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

Hemophagocytic lymphohistiocytosis (HLH) is not a reportable disease because it is not listed in the Heme DB.

Per our expert pathologist consultant, "HLH is a lymphocyte driven hemophagocytic syndrome which may be either genetically based or caused by over-activated lymphoid cells, often in response to a viral infection. It is an abnormal immune response and is not considered a malignant disease, and is, therefore, not reportable. It is not synonymous with EBV-positive T-cell lymphoproliferative disease of childhood (9724/3)."

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, 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.

Code the Systemic/Surgery Sequence to 7 [surgery both before and after systemic therapy] for this case.

The answers to SINQ 20091055 and 20071102 do not apply to a case diagnosed in 2013. These answers were posted prior to code 7 becoming effective in 2012.

For cases diagnosed 2018 and later

HGSIL of the vulva or vagina is reportable. HGSIL is a synonym for squamous intraepithelial neoplasia, grade III.

Code the primary site to C068 [overlapping lesion of the mouth] per Rule PH24. Code the primary site to the organ when lymphoma is present only in an organ.

This lesion overlaps the left soft tissue of the maxilla (the maxillary gingiva) [C030] and the left vestibule of the mouth [C061]. There is no documentation indicating in which specific site the lesion arose. The maxilla is the upper jawbone. The soft tissue that overlies the maxilla is a part of the oral cavity. It is reasonable to interpret the documentation such that the tumor in the maxilla is an extension of the overlapping oral mucosa tumor.

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 C421 [bone marrow] per Rule PH5. Code the primary site to the bone marrow when the peripheral blood is involved, even if no bone marrow biopsy is performed.

According to the notes for Rule PH5, CLL always has peripheral blood involvement (PH5 Note 1). CLL/SLL may also have involvement of lymph node regions in later stages (PH5, Note 2). For this patient a bone marrow biopsy was not performed but he had extensive lymph node and peripheral blood involvement. Therefore, the primary site is coded to C421. In addition, the physician's documentation specifies this patient has Stage 0 disease which indicates this disease process is being classified as leukemia (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.

Accession a single primary, Hodgkin lymphoma, nodular sclerosis type [9663/3] diagnosed in 2009 per Rule M2.

Accession a single primary when there is a single histology. Note 2 for Rule M2 indicates timing is not relevant. This is disease progression or recurrence and not 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.

This case should be accessioned as a single primary, non-Hodgkin lymphoma (previously called poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell) [9591/3] diagnosed in 1991.

Determining the number of primaries is based on the rules in effect at the time of each diagnosis. The original lymphoma was diagnosed in 1991 and the first transformation to follicular lymphoma in 2008. The pre-2010 rules for coding histology and determining multiple primaries must be applied first because the rules changed for diagnoses occurring 2010 or later. Per the Single Versus Subsequent Primaries Table, poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell [9591/3] is the same primary as follicular lymphoma [9690].

The Heme DB and Manual are used to confirm that the 2010 recurrences of follicular lymphoma, grade 1 [9695/3], and follicular lymphoma, grade 3 [9698/3], are the same primary according to the Heme Calculator check required per Rule M15. Per the Heme DB page, the diagnoses follicular lymphoma, grade 3 [9698/3] and follicular lymphoma, grade 1 [9695/3] are comparable to follicular lymphoma [9690] as stated in the 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.

Laterality coding for lymphomas is based on the primary site not histology. Laterality describes the side of a paired organ or side of the body on which the reportable tumor originated. Determine whether laterality should be coded for each primary.

Laterality coding instructions are located in the SEER Program Coding and Staging Manual. See pages 68-70 in the 2013 manual,

http://www.seer.cancer.gov/manuals/2013/SPCSM_2013_maindoc.pdf.

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, myelodysplastic syndrome (MDS) [9989/3] diagnosed 10/2/10 and chronic myelomonocytic leukemia (CMML-2) [9945/3] diagnosed 6/19/12 per Rule M15. Per Rule M15, use the Multiple Primaries Calculator when rules M1-M14 do not apply.

When myelodysplastic syndrome (MDS) became reportable, the rules in effect at that time resulted in MDS often being the only diagnosis reported when both MDS and a leukemia were diagnosed. Statistics for some leukemias were impacted. Now we report both the MDS and the leukemia for greater accuracy.

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.