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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.

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.

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?

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.

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.

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?

Details

4/15/2018:  Right abdominal wall mass excision: infiltrating sebaceous carcinoma.  Noted to have a history of Muir-Torre/Lynch syndrome.

1/21/2019:  Two left upper back mass excisions and two lower back (laterality not specified) mass excisions: infiltrating sebaceous carcinomas

8/7/2019:  Excision of multiple sebaceous carcinomas from the right posterior back, left posterior thigh, left anterior abdominal wall, left anterior thigh, right scrotum, right lower abdominal fold, all positive for sebaceous carcinoma on pathology report

9/30/2020:  Right gluteal mass, left gluteal mass, back (NOS) excisions: sebaceous carcinomas.  

10/14/2020:  Right back excision: sebaceous carcinoma. Op note:  History of Lynch syndrome with multiple sebaceous carcinomas, recurrent back mass, site of prior mass resection.

10/18/2021:  Right thigh excision: sebaceous carcinoma

Proposed primaries using MP/H Other Sites Rules

#1:       4/15/2018:  C445-1

#2:       1/21/2019:  C445-2, separate from #1 per M8, same as 1/21/19 C445-9 per M18

#3:       8/7/2019:  C445-1, separate from #1 per M10, separate from #2 per M8

#4:       8/7/2019:  C447-2, separate from #1 & #3 per M8, separate from #2 per M12

#5:       8/7/2019:  C632, separate from #1 per M10, separate from #2-#4 per M11

#6:       9/30/2020:  C445-2, separate from #1 & #3 per M8, separate from #2, #4 & #5 per M10

#7:       9/30/2020:  C445-1, separate from #2, #4 & #6 per M8, separate from #1, #3 & #5 per M10; I do not think the back, NOS (C445-9) is a new primary per M18.

#8:       10/18/2021:  C447-1, separate from #2, #4 & #6 per M8, separate from #1, #3, #5 & #7 per M10

Example: The neoadjuvant therapy was started, the patient progressed, the treatment plan was altered, and a new course of systemic therapy was started; surgery was cancelled.

01/25/21 Bile duct brushing: Malignant cells present, adenocarcinoma

01/26/21 Surgical oncology consult: Currently unresectable; recommend neoadjuvant chemo

02/22/21-3/29/21 Neoadjuvant Gemzar & Abraxane, two cycles, discontinued due to disease progression

04/17/21 Surgical oncology re-eval: CT positive for disease progression, need to change Rx

04/26/21 Second change of treatment due to progression: Irinotecan, Oxaliplatin, and 5FU

07/16/21 Surgical oncology re-eval: Unresectable, advise 4-6 months of chemo followed by radiation