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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.

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.

This case is not reportable as is. The clinical diagnosis on the 9/13/13 MRI was "cerebral cavernous angiomas," but the final impression on the MRI was a re-demonstration of the numerous scattered foci consistent with cavernous malformations seen on the previous 9/9/11 MRI. There was no reportable statement of cavernous angioma. Cavernous malformation is not a reportable neoplasm; it has no valid ICD-O-3 code.

Vascular tumors of the CNS are reportable when they arise in the dura or parenchyma of the CNS. When they arise in blood vessels or bone, they are not reportable. Do not report vascular tumors when there is not enough information to determine whether they arise in the dura or parenchyma or elsewhere.

There is currently no rule in the urinary site MP/H Rules for this combination of histologies. The best option is to code the histology to 8045/3 [mixed small cell carcinoma], a combination of small cell with other types of carcinoma. The presence of small cell carcinoma drives the treatment decisions for this case.

This issue will be addressed in the next revision of the MP/H Rules.

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.

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, diffuse large B-cell lymphoma diagnosed in 2011 per Rule M7. Note 4 for Rule M7 states to change the histology code on the original abstract to the more specific histology, diffuse large B-cell lymphoma in this case. There is no time restriction for rule M7. Apply rule PH11 and code the histology as 9680/3 [DLBCL] when both DLBCL and follicular lymphoma are present in the same lymph node(s).

Ambiguous terminology is not used to code a more specific histologic type per the Heme Manual. The information submitted states only that this low grade B-cell lymphoma was "most consistent with follicular lymphoma." The term "consistent with" is an ambiguous term per SEER and cannot be used to code the histology of the 2011 neoplasm as follicular lymphoma. There was no subsequent clinical statement that this patient was diagnosed with follicular lymphoma in 2011.

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.

Although the ambiguous terminology on the pathology report is not used to code the histology to follicular lymphoma, had there been a subsequent clinical statement that this patient had follicular lymphoma, the histology would be coded to follicular lymphoma [9690/3]. A diagnosis of follicular lymphoma followed by a diagnosis of DLBCL more than 21 days later is a new primary per rule M12.

Accession two primaries per Rule M17, neuroendocrine carcinoma [8246/3] of the rectosigmoid junction [C199], and adenocarcinoma in a villous adenoma [8261/3] of the rectosigmoid junction [C199]. There are two tumors with ICD-O-3 histology codes that differ at the third number.

Rule M13 does not apply to neuroendocrine carcinoma. Rule M16 does not apply to this case because there are two specific histologies.

Per Rule M4, this is a single primary. According to Rule M4, one is to abstract a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s).

Per Rule PH11, code the histology to 9680/3 [diffuse large B-cell lymphoma] and the primary site to C422 [spleen]. According to PH11, one is to code the primary site to the site of origin, lymph node(s), lymph node region(s), tissue(s) or organ(s) and histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.