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Report Produced: 02/04/2023 17:51 PM

Report Question ID (Ascending) Question Discussion Answer
20220001

Solid Tumor Rules (2022)/Histology--Bladder:  Can the term configuration be used to code the more specific histology for bladder primaries diagnosed 2022 and later? See Discussion.

In the September 2021 Urinary Sites Solid Tumor Rules update, the term configuration was removed from the “DO NOT CODE histology when described as” list. However, it was not added as a term that can be used to code the more specific histology for urinary tumors.

Can configuration be used to code the more specific histology 8130 (papillary urothelial carcinoma) when the diagnosis is urothelial carcinoma, tumor configuration: papillary?

Beginning with cases diagnosed 1/1/2022, the term "configuration" can be used to code histology for urinary sites only. At the request of the AJCC urinary experts, the instructions were changed to allow configuration to be used to code histology.

20220002

Solid Tumor Rules (2018, 2021)/Histology--Cervix:  For cases diagnosed 1/1/2022 and later, how is histology coded for the following three cervix cases relating to p16? See Discussion.

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.

For cases diagnosed beginning 1/1/2022, assign histology based on new codes and terms for the examples of cervical cancer using the available p16 results as follows.

1.  Adenocarcinoma, HPV-independent, NOS (C53._) (8484/3)

2.  Carcinoma, squamous cell, HPV-associated (C53._) (8085/3)

3.  Carcinoma, squamous cell, HPV-independent  (C53._) (8086/3)

The 2022 SEER Manual states: Beginning with cases diagnosed 01/01/2022 forward, p16 test results can be used to code squamous cell carcinoma, HPV positive (8085) and squamous cell carcinoma, HPV negative (8086).  Use the available results as the rules for Other Sites have not been updated yet. The SSDI Manual data item p16 for Cervix schema also states that p16 is based on testing results and not a physician statement.  We can address these situations in a future version of the Solid Tumor Rules. The Other Sites rules will provide document priority when coding hsitology: biopsy vs. resection, cytology vs. histology, primary site vs. mets or regional site. 

20220003

Reportability/Histology--Anus:  Are 2021 diagnoses of anal intraepithelial neoplasia (AIN) II or AIN II-III reportable in patients with a known history of AIN II or AIN II-III diagnosed prior to 2021? See Discussion.

Patient has a history of AIN I/low-grade squamous intraepithelial lesion (LSIL) dating back to at least 2015, was diagnosed with AIN II-III in 12/2019, and then diagnosed again with AIN II-III in 08/2021. There is no indication of treatment or a disease-free interval for this patient.

SINQ 20210015, while not an exact match to this case, implies there is no clear disease-free interval for these AIN diagnoses, so it is the same non-reportable neoplasm diagnosed prior to reportability (12/2019). However, there was a diagnosis of a reportable neoplasm in 2021, so it also seems possible this would be accessioned as a reportable tumor based on a diagnosis of reportable tumor diagnosis in 2021.

With the reportability changes for these intraepithelial neoplasia II/II-III tumors, these situations will arise more frequently.

Report AIN II and AIN II-III cases when initially diagnosed in 2021 or later.  Do not report retrospective cases; that is, cases with diagnoses prior to 2021 with continuation of AIN II or AIN II-III extending into the reportable period. 

20220004

First Course Treatment/Cancer-directed Treatment:  What information can registrars use to determine disease progression and whether treatment counts as first course treatment? See Discussion.

Is a physician’s statement of progressive disease adequate to determine disease progression in coding first vs. second course treatment? Can an increase in tumor burden (i.e., a change in overall stage) be used by the registrar to determine disease progression?

Often, determining disease progression is difficult as there are no guidelines in the SEER Manual related to this topic. It seems a physician’s statement of progressive disease should always be accepted. However, that statement is not always available. While it seems an increase in tumor size alone would not be “progressive disease” as tumors will continue to grow, can registrars use an increase in tumor burden to make this determination?

The instructions for coding first vs. second course treatment are clear when a treatment plan is changed, but determining whether there has been disease progression, recurrence, or treatment failure can be difficult without a physician’s assessment.

For example, a patient was diagnosed with a newly diagnosed resectable pancreatic cancer; the documented treatment plan was for upfront chemotherapy, followed by repeat staging, followed by pancreatectomy. The patient completed 3 cycles of FOLFIRINOX, but the physician noted that the CT scan shows progressive disease, and the plan was to start a new treatment regimen with Abraxane, Gemzar, and stereotactic body radiation (SBRT) (Cyberknife). The patient completed the additional chemotherapy, radiation, and proceeded to the initially planned surgery. The pathologist staged this as yp disease, but the surgery appears to be second course treatment, and we would not code the surgery, or collect the staging (yp staging) since the physician stated this was progressive disease. The classification as yp staging can be misleading, since the resection is technically after neoadjuvant treatment, but is not collected per our guidelines. In this case, is it correct to code first course treatment as FOLFIRINOX only?

Determining first course treatment is based on knowing the treatment plan and its course as to whether it was completed as initially planned. Read the medical record, scans, labs, and physician notes. First course of therapy ends when the treatment plan is completed as planned.  Alternatively, first course of therapy ends when there is documented disease progression, recurrence, or treatment failure.  A change to a drug in a different group or a change to a different treatment modality indicates the end of the first course of treatment.  While a physician/clinician statement of progression, additional imaging, or other procedures that assess treatment efficacy, or increase in tumor burden can be used to denote progression, recurrence, or failure, a change to the initial treatment plan is a signal to to the registrar to suspect the end of first course of therapy.  Once the initial treatment plan is changed, everything after the change is subsequent treatment.

In the scenario provided, code FOLFIRINOX as first course of treatment.  Based on the information provided, the Abraxane, Gemzar, and SBRT are second course and everything that followed that is second or subsequent course. The physician noted progressive disease and a new treatment regimen was started -- this is a clear indication of the end of the first course of treatment. The planned treatment course was FOLFINOX and surgery. Once that initial treatment plan is changed, everything after the change is no longer first course of treatment. Use text fields to document the details.

20220005

Reportability--Ambiguous Terminology:  Can the term “at most” preceding a statement of a reportable diagnosis be used to accession a case? See Discussion.

A January 2022 endometrium biopsy and curettage both show final diagnosis of “mild cytologic atypia and glandular crowding, at most endometrioid intraepithelial neoplasia.”  Any subsequent surgery path is unlikely to provide clarification.

Do not report the case in this scenario based on the diagnosis alone of mild cytologic atypia and glandular crowding, at most endometrioid intraepithelial neoplasia.  "At most" is not an ambiguous term for reportability.  It appears that "at most" in this case refers to the worst possible option within other possible options (differential diagnosis).  Differential diagnoses are "educated guesses" or hypotheses and are usually not reportable unless proven otherwise.  As there is no clear statement of the diagnosis in this case, we recommend that you seek additional information, for example, clinical diagnosis, treatment, and patient care.

20220006

Histology/Brain and CNS:  How is histology coded for a 2021 diagnosis of “neuroepithelial tumor with PATZ1-EWSR1 fusion, not elsewhere classified” found during a right thalamic mass resection? See Discussion.

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.

Assign 8000/1. Neuroepithelial tumor with PATZ1-EWSR1 fusion, not elsewhere classified, is not recognized as a distinct entity at this time. It is not listed in ICD-O-3.2 or in the 5th edition of the WHO CNS classification.

20220007

Histology:  Is there any guidance on using STRATA Oncology testing (molecular tumor profiling tests), such as StrataNGS and StrataEXP, to code SSDIs, histology, etc? I do not see anything in STR, SEER Program Manual, SINQ, or CAnswerForum. We are seeing the testing with our 2021 paths.

We recommend that you do not use information from these molecular tumor profiling tests until they become a standard diagnostic tool. If/when that happens, we will add information to the various manuals.

20220008

Reportability/Histology--Soft Tissue:  Is atypical spindle cell neoplasm, primitive myxoid mesenchymal tumor of infancy (PMMTI) from the soft tissue of the leg in August of 2019, reportable? 

Primitive myxoid mesenchymal tumor of infancy (PMMTI) is reportable. PMMTI is listed in the new WHO 5th edition Classification of Soft Tissue and Bone Tumors under round cell sarcomas. This is a variant of BCOR sarcomas. There is a new ICD-O histology code assigned for cases diagnosed in 2022 or later (9368/3). Code this 2019 case to round cell sarcoma, undifferentiated 8803/3. Use text fields to explain the details.

20220009

First Course Therapy/Reason for No Surgery of Primary Site:  What code should be used for Reason for No Surgery of Primary Site in 2020 in situations affected by the pandemic when abstracting all sites?  See Discussion.

Example:  Patient scheduled for left nephrectomy on 3/10/20 due to left renal papillary renal cell carcinoma diagnosed on 2/11/20 via needle core biopsy. Abstract indicated surgery was cancelled due to the pandemic. Abstract also indicated the surgery was not rescheduled.

There is no available code that fits this situation. We recommend assigning code 6 (Surgery of the primary site was not performed; it was recommended by the patient’s physician, but was not performed as part of the first course of therapy. No reason was noted in patient record.) and documenting the situation in a text field.

20220010

EOD 2018/Heme & Lymphoid Neoplasms--Myeloid Sarcoma:  How is Extent of Disease (EOD) Primary Tumor coded for a myeloid sarcoma with multifocal skin involvement?  See Discussion.

Patient has a diagnosis of myeloid sarcoma presenting as multiple erythematous papules and nodules on back, chest, right arm & shoulder. Oncologist did not mention any evidence or suspicion of an associated AML diagnosis.

HemeRetic schema EOD Primary Tumor Note 1 states that myeloid sarcoma can be coded as localized (code 100) or systemic (code 700). It is not clear what would qualify as systemic disease for myeloid sarcoma.

Assign code 100, localized, using the 2018 EOD Primary Tumor, HemeRetic schema, for the myeloid sarcoma with skin involvement since only the skin is involved.  Use code 700, distant or disseminated, when multiple organs are involved.