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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] per Rule PH5 when CLL/SLL [9823/3] involves the bone marrow.

In the later stages of CLL/SLL, there may be involvement of bone marrow AND lymph node(s), lymph node region(s), organ(s), or tissue(s). As long as the peripheral blood and/or bone marrow are involved, the primary site is bone marrow.

WHO states that the diagnostic criteria for CLL versus SLL is not clearly defined. According to WHO guidelines, it is better to code to CLL/SLL and code the primary site to bone marrow when the marrow is involved and to lymph nodes, organ, or tissue when there is no bone marrow 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 1998-2003:

Code the EOD-Extension field to 20 [Involvement of two or more lymph node regions on the same side of diaphram]. The AJCC lists mesenteric as a core nodal region, but does not list retroperitoneal lymph nodes as a part of this region, so retroperitoneal is a separate region.

The EOD staging scheme for lymphoma uses lymph node REGIONS as the criteria for assigning the extension code. Use the AJCC Cancer Staging Manual as the definitive source for classifying lymph node regions, not the ICD-O-3. If it is a separate LN region per the AJCC, it is coded in the EOD as a separate region.

According to the AJCC curator, the nodal regions are defined in Kaplan's book on Hodgkin disease. Bilateral cervical, or axillary, or hilar, or pelvic, or inguinal nodes count as two regions. Mediastinal and para-aortic lymph nodes count as one region regardless of laterality as they are centrally located. A large mediastinal mass constitutes one region involved regardless of the size.

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

Per PH Rule22, code the primary site to C779 [lymph nodes, NOS].

Rule PH22 is a default rule for lymphomas that is used when there is no other information regarding the primary site and the Heme DB does not indicate a primary site under its Primary Site(s) section. Rule PH27, code the primary site to unknown [C809], does not apply. Only use C809 [unknown] as the primary site when there is no evidence of lymphoma in lymph nodes AND the physician documents that the lymphoma originates in an organ(s).

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 M7 applies when comparing the 2008 diagnosis to the 2013 diagnosis: multiple primaries.

Rule M8 applies to the tumors identified in 2013: single primary.

Based on the information provided, code the primary site for 2013 to C689 because there is no indication of the site of origin among the involved sites.

The primary site is C482 [Peritoneum, NOS]. "Paracaval" means alongside or near the vena cava.

Code the site in which the primary tumor originated.

For cases diagnosed between 1998-2003:

Lymph nodes that are only "keratin positive" would not be coded as involved lymph nodes. The pathologist uses this expression to mean that the nodes stained positive for keratin that does not mean they are also involved with cancer.

However, if the pathologist uses these stains to make a definitive diagnosis of metastatic carcinoma (i.e., uses the expression "keratin positive for metastasis"), then code the nodes as involved.

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

Per Rule PH30, one is to use the to determine the primary site and histology when rules PH1-PH29 do apply. Code the primary site to C421 [bone marrow] because that is the only site listed under the Primary Site section of the Heme DB.

Under the Abstractor Notes section in the Heme DB, it indicates that the bone marrow is always involved, and the white and red pulp of the spleen may be involved with systemic mastocytosis. This is how this patient presented; therefore, the bone marrow is the primary site. The spleen is secondarily involved because the spleen cleanses the blood and the neoplastic cells have infiltrated the red and white pulp of the spleen. The same is true for the lymph nodes. Although the lymph nodes are rarely involved, they may be involved when the patient has systemic mastocytosis.

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 2007 or later, assign histology code 9505/1 [Ganglioglioma, NOS].

According to our pathologist consultant, low grade neuroglial tumor of the fourth ventricle correlates best to the "rosette-forming glioneuronal tumor of the 4th ventricle" which is a new WHO entity. There is no current ICD-O-3 code for this. The best code available at this time is 9505/1.