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This case is not reportable. Rosai-Dorfman disease is not listed in the ICD-O-3. To be reportable, a neoplasm must be listed in the ICD-O-3 and originate in a reportable brain/CNS site.

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.

Per Appendix F, do not report this case based on the information provided.

The oncologist likely used the pathology report and clinical factors to determine the diagnosis of hemophagocytic lymphohistiocytosis, which is not reportable. Hemophagocytic lymphohistiocytosis is caused by an over stimulated immune system (infection, etc.). This clinical syndrome is associated with a variety of underlying conditions. To be reportable, it must state "fulminant hemophagocytic syndrome" (in a child) to be reportable (9724/3).

The pathology report for this case is not definitive. It states that the process "may" represent the EBV-associated lymphoproliferative disorder in children.

Follow back on this case to confirm reportability if possible.

For cases diagnosed 2010 and later

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

Do not use FIGO grade to code the grade field. See the sentence below the table in Instruction #6 in the Grade Coding Instructions for cases diagnosed 2014 and later, http://seer.cancer.gov/tools/grade/

For one tumor involving a contiguous site, when there is no tissue specimen available from the primary site, you may code the grade based on the tissue from the tumor in the contiguous site.

This instruction is included in the upcoming grade instruction document.

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

Per PH27, code the primary site to C809 [unknown]. Rule PH27 states one is to code the primary site to unknown [C809] when there is no evidence of lymphoma in lymph nodes AND the physician documents in the medical record that he/she suspects that the lymphoma originates in an organ(s) OR there is multiple organ involvement without any nodal involvement.

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.

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, multiple myeloma [9732/3] diagnosed on 2/3/12 per Rule M8. Abstract the acute neoplasm as a single primary when both a chronic and acute neoplasm are diagnosed simultaneously or within 21 days AND there is documentation of only one positive tissue biopsy.

This patient only had a tissue biopsy of the nasal cavity proving the chronic neoplasm (extraosseous plasmacytoma). The acute neoplasm (multiple myeloma) was diagnosed clinically based on the scans.

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.