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Default the coding of the Primary Site field for chordomas to the bone where the tumor began in the body if the primary site is not clearly stated to be soft tissue. Bone is often the primary site for chordomas.

Based on advice from pathologist consultants: This is one of those situations where we can be quite comfortable with a default, in this case to bone, not soft tissue. Chordoma is a tumor arising in the nucleus pulposis, presumably from remnants of notochord - thus its exclusive origin is in the sacrococcygeal region, spheno-occipital region, and vertebral bodies, otherwise known collectively as the axial skeleton. Any "chordoma" in soft tissue (with no relationship to axial skeleton) is probably a myxoid chondrosarcoma or parachordoma (extremely rare).

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 tumors diagnosed prior to 2007:

Code Histology to 8255 [Adenocarcinoma with mixed subtypes]. This is a single tumor containing both a scar carcinoma and a bronchiolo-alveolar carcinoma--use 8255. The synonym for 8255 is adenocarcinoma combined with other types of carcinoma (not just subtypes).

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.

Based on the information provided, this case is not reportable. Low grade B-cell lymphoproliferative disorder is not reportable, nor is monoclonal gammopathy. There is no definitive diagnosis for marginal zone or lymphoplasmacytic lymphoma. The terminology used includes "question" and "versus" which are not acceptable ambiguous terms for reportability. If possible, follow up with the physician regarding the definitive diagnosis.

Donor lymphocyte infusion (DLI) is coded as immunotherapy. The lymphocytes are donated by the same person who donated the original stem cell transplant. The lymphocyte infusion creates an immune response in which the T-cells are activated to attack the cancer cells.

See "Treatments" for CLL/SLL (9823/3)

Do not assign a behavior code of /2 to these cases unless you have a way to flag them so that they are not reported to the standard setters as in situ cases. Work with your state central registry to ensure that these cases are not unintentionally included in state case submission.

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

Aplastic anemia is not reportable and it is not an alternative name for refractory anemia.

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 tumors diagnosed prior to 2007:

Assign code 8255 [Adenocarcinoma with mixed subtypes]. According to histology rule #4 for a single tumor on page 86 of the 2004 SEER manual, use a combination code if one exists.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.