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2021-Right pleural fluid:  Negative for carcinoma.

5/18/2021: Right iliac crest bone marrow core biopsy, aspirate smear, clot section and peripheral blood smear:  Hypercellular bone marrow, morphological findings are suspicious for a myeloproliferative neoplasm. Flow Cytometry: Slight immunophenotypic abnormalities of the myeloid cells. No abnormal B cell, T cell, or NK cell populations identified. Normal female karyotype. KARYOTYPE: 46,XX[20]. Negative for deletion of 13q14.3 (D13S319) by FISH. Negative for deletion of 13q34 (LAMP1) by FISH. Negative for hyperdiploidy involving chromosome 9 by FISH. Negative for t(9;22)(q34;q11.2) by FISH. Negative for deletion of the EGR1 gene on 5q31 by FISH. Negative for monosomy 5 by FISH. Negative for deletion of 7q31 by FISH. Negative for monosomy 7 by FISH. Negative for deletion of 20q12 by FISH. Negative for trisomy of chromosome 8 by FISH.

6/4/21-Left adrenal; biopsy: poorly-differentiated malignant neoplasm with extensive necrosis. Immunohistochemical stains show the neoplastic cells to be negative for CK7, TTF-1 and p63. Negative CK7 and TTF-1 would argue against a lung primary. Correlation with clinical and radiological findings is advised.

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Example: Patient is diagnosed with grade 1 well-differentiated neuroendocrine tumor of the ileum, confirmed on ileocolic resection in 2023. The final diagnosis is a 2.8 cm ileal mass, with focal lymph-vascular invasion and a single 0.6 cm tumor deposit within mesenteric fat; primary tumor completely resected with widely negative margins and 10 regional nodes negative for malignancy.

According to AJCC, mesenteric masses less than 2 cm should be stated in the pathology report as being present and collected by registrars but do not affect stage. EOD Regional Nodes has a code for large mesenteric masses greater than 2 cm only. How should we record these smaller tumor deposits if they are not supposed to affect stage?

The 2023 SEER Manual instructions contain a new note in Hematologic Transplant And Endocrine Procedure, Coding Instruction 6, regarding bilateral salpingo-oophorectomy (BSO) when performed for hormonal effect for breast, endometrial, vaginal, and other primary cancers. While we have observed BSO being performed for breast primaries, we do not recall ever seeing a statement for endometrial or vaginal primaries regarding a “BSO being done as hormonal manipulation” when scheduled either with or without a hysterectomy being performed simultaneously. As a result, we are not clear exactly when a BSO would be captured in the Hematologic Transplant And Endocrine Procedure field for these gynecologic primary sites.

Also, if these types of procedures are Hematologic Transplant And Endocrine Procedures, are they also captured and coded in the Surgery of Primary Site codes that directly relate to those same organs? Does timing have any effect on the coding of either field?

Small cell carcinoma is a specific type of neuroendocrine carcinoma for the lung. However, Table 3 lists neuroendocrine carcinoma, NOS as the more specific subtype/variant in Column 3.

Using Lung Solid Tumor Rules, Rule H6, a diagnosis of poorly differentiated neuroendocrine carcinoma (small cell carcinoma)” would be coded as 8246, instead of 8041, because there are two histologies under consideration (an NOS and a subtype/variant in Table 3), and the rule tells us to code the subtype/variant. However, small cell carcinoma is more specific than the NOS diagnosis (neuroendocrine carcinoma, NOS). Should Table 3 be updated to reflect which histology is the NOS and which is the more specific?

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.

The patient has adenopathy in multiple lymph node regions above and below the diaphragm and a lymph node biopsy pathology proved CLL/small lymphocytic lymphoma (SLL). Further work-up with peripheral blood proved an abnormal CD5-positive B-cell population comprising only a small percentage of the white blood cells (WBCs). The pathologist noted this neoplastic B-cell population comprises “3.5% of white blood cells and has an immunophenotype characteristic of CLL/SLL and is similar to the recent lymph node biopsy in this patient.” The managing physician indicated this was a Lugano Stage III SLL. The registrar coded the peripheral blood involvement in EOD Primary Tumor.

If this small percentage of WBCs with an abnormal B-cell population is included in EOD Primary Tumor as peripheral blood involvement, then this would indicate peripheral blood/bone marrow involvement and primary site would need to be coded to C421 per Rule PH5. Rules PH5 and PH6 confirm primary site must be coded C421 if peripheral blood or bone marrow are involved.

Is there a cutoff value for these abnormal B-cell populations in the peripheral blood? Or should these abnormal B-cell populations be ignored unless the pathologist states the abnormal B-cell population is consistent with CLL/SLL (not just immunophenotypically characteristic of CLL/SLL)?

The patient has a history of ganglioglioma, WHO grade I, involving the deep right parietal lobe diagnosed on resection in 07/2012. Tumor recurrence in 2017 was treated with radiation.

The patient then had right temporal tumor biopsy and resection 06/2021 with final diagnosis of anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III. Pathologist notes that the tumor demonstrates a ganglioglioma with frequent mitoses and possible vascular proliferation. Subsequent consult findings support an anaplastic glioneuronal tumor, compatible with progression of the patient's ganglioglioma that is post-irradiation. However, the pleomorphic and epithelioid areas are also reminiscent of pleomorphic xanthoastrocytoma, which may occur in combination with ganglion cell components. There is no related SINQ to code this histology.

Since the peripheral nerves are included in the Malignant CNS schema of the Solid Tumor Rules, neither the differences in subsite nor timing indicate these are separate primaries (Rule M10 indicates a single primary). However, these are separate MPNSTs in different sites and the tumors are not stated to be metastasis. Additionally, these are treated as separate primaries by the managing physician.

While the malignant CNS tumors do not take timing into account, is this correct for these peripheral nerve tumors that are often treated similarly to soft tissue tumors? Should Rule M8 be updated to include tumors in different peripheral nerve subsites?

The patient had residual tumor following the 2018 transsphenoidal resection and underwent an additional surgery after the residual tumor increased in size. Since pituitary adenoma/pituitary neuroendocrine tumor (PitNET) is a new malignant neoplasm for cases diagnosed 2023 and later, should this be a new primary per M5? Or do we disregard the change in behavior and apply rule M2 (single tumor is a single primary) for this scenario?

This patient had serosal involvement and the pathologist and managing physician staged this as pT4a disease. This extension seems best captured by EOD Primary Tumor code 500. Additionally, the patient had discontinuous metastatic involvement of the peritoneum, and this was staged by the pathologist and managing physician as pM1b (Intraperitoneal metastasis only, including peritoneal mucinous deposits containing tumor cells). Although this peritoneal involvement was present in the right lower quadrant, it was staged as distant metastatic disease and not as part of the primary tumor category. However, currently EOD Primary Tumor code 600 would seem to apply since the peritoneal tumor was in the right lower quadrant. Code 600 is defined as mucinous tumors with peritoneal involvement confined within right lower quadrant. This EOD Primary Tumor code and the physician’s M category assignment do not align; the physician has staged this as distant metastasis (M category, not the T category). Should the peritoneal metastasis (even limited to the right lower quadrant) be included in the EOD Mets field and not in the EOD Primary Tumor field? In other words, should the peritoneal involvement included in EOD Primary Tumor code 600 be reclassified in EOD Mets code 30 (Intraperitoneal metastasis (peritoneal carcinomatosis) WITH or WITHOUT peritoneal mucinous deposits containing tumor cells)?