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

Hemophagocytic lymphohistiocytosis (HLH) is not a reportable disease because it is not listed in the Heme DB.

Per our expert pathologist consultant, "HLH is a lymphocyte driven hemophagocytic syndrome which may be either genetically based or caused by over-activated lymphoid cells, often in response to a viral infection. It is an abnormal immune response and is not considered a malignant disease, and is, therefore, not reportable. It is not synonymous with EBV-positive T-cell lymphoproliferative disease of childhood (9724/3)."

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].

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.

Code the histology to 9735/3 [plasmablastic lymphoma] and the primary site to C809 [unknown] per Rule PH27.

Code the histology specified when the only histology for the case is preceded by ambiguous terminology. For this case, code the histology to plasmablastic lymphoma because it is the only histology mentioned in the diagnosis.

Per the Heme DB Abstractor Notes section for plasmablastic lymphoma, most patients present with Stage III-IV disease. The positive pleural fluid is likely due to advanced disease. In the absence of any other information for this case, Rule PH27 applies, "Code primary site to unknown primary site 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 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.

Per the Abstractor Notes section, this case represents a single primary. Histology is coded to 9732/2 [multiple myeloma], which is now advanced.

Review the Abstractor Notes section in the Heme DB for multiple myeloma. It states that in multiple myeloma there is generalize bone marrow involvement. It further states that lytic bone lesions and bone tumor masses of plasma cells (plasmacytomas) are signs of advanced disease. According to the Discharge Summary, this patient had multiple lytic lesions and plasmacytoma which indicates advanced disease.

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 reportability of schwannomas was not initially agreed upon by the standard setters. After the issue was discussed by the CoC, NPCR and SEER Technical Workgroup and an agreement was reached. See #2 under Reportability in the Data Collection Answers from the CoC, NPCR, SEER Technical Workgroup http://www.seer.cancer.gov/registrars/data-collection.html#reportability.

The most accurate and most current instruction is to report these spinal tumors when they arise within the spinal dura or spinal nerve roots, or when they are stated to be "intradural" or "of the nerve root." Do not report these tumors when they arise in the peripheral nerves. The peripheral nerves are the portion of nerve extending beyond the spinal dura.

Spinal cord intradural schwannomas originate in spinal nerve roots. Spinal nerve root is best classified as spinal cord, C720.

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

Code the histology to 9823/3 [chronic lymphocytic leukemia/small lymphocytic lymphoma]. This primary was accessioned based on reportable ambiguous terminology. The surgical pathology report was compatible with B-cell small lymphocytic lymphoma/chronic lymphocytic leukemia, "compatible with" is a reportable ambiguous term. A neoplastic lymphoid infiltrate is not a reportable diagnosis. Therefore, a diagnosis compatible with CLL/SLL is coded as histology code 9823/3.

The statement that you do not use ambiguous terms to code histology is intended for those NOS histologies with an ambiguous term being used to describe the subtype.

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.

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.

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.