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History provided by the oncologist

Right neck mass since 2019; 04/07/20, initial biopsy p16-negative SCC, delay of treatment due to patient preference, agreed to biopsy of tonsil and work-up August 2022; right tonsil biopsy: p16-positive, G2 SCC, nodal mass at that time >6 cm with extensive extranodal extension, Stage III (cT2, cN3, cM0, p16-positive); based on this history, was staged as a tonsil primary and p16-positive.

Patient details

1. March 2020, CT neck and chest revealed a 0.5 x 2.7 x 2.3 cm low-density necrotic nodal mass at right neck level 2 suspicious for metastatic disease.  There was a slight asymmetric increased size of the right palatine tonsil.  There are a few sub-4 mm pulmonary nodules which are nonspecific.

2.  April 7, 2020, FNA of right neck mass with pathology revealed p16-negative SCC   

3.  April 20, 2020, PET/CT revealed 3 x 2 cm right-sided level 2 node with FDG avidity  

4.  May 5, 2020, flexible laryngoscopy showed no obvious primary lesion   

5.  May 2020, after evaluation by a medical oncology, patient declined any treatment  

6.  June 17, 2022, return visit in medical oncology after PET/CT demonstrates significant progression in the neck; patient definitively declines chemo, but would like surgical opinion.  Now has more rapidly progressive disease with skin breakdown and weeping from malignant lesion right neck.  

7.  June 22, 2022, radiation oncology consultation   

8.  July 15, 2022, tonsil biopsy: Invasive squamous cell carcinoma, moderately differentiated with LVI, p16-positive  

9.  Patient now agreeing to treatment with radiation: Tooth extractions 8/30/2022, radiation planning 9/14/2022  

10.  Patient consulted with cancer specialist who explained surgery is not recommended given level of extranodal extension and risk of seventh cranial nerve paralysis and fistula formation with surgical excision and who recommended chemoradiation

11.  September 9, 2022, patient presented for radiation CT simulation/treatment planning and informs treatment team. Patient declined/refuses concurrent chemotherapy despite recommendations from two cancer institutions.

There is an ICD-O histology code for follicular carcinoma, minimally invasive (8335/3) as well as follicular carcinoma, oxyphilic cell (8290/3). Per SINQ 20150045, the term oncocytic is synonymous with oxyphilic in this context.

The Multiple Primaries/Histology General Instructions and histology rules do not include the term “variant” as a term that can be used to code a further histologic subtype. The term “variant” can be used for the Other Sites (non-updated STR sites) when the ICD-O-3.2 (or ICD-O-3 for older cases) provides the term “variant” in the histology name.

The left thyroid lobectomy/isthmusectomy returned a diagnosis of sclerosing mucoepidermoid carcinoma with eosinophina, 6.5 cm, replacing nearly the entire left lobe of the thyroid.

The patient has a completion thyroidectomy of the right lobe and returned the diagnosis of papillary thyroid carcinoma, 0.5 mm, in maximum dimension.

The endocrinologist describes it as "co-exsisting" and states the tumor is iodine non-avid.

2016 Total thyroidectomy, Multifocal

-Dominant Tumor:  Right Lobe, Papillary thyroid carcinoma (8260/3)

-Tumors two through five:  Three tumors Papillary thyroid carcinoma (8260/3), and one tumor Papillary thyroid carcinoma, follicular variant (8340/3)

-An additional tumor: Non-invasive follicular thyroid neoplasm with papillary-like nuclear features (8343/2)

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 had a partial nephrectomy with final diagnosis of papillary renal neoplasm with reverse polarity.  Diagnosis comment states: Papillary renal neoplasm with reverse polarity is currently considered to be a histologic variant of papillary renal cell carcinoma; however, recent studies suggest that it has a very indolent clinical behavior.

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.

The Schema ID for C051 (soft palate, NOS) and C052 (uvula) is Oropharynx (either 00100 or 00111 depending on p16). The Solid Tumor Rules Manual includes these site codes are under Table 4: Tumors of Oral Cavity and Mobile Tongue site group for histology coding. We are aware of the notes that allow coding of 8086 for keratinizing SCC, HPV-negative for sites listed in Table 5 only. However, it seems like C051 and C052 were incorrectly omitted from Table 5 (mis-categorized under Table 4). Can we code 8085 for 8086 for C051 or C052 based on p16/HPV status?

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?

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.