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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 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?

Table 19: Vulva Histologies of the Other Sites Solid Tumor Rules does not include entries for either keratinizing or nonkeratinizing squamous cell carcinoma in the “Squamous cell carcinoma, NOS” row. However, these are two distinctly different histologies per the ICD-O-3.2.

All other Solid Tumor Rules schemas include an M Rule instructing one to abstract multiple primaries when separate/non-contiguous tumors are two or more different subtypes/variants in Column 3 of the Specific Histologies, NOS, and Subtype/Variants Table for the schema (e.g., Rule M6 for Lung). The timing of these tumors is stated to be irrelevant. The Notes confirm the tumors may be subtypes/variants of the same or different NOS histologies and tumors in column 3 are all distinctly different histologies (even if they are in the same row). However, the 2023 Other Sites schema appears to be missing this rule.

Should these distinctly different histologies be accessioned as separate primaries? Is an M Rule missing from the Other Sites schema to address distinctly different histologies?

Patient had a right thyroid lobectomy on 12/2022, with initial diagnosis of “thyroid carcinoma pending expert consultation for definitive classification.”  The slide review documented in the addendum shows a final diagnosis of “Angioinvasive oncocytic thyroid neoplasm, see comment.”  The subsequent comment states, “I would classify this lesion as an angioinvasive oncocytic thyroid neoplasm with features worrisome for a poorly differentiated oncocytic carcinoma.”  The comment goes on to state, “Additional immunostains were performed which demonstrate the carcinoma cells are positive for thyroglobulin and negative for calcitonin. The diagnosis remains unchanged.”

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.

We are unable to contact the provider.

Patient has a remote history of a right thalamic mass status-post two resections; reported as malignant oligodendroglioma (pathology not received) and chemo/radiation therapy, who recently presented with persistent headaches. Imaging revealed a 3.4 cm heterogeneous lobulated right thalamic mass with coarse calcifications and a probable cystic component.

Pathologist indicates the histologic and immunophenotypic features of this neoplasm are that of relatively circumscribed neuroepithelial tumor without high grade features (mitotic activity, microvascular proliferation, necrosis). Molecularly this neoplasm is characterized by a PATZ1-EWSR1 fusion, which has recently been proposed to be a distinct neuroepithelial tumor entity with a broad histological spectrum.

The SEER Manual states that the Other Therapy data item identifies treatments given that cannot be classified as surgery, radiation, systemic therapy, or ancillary treatment; and the instructions for code 2, Other-Experimental, say to assign this for any experimental or newly developed treatment, such as a clinical trial, that differs greatly from proven types of cancer therapy.  Does this mean that only unclassifiable treatments should be coded in Other Therapy, even if given as part of a clinical trial? 

For example, if a patient is given a drug as part of a trial that is categorized in SEER*Rx as immunotherapy, should it be assigned both Immunotherapy (NAACCR #1410) code 1 and Other Therapy code 2, or only coded in Immunotherapy since it is classified as such?  How should a clinical trial drug be coded if it has a treatment classification in SEER*Rx, but the type of cancer being treated is not listed under the Primary Site or Remarks sections as being FDA approved?  A real case scenario is atezolizumab given for colon cancer as part of a trial; this drug's category is Immunotherapy in SEER*Rx but colon is not listed under Primary Sites or in the Remarks detailing FDA approvals.

Patient has a 2021 left parotidectomy showing myoepithelial carcinoma ex-pleomorphic adenoma.  Is this coded to myoepithelial carcinoma (8982/3) or carcinoma ex-pleomorphic adenoma (8941/3)?  It is unclear how to arrive at the correct histology code using the current Solid Tumor Rules.

The 2022 SEER Manual indicates the p16 status (positive or negative) can be used to code more the specific histology for squamous cell carcinoma, human papilloma virus (HPV) positive (8085) and squamous cell carcinoma, HPV negative (8086). However, the histology coding instructions in the Other Sites schema have not been updated and the 2022 SEER Manual does not cover all situations commonly encountered in the registry. Does the clarification regarding p16 apply to these other situations?

1. How is histology coded when the final diagnosis of the most representative specimen is adenocarcinoma (NOS), but the immunohistochemistry is p16 negative? Is this adequate to code histology to 8484/3 (adenocarcinoma, HPV-independent, NOS)? The pathologist did not specifically indicate this was HPV-independent adenocarcinoma, and the clarification in the 2022 SEER Manual does not include this more specific adenocarcinoma histology.
2. How is histology coded when the Pap smear is positive for squamous cell carcinoma, p16 positive, but the most representative specimen from the primary tumor (the subsequent cervix biopsy) is only stated to be squamous cell carcinoma (NOS)? The p16 studies were not repeated on the most representative specimen, and the existing 2007 Multiple Primaries/Histology (MP/H) General Rules indicate to code the histology from the most representative specimen over a cytology report. Following the existing 2007 MPH General Rules, the histology should be 8070 (squamous cell carcinoma, NOS). However, this does not account for the p16 status of the tumor.
3. How is histology coded when a biopsy of a metastasis (e.g., a lymph node metastasis) proved squamous cell carcinoma, p16 negative, but a subsequent biopsy of the primary cervix tumor proved squamous cell carcinoma (NOS) without additional IHC studies? Again, the 2007 MP/H General Rules confirm the primary site specimen should be used to code the histology, resulting in a diagnosis of 8070 (squamous cell carcinoma, NOS), but this ignores the p16 status of the tumor.