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

This case is accessioned as a single primary. Code the histology to 9680/3 [diffuse large B-cell lymphoma] and diagnosis date to 2004. Per Rule M2, abstract as a single primary when there is a single histology.

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.

Apply rule PH22 to code the primary site to C779 [lymph nodes, NOS].

While the pathology does not indicate that this particular case represents a B-cell lymphoma, unclassifiable with features intermediate between DLBCL and Burkitt variant, in the Abstractor Notes section of the Heme DB for diffuse large B-cell lymphoma it does indicate that its presentation, "may have lesions in ileocecal region or jaws. Bone marrow and peripheral blood may be involved. Patients present with lymphadenopathy or mass lesions in extranodal site." This patient does have involvement of two extranodal sites and involvement of regional lymph nodes for only one of those sites.

PH22 indicates one is to code the primary site to C77.9 [lymph nodes, NOS] when lymphoma is present in multiple organs and lymph nodes that are not regional for that organ and the origin cannot be determined even after consulting the physician. There are two extranodal sites of involvement and only one chain of lymph nodes is regional to one of those sites so this rule applies.

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 Appendix F, a secondary myelofibrosis is not a reportable case.

Secondary myelofibrosis is not listed as a synonym for primary myelofibrosis in the Heme DB. The term "secondary myelofibrosis" means that the myelofibrosis was caused by, in this case, the essential thrombocythemia.

The diagnosis "consistent with myeloproliferative disorder" is also not a new reportable diagnosis. "Myeloproliferative disorder" refers to a group of diseases (an NOS category) that includes essential thrombocythemia, which was originally diagnosed in 1998, prior to reportability for this disease type.

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 Appendix F, monoclonal B-lymphocytosis of uncertain significance (MLUS) is not reportable.

Some papers point out that a lymphocyte count less than five thousand is equivalent to monoclonal B-lymphocytosis of uncertain significance (MLUS) or monoclonal B-cell lymphocytosis (MBL). A lymphocyte count of five to thirty thousand could be smoldering chronic lymphocytic leukemia (CLL). The diagnosis of MLUS is a benign process that does not meet the criteria for CLL.

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 is a reportable case. Code the histology as malignant gastrinoma [8153/3].

Gastrinomas are usually malignant. This one is apparently present in a metastatic site (periportal lymph node) which confirms the malignancy.

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

Code the primary site to C77.8 [multiple lymph node regions, NOS].

Per Rule PH6, code the primary site to the involved lymph node region(s) when there is no bone marrow involvement or when it is unknown whether the bone marrow is involved. To determine the more specific lymph node subsite to code, use Rule PH21. It indicates one is to code the primary site to C778 [multiple lymph node regions, NOS] when multiple lymph node regions, as defined by the ICD-O-3 (see Table C1: Lymph Node/Lymph Node Chain Reference Table in Appendix C), are involved and it is not possible to identify the lymph node region where the lymphoma originated.

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 9680/3 [diffuse large B-cell lymphoma (DLBCL)].

For CLL (and CLL/SLL), Richter's transformation represents when CLL changes into DLBCL. In this case, there was a biopsy that demonstrated a diagnosis of the chronic disease (CLL/SLL) transforming (Richter's transformation) into an acute disease DLBCL.

Per Rule M8, one is instructed to abstract the acute neoplasm as a single primary when both a chronic (CLL/SLL) and an acute neoplasm (diffuse large B-cell lymphoma (DLBCL)) are diagnosed simultaneously there is documentation of only one positive bone marrow biopsy, lymph node biopsy or tissue biopsy.

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 Rule PH30, use the Heme DB to determine the primary site and histology when rules PH1-PH29 do not apply. Code the primary site to C419 [bone, NOS], assuming there are multiple bones involved in this case. If only one bone is involved, code the primary site to the specified bone.

In the Abstractor Notes section in the Heme DB, it indicates the primary site may differ for LCH in the solitary disease and multisystem disease. This patient has multisystem disease with involvement of the bone, liver, spleen and retroperitoneum. The most common sites for multisystem involvement include three of the four above sites (bone, liver, and spleen).

Determine the primary site based on the knowledge of the usual sites of involvement for this disease, the actual sites of involvement for the case presented, and identifying which sites of involvement are likely metastatic and which are the potential primary sites. There are two potential primary sites of involvement: the bone and the retroperitoneum. Bone is a common site of involvement for LCH while the retroperitoneum is not. Code the primary site to C419 [bone, NOS] because multiple bones are involved for this patient and bone is the most common site for LCH based on the documentation in the Abstractor Notes.

The spleen and liver are typically not primary sites for this disease process. They become involved when there is multisystem involvement because they filter the blood. They are typically sites of metastatic involvement. This information will be added to the ABSTRACTOR NOTE section.

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.

Secondary polycythemia vera is not reportable. See Appendix F.

Primary polycythemia vera is a condition in which there is an overproduction of blood cells due to a neoplastic process. Secondary polycythemia vera is an over production of red blood cells caused by a co-morbidity, in this case, volume depletion.

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.