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This case is not reportable as is. The clinical diagnosis on the 9/13/13 MRI was "cerebral cavernous angiomas," but the final impression on the MRI was a re-demonstration of the numerous scattered foci consistent with cavernous malformations seen on the previous 9/9/11 MRI. There was no reportable statement of cavernous angioma. Cavernous malformation is not a reportable neoplasm; it has no valid ICD-O-3 code.

Vascular tumors of the CNS are reportable when they arise in the dura or parenchyma of the CNS. When they arise in blood vessels or bone, they are not reportable. Do not report vascular tumors when there is not enough information to determine whether they arise in the dura or parenchyma or elsewhere.

For cases diagnosed 2007 or later, accession a single primary, papillary transitional cell carcinoma of the bladder and ureter [C689, urinary system, NOS] diagnosed in 2010. The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Urinary MP Rules because site specific rules exist for this primary.

Start at the MULTIPLE TUMORS module, rule M3. The rules are intended to be reviewed in consecutive order within a module. This patient has urothelial tumors in two or more of the listed sites (bladder, ureter and renal pelvis) diagnosed within 3 years.

When C689 is assigned because tumors of the bladder and tumors of the ureter were determined to be a single primary and the site of origin is not known (as in this example), rule M8 is applied when a subsequent tumor is diagnosed in one of the listed sites. However, if site C689 [urinary system, NOS] was assigned for other unknown urinary primary site situations, rule M8 would not be used.

Rule M8 was written specifically for urothelial tumors in the renal pelvis, ureter, bladder and urethra. Paraurethral gland [C681] and overlapping lesions of urinary organs [C688] do not belong in rule M8.

We will add this issue to the list of possible revisions for the next edition of the MP/H Rules.

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

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]. Leukemias are coded to the bone marrow per the Heme DB.

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 soft tissue of the back, NOS [C496] per Rule PH24 and the Abstractor Notes in the Heme DB for B-cell lymphoma intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma.

Code the primary site to the organ when lymphoma is present only in an organ. The lesion is described as epidural (tissue surrounding the dura) and involving paraspinal muscle, NOS. Both are connective or other soft tissues of the trunk, NOS [C496].

B-cell lymphoma intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma is a synonym for DLBCL 9680/3. When there is no primary site listed in the Heme DB, go to the Abstractor Notes. In the Abstractor Notes section it states that patients present with lymphadenopathy OR mass lesions in extranodal sites.

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.

Abstract this case as a single primary. Code the histology to 9732/2 [multiple myeloma].

Review the Abstractor Notes section in the Heme DB for multiple myeloma. It states that in multiple myeloma there is generalized bone marrow involvement and that extramedullary involvement is diagnostic of advanced disease. This is a case of advanced multiple myeloma.

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

Code the histology to diffuse large B-cell lymphoma [9680/3] per Rule PH11.

Code the histology to 9680/3 [diffuse large B-cell lymphoma] when DLBCL and any other non-Hodgkin lymphoma (follicular lymphoma, grade 3 in this case) are present in the same anatomic location. DLBCL is coded because it is the more aggressive histology.

NOTE: This answer assumes these two histologies are present simultaneously in the same anatomic location. Per Rule M4, this is a single 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.

Per Appendix F, do not report this case based on the information provided.

The oncologist likely used the pathology report and clinical factors to determine the diagnosis of hemophagocytic lymphohistiocytosis, which is not reportable. Hemophagocytic lymphohistiocytosis is caused by an over stimulated immune system (infection, etc.). This clinical syndrome is associated with a variety of underlying conditions. To be reportable, it must state "fulminant hemophagocytic syndrome" (in a child) to be reportable (9724/3).

The pathology report for this case is not definitive. It states that the process "may" represent the EBV-associated lymphoproliferative disorder in children.

Follow back on this case to confirm reportability if possible.

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

Transformations do not always indicate a multiple primary is to be reported. Always apply the M Rules to determine the number of primaries. Refer to Rules M8-M13 in the Heme Manual address to determine the number of reportable primaries when chronic and acute neoplasms (transformations) are indicated on a case. Do not use the MP Calculator to determine the number of primaries unless the M Rules direct you to use it.

This case should be accessioned as two primaries, chronic lymphocytic leukemia [9823/3] diagnosed in 2000, and diffuse large B-cell lymphoma [9680/3] diagnosed on 04/26/2012 per Rule M10. Abstract a new primary when a neoplasm is originally diagnosed as a chronic (less aggressive) neoplasm (CLL) and there is a second diagnosis of an acute neoplasm (DLBCL) more than 21 days later.

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.