosis is reportable (9741/3).

This case should be accessioned as a single primary. Code the primary site to the colon and rectum [C199] and the histology to adenocarcinoma in familial polyposis coli [8220/3] per MP/H Rule H17.

For cases of familial polyposis, when the rectosigmoid or rectum are involved, assign code C199 [colon and rectum]. When the rectosigmoid or rectum are not involved, assign code C189 [colon, NOS].

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

This case should be accessioned as a single primary lymphoma of bilateral orbits per Rule M2. Abstract a single primary when there is a single histology. Both orbits showed the same histology. Note 1 for Rule M2 states bilateral involvement of lymph nodes and/or organs 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.

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.

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 PH1. Code the histology as 9680/3 [DLBCL], the histology of the accompanying lymphoma, when the diagnosis is PTLD and any B-cell lymphoma.

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.

Indolent myeloma [9732/3] and smoldering myeloma [9732/2] are reportable terms synonymous with plasma cell myeloma. Monoclonal gammopathy of undetermined significance (MGUS) [9765/1] is not reportable.

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.