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Physician note: Bone marrow shows 10% involvement with low grade lymphoma. Assessment: Low grade mature B cell lymphoma with IgM paraprotein - macroglobulinemia.

The multiple primaries calculator indicates two primaries are to be reported. However, the physician stated that Waldenstrom's macroglobulinemia is another name for this patient's lymphoma.

There were no enlarged lymph nodes seen on the CT scan. The proposed treatment for this patient is Rituxan for the macroglobulinemia.

Pituitary blastoma was not added to Table 3 (Specific Histologies, NOS, and Subtypes/Variants) of the 2018 Malignant CNS and Peripheral Nerves Solid Tumor Rules as part of the December 2020 update. This is a new malignant CNS histology for 2021 and later. Not including this histology in Table 3 results in the registrars being required to check another source to correctly code this histology. If this histology cannot be used for cases diagnosed prior to 2021, should that diagnosis year clarification be included in the STR?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Please comment on these specific scenarios.

1. A prostate biopsy is suspicious for adenocarcinoma, but the urologist is not considering this malignant, nor is the urologist treating for malignancy, but there is no clear statement from the physician that this is not reportable.  Should this case be abstracted?
2. A prostate biopsy is suspicious for adenocarcinoma, and there is a statement by the physician that this is not yet malignant. Should this case be abstracted?
3. Prostate MRI with PIRADS 4 or 5; however, the treating urologist does not call this malignant and is not treating patient for prostate cancer.  [Example: 2024 Prostate MRI - PI-RADS 4. Follow up with Urologist states patient to repeat Prostate MRI in 1 year and continue with yearly PSA levels and there is no mention of prostate cancer other than the PI-RADS score]. Should this case be abstracted?

Please confirm correct coding for grade for papillary urothelial carcinoma of the bladder/renal pelvis and urothelial carcinoma of the bladder/renal pelvis. SEER Manual 2014 and 2015 state: "Do not use these tables to code grade for any other groups including WHO (CNS tumors), WHO/ISUP (bladder, renal pelvis), or FIGO (female gynecologic sites) grades." They also state "In transitional cell carcinoma for bladder, the terminology high grade TCC and low grade TCC are coded in the two-grade system" in the Grade section. These statements are not included in SEER Manuals prior to 2014.

Please discuss the significance of JAK2 point mutation.

Example: Bone marrow biopsy showed hypercellular marrow with increased megakaryocytes associated with JAK2 point mutation consistent with myeloproliferative syndrome. Path comment: While the morphologic changes would be compatible with a myeloproliferative syndrome, they are not specific for this as similar findings can be seen in reactive conditions. However, a molecular diagnostic test demonstrated a positive JAK2 point mutation which would support the diagnosis of myeloproliferative syndrome. In summary, the combined histologic and molecular diagnostic findings support a myeloproliferative syndrome. The differential diagnosis would be between polycythemia vera and essential thrombocythemia. Subsequent clinical diagnosis: polycythemia vera.

Polycythemia, NOS is not reportable. However, there is a statement in the Heme Manual Glossary for JAK2 that states, "When JAK2 is positive, the MPN is definitely reportable." Does a positive JAK 2 always mean there is a reportable myeloproliferative disorder or must there also be an associated statement of a reportable neoplasm (e.g., myeloproliferative disorder, polycythemia vera, or essential thrombocythemia)?

Preoperative diagnosis was familial polyposis with rectal and rectosigmoid cancer.

The pathology report from the colon resection showed:

1. 5 mm tumor in the sigmoid. Histologic type: adenocarcinoma.

Gross description: The mucosa of the colon is tan pink with polyposis throughout; more than 1000 tan sessile polyps.

Should this be a single primary per MP/H Rule M3, histology coded to 8220/3 [familial polyposis] per MP/H Rule H17, and primary site coded to C199?

Previous instructions from the CDC (Cancer - Collection and Coding Clarification for CNS Tumors - NPCR) stated that non-malignant blood vessel tumors in CNS sites are reportable and should be coded to the CNS site in which they arose. SINQ 20081113 also states that a blood vessel tumor, cavernoma/cavernous hemangioma, in the brain is reportable. However, SINQ 20120034 contradicts this previous answer stating the site should be coded to C490 [blood vessel] for a blood vessel tumor (venous angioma) in the brain.

If blood vessel tumors arising in a CNS site are no longer reportable, please specify the site/histology codes for these non-reportable tumors and when this change took place.

Previous SINQ 20091100 has a similar scenario and the instruction was to code as lentigo maligna (8742/2); however, it does not appear to be applicable to cases diagnosed after 2020.

The WHO Blue Book does not list melanoma, lentiginous type or lentiginous melanoma in situ as an alternate term for lentigo maligna and neither do the STR or the ICD-O-3.2.

Procedure stated = Bilateral skin-sparing mastectomies, left axillary sentinel lymph node biopsy. On the pathology report it indicates two additional lymph nodes were removed that were not SLN. The axillary aspect measures 2 x 2 x 1 cm. Two lymph nodes are identified ranging from 0.5 up to 1 cm. The lymph nodes are bisected and entirely submitted. Final Diagnosis Left breast, mastectomy including nipple: no residual carcinoma; FINAL DIAGNOSIS for LN = Lymph nodes, left axillary sentinel #1; excision: Two lymph nodes examined - negative for tumor (0/2); Two lymph nodes - negative for tumor (0/2)