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Per Solid Tumor Manual Other Sites Rule M6, despite the number of areas of involvement, any presentation of Kaposi sarcoma is always a single primary. The primary site is skin using the Kaposi Sarcoma for All Sites Coding Guidelines (Appendix C, 2023 SEER Manual). 

Does SEER Program Coding and Staging Manual Laterality Coding Instruction #4 preclude the use of code 4 [Bilateral involvement at time of diagnosis...] if a patient presents with KS involvement of only both arms or only both sides of the face?

The final diagnosis on bone marrow biopsy was high grade myeloid stem cell neoplasm, 17% blasts by differential count.  The pathologist further states that this could be classified as “MDS with increased blasts (MDS-IB2) per the WHO 5th edition classification, or MDS/AML per the international consensus classification (ICC).”  FISH and cytogenetics revealed a loss of 7q, but no other AML-related genetic abnormalities. The physician confirms the patient has MDS/AML.

Treatment for estrogen receptor positive (ER+) breast cancer started with tamoxifen (non-steroidal estrogen subcategory) and switched to letrozole (non-steroidal aromatase inhibitor subcategory). Patient being treated with immunotherapy, Avastin (cytostatic agent-antiangiogenesis agent subcategory), and then changed to Atezolizumab (monoclonal antibody subcategory).

Is Atezolizumab a new course of therapy because it is a different subcategory?

Patient had a total abdominal hysterectomy-bilateral salpingo-oophorectomy performed in January 2023 with final diagnosis of myxoid and epithelioid leiomyosarcoma.

Diagnosis comment states: The tumor is 15 cm per report. It grows in nests and poorly formed interanastomosing trabeculae and cords that are separated by abundant myxoid background. The cells have an epithelioid morphology with eosinophilic cytoplasm, large nuclei, and very prominent nucleoli. The mitotic activity is overall low ranging from 1 to 3/10 HPFs.

Immunohistochemical stains performed at the outside hospital showed diffuse positivity for SMA, desmin, caldesmon, and PR. They are negative for CD10, claudin-4, calretinin, HBM45, MART1 (rare weakly positive cells), PANCK, and SOX10. This immunohistochemical profile supports a smooth muscle derivation of this neoplasm. As this tumor is extensively myxoid, diagnostic criteria differ from the spindle cell leiomyosarcoma.

Per Solid Tumor Rules Other Sites, Table 16: Uterine Corpus Histologies, Epithelioid Leiomyosarcoma (8891/3) and Myxoid Leiomyosarcoma (8896/3) are both subtypes of Sarcoma, NOS (8800/3).  Per Rule H21, use a combination code when there are multiple specific histologies AND the combination is listed in Table 2 OR there are coding instructions for the combination in the applicable histology Tables 3-21 OR you receive a combination code from Ask A SEER Registrar.  Since there is no combination listed in Table 2 and there is no instruction for the combination in Table 16, how should the histology be coded for this tumor?

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?

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 was diagnosed with a biopsy-proven 12/2020 LUL SCC treated with radiation only, followed by a right hilar lymph node biopsy in 07/2022, that proved “metastatic pulmonary adenocarcinoma” per pathology and treated with radiation, followed by a biopsy-proven 12/2022 RLL SCC to be treated with immunotherapy only. The imaging never definitively identified a lung tumor that can be assumed to be a primary adenocarcinoma tumor.  In 06/2022, a PET scan only described a “strongly PET positive Rt inferior hilar LN vs infrahilar pulmonary mass,” as well as the subsequently biopsy-proven SCC in the RLL (12/2022 SCC primary). The biopsy path indicates this was a right hilar lymph node metastasis and does not indicate this is an infrahilar pulmonary mass. No other PET positive pulmonary lesions were seen at the time.

The oncologist’s assessment indicates the right hilar node was the only positive finding on the biopsy, and it was unclear if this right hilar node metastasis was from the left lung or if the primary was “not detectable.” The oncologist summarized this as a LUL lung lesion radiated for SCC, a right hilar lesion radiated for adenocarcinoma, and a RLL lung lesion on pathology found to be SCC.

Should the interval occurring metastatic adenocarcinoma be accessioned as a separate lung, NOS primary based on the histology difference? While the Solid Tumor Rules do not apply to metastasis, the oncologist did treat these three malignancies separately and does not indicate the hilar lymph node metastasis was felt to be from either SCC primary.

March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation.

Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement?

The Solid Tumor Rules indicate p16 is a valid test to determine HPV status and can be used to code HPV-associated/-independent.

In this case, we do not know whether the HR-HPV test was done on cytologically malignant cells, or on benign cervical cells. It may be impossible to tell unless 100% of the cytology specimen is malignant, but we will not have access to that information. Also, HR-HPV testing is routine on Pap smears, so this testing does not mean the tumor cells specifically harbor HPV.