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Disease History

2018 - Lymphomatoid papulosis (non-reportable)

2020 - Transform to CTCL (and called Mycosis Fungoides specifically) (CTCL/MF same primary)

2021 - Transform to CTCL with large cell transformation

This patient does not have a previous diagnosis of prostate adenocarcinoma nor a previous history of androgen-deprivation therapy.

Does the logic in the Other Sites Solid Tumor Rules (STRs) noted in SINQ 20200052 still apply? This SINQ confirms a diagnosis of mixed prostatic adenocarcinoma and small cell neuroendocrine carcinoma is 8045. This matches the STRs instructions for Rule H21 and Table 2 (Mixed and Combination Codes), row 1. Row 1 indicates a mixed small cell carcinoma and adenocarcinoma is combined small cell carcinoma (8045). For a patient without previous treatment, is this the correct mixed histology code?

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 WHO Blue Books do not include dedifferentiated carcinoma as a valid histology for the breast.  However, there is known to be progression of ductal carcinoma that is essentially dedifferentiation of an estrogen receptor, progesterone receptor, and HER2 breast carcinoma to a triple negative "dedifferentiated" carcinoma which it appears this patient has. Whether we should accession this as a separate 8020/3 primary per M14 is unclear and the Solid Tumor Manual does not address this scenario.

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)?

Patient has a diagnosis of invasive carcinoma of the right breast from a core biopsy on 04/2023.

Subsequent bilateral mastectomy and sentinel node biopsy proves one positive sentinel node and one negative intramammary node.

One month later there is a completion axillary node dissection with 15 nodes negative for malignancy.

Per previous SINQ 20190074, the initial mastectomy and sentinel node excision with intramammary node removal should be coded as Scope of Regional Lymph Node Surgery 6. It is unclear how the resulting axillary dissection should be recorded in Scope of Regional Lymph Node Surgery. There is no code for sentinel node biopsy and 3, 4, or 5 at same time (code 6) PLUS an additional subsequent axillary dissection.

Please provide coding instructions for Sentinel Lymph Nodes Positive, Sentinel Lymph Nodes Examined, and Scope of Regional Lymph Node Surgery in this scenario.

While SEER*Rx does indicate infliximab should be coded as biological response modifier (BRM)/Immunotherapy, the manufacturer website for this medication indicates it is given for: Crohn’s disease, ulcerative colitis, rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and plaque psoriasis. In addition, SEER*Rx does not indicate which primary sites this treatment may be given for. If it is indeed cancer directed treatment, can the typical primary sites be added for clarity?

Case example: Patient is diagnosed with colorectal cancer and also has an existing diagnosis of Crohn’s disease; received surgery and FOLFOX6, as well as infliximab. There was no statement of what disease the infliximab was given to treat.  

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.

The patient was not synchronously diagnosed with multiple tumors, but three separate tumors with three different histologies were diagnosed at different times and no more specific histology was provided for the NSCLC. The timing rules do not apply to this case (the tumors were not greater than 3 years apart and they were not synchronously/simultaneously diagnosed).

While NSCLC is a NOS histology for both adenocarcinoma and squamous cell carcinoma, it is unclear if Rule M8 should apply because NSCLC is not listed in Table 3 (Table 3 is not an exhaustive list). In some situations, Rule M8 would apply if the tumors were different histologies and one of the histologies was not listed in the Table. Does that logic still apply if one of the tumors is NSCLC? If NSCLC is excluded from Rule M8, is Rule M14 the appropriate M Rule for the 2022 NSCLC diagnosis?