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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.

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 example:

-ultrasound of the right eye: consistent with a nevoma/melanoma; we could not find any indication that nevoma is a reportable term

-bladder biopsy pathology report:  severe urothelial dysplasia/carcinoma in situ (CIS)

As a central registry, we receive some limited information cases like this where there is no record of treatment or possibility to follow-back to physicians for clarification, so we want to make sure we are reporting them correctly.

11/16/20: Patient diagnosed with endometrial cancer on by MRI of the pelvis; 11.5 cm uterine mass consistent with cancer with no lymphadenopathy.

1/6/21: Patient had a total abdominal hysterectomy/bilateral salpingo-oophorectomy and pelvic lymph node dissection. Operative report stated patient had mildly enlarged bilateral pelvic nodes.

Path report: Endometrioid adenocarcinoma with invasion of the serosa. Five bilateral pelvic nodes were sampled and negative. Originally, staging had patient as node negative.

1/22/21: Patient had post op imaging done that showed metastatic retroperitoneal, aortocaval, and possibly left iliac lymph nodes. Physician changed staging to include the lymph node involvement.

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.

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.

Acinar predominant adenocarcinoma measures at least 12 mm and involves wedge biopsy margins, while the lepidic predominant adenocarcinoma measures 11 mm and does not involve the margins of that separate specimen. Pathologist also notes, “CT findings of diffuse coarse reticular nodular opacity, these findings may represent pneumonic type adenocarcinoma/diffuse pulmonary involvement or intrapulmonary metastasis.  Both of these scenarios have the corresponding stages of pT4 (if thought to be ipsilateral) or M1a (if thought to also involve the contralateral lobe).”

Patient declined any further treatment and transitioned to hospice before expiring less than 1 month after wedge biopsies.

It is unclear if Rule M6 would apply to these two specimens with different subtypes since this scenario is not specifically addressed in the M rule definitions.

The pathology report states this is a rare entity described in case reports and not incorporated into the WHO classification of tumors. A subsequent endocrinology note stated “papillary tumor, benign by path; tumor was not an adenoma; based on one Mayo study, the recurrence risk is low.”

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 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.