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

This case is not reportable. Neither plasma cell dyscrasia nor MGUS are reportable. Smoldering myeloma was given as a possible diagnosis, but never confirmed.

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 C809 [unknown].

Langerhans Cell Histiocytosis (LCH) includes three major groups:

• Unifocal: Slowly progressing disease, involves one organ.

When the disease is both multifocal and multisystem, code the primary site to unknown [C809] because there is no way to identify the origin of the neoplasm in this situation.

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.

Rule PH21 applies to this situation which states to code the primary site to multiple lymph node regions, NOS (C778) when multiple lymph node regions, as defined by ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated. Axillary nodes are coded to C773 and inguinal nodes are coded to C774. There are two lymph node regions involved. Code the primary site to C778 [multiple lymph nodes].

If this patient had only bilateral axillary OR only bilateral inguinal nodes are involved, then PH20 would have applied and you would code to the specific lymph node region mentioned.

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas with low grade dysplasia, also referred to as IPMN adenoma, is not reportable.

IPMN of the pancreas is reportable when stated as "IPMN with high-grade dysplasia," or "IPMN with an associated invasive carcinoma," or  "IPMN with an associated in situ carcinoma."

The case in SINQ 20021099 is stated to have "multifocal high grade dysplasia (so-called borderline tumor and carcinoma in-situ)" and is reportable because there is an explicit statement of carcinoma in situ, not because of the reference to the presence of high grade dysplasia. It is coded 8453/2 [Intraductal papillary-mucinous carcinoma, non-invasive].

Hyalinizing trabecular neoplasm is not reportable.

Hyalinizing trabecular neoplasm, or hyalinizing trabecular tumor, is a synonym for hyalinizing trabecular adenoma [8336/0] in the ICD-O-3. The 2004 WHO classification states that "fine needle aspiration biopsy is often interpreted as papillary carcinoma because of the nuclear features in the tumor."

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

Code the histology to 9950/3 [polycythemia vera], the more specific histology, per Rule PH29. Myeloproliferative syndrome is a non-specific (NOS) histology and polycythemia vera is a specific type of myeloproliferative 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.

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 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.