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Vascular tumors of the CNS are reportable when they arise in the dura or parenchyma of the CNS and should be coded accordingly. The instructions in the CDC book regarding primary site coding are not the most current instructions.SEER assumed responsibility for brain and CNS reporting instructions in 2007.

The tumor in SINQ 20120034 is not reportable because it arises in a blood vessel. The cavernous hemangioma in SINQ 20081113 is reportable because the primary site is the white matter of the cerebral cortex.

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

There are two reportable primaries for this case -- follicular lymphoma in 2010 and DLBCL in 2012.

First determine the histologies needed to to determine the number of primaries to report. We determined the histologies are follicular lymphoma, grade 1 for 2010 and DLBCL for 2012 as follows:

• Rule M4, a mix of DBLCL and follicular lymphoma, grade 3 is 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). Additionally, the instruction per Rule PH11 indicates one is to code the histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma (i.e., follicular lymphoma, grade 3) are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.

Per the Hematopoietic database, follicular lymphoma (all types are chronic) transforms to DLBCL (acute). Per Rule M 10 instructions, "Abstract as multiple primaries when a neoplasm is as a neoplasm there is a of an neoplasm after the chronic diagnosis." Therefore, abstract the DLBCL as a second primary.

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.

Based on the information provided, this case of adult granulosa cell tumor is malignant and reportable. According to our expert pathologist consultant, "though granulosa cell tumor NOS/ adult NOS is 8620/1, the presence of peritoneal implants or metastases, and/or lymph node metastases indicates the tumor is malignant, and it should be coded /3."

Note that the presence of implants or metastases does not indicate malignancy in the case of low malignant potential ovarian epithelial tumors. Our path expert explains "in contrast, by convention the behavior of borderline/LMP ovarian epithelial tumors is determined by the ovarian primary, and is /1, even though there may be peritoneal implants/metastases, or metastatic disease in lymph nodes. The treatment may vary in these circumstances, but to my knowledge the decision as to the tumor designation remains based on the primary tumor."

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.

For cases diagnosed 2018 and forward, indolent systemic mastocytosis is not reportable (9741/1). Smoldering systemic mastocytosis is reportable (9741/3).

Code the histology to duct and lobular carcinoma in situ [8522/2].

For cases diagnosed 2007 and later, the steps used to arrive at this decision are:

Go to the Breast MP rules found in the Multiple Primary and Histology Coding Rules Manual. Start at the MULTIPLE TUMORS Module Rule M4 because the patient had multiple foci of DCIS and a separate, single focus of LCIS. The rules are intended to be reviewed in consecutive order within the applicable Module. Tumors that are lobular and duct are a single primary.

Go to the Breast Histology rules found in the Multiple Primary and Histology Coding Rules Manual. Start at the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY Module Rule H20 because the patient has multiple foci of DCIS and LCIS. Code the histology as 8522/2 [duct and lobular carcinoma in situ] when there is any combination of lobular [8520] and duct carcinoma.

The DCIS and LCIS are separate tumors. The DCIS was removed by the lumpectomy and the LCIS by the mastectomy. The most representative specimen for the DCIS is the lumpectomy. The most representative specimen for the LCIS is the mastectomy. Both pathology reports must be used in this case to determine the histology.

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 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.

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

This case should be accessioned as two primaries per Rule M15. Code the histology for the first primary to 9698/3 [nodular histiocytic lymphoma. Per the Alternate Names section in the Heme DB, this histology is synonymous with follicular lymphoma, grade 3. Code the histology for the second primary to 9823/3 [B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma].

Nodular histiocytic lymphoma does not transform into CLL/SLL (Transformations to), nor does CLL/SLL transform to nodular histiocytic lymphoma (Transformations from). Rule M15 indicates we are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries in this case because none of the rules from 1-14 apply. Per the calculator, the CLL/SLL is a new primary.

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.