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A common scenario is a patient has a positive CT of the abdomen/pelvis for liver mass only. Biopsy of the liver mass is positive for cholangiocarcinoma. The physician is also calling the liver tumor the primary site with histology of cholangiocarcinoma. There is no evidence of intrahepatic bile duct (C221) or gallbladder (C240) involvement which are sites specific to this histology. The hematology/oncology consult stages this as Stage IIIA, T3N0M0 intrahepatic cholangiocarcinoma.  Can we code cholangiocarcinoma with site code C220 (liver) or should we assume that C221 (intrahepatic bile ducts) would be a better code to reflect this histology?  

There is an ICD-O histology code for papillary carcinoma, columnar cell (8344/3) as well as papillary carcinoma, encapsulated (8343/3). Per Rule H13, the terms “with features of” may be used to identify a subtype.

Considering these two subtypes, and knowing there is no specific histology code for this combination, is the first rule that applies H17 (code the numerically higher histology code)?

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 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.

In the WHO Classification of Female Genital Tumors, 5th edition, for the uterine cervix squamous intraepithelial lesions, there is related terminology for high grade squamous intraepithelial lesion HSIL (CIN3) 8077/2 and it is severe squamous dysplasia; squamous cell in situ. However, in the online WHO Classification of Digestive System Tumors, 5th edition, there is no related terminology for esophageal high-grade squamous dysplasia, 8077/2. Can you collect cases of severe dysplasia the same as cases of high grade dysplasia? 

The patient underwent a resection of the transitional meningioma in 2012, but residual tumor was left behind. The patient was on surveillance until imaging showed growth of the residual tumor. The resection in 2022 proved atypical meningioma.

Rule M2, the first rule that applies, indicates this situation represents a single primary (a single tumor). However, Rule M4 states the transformation from a benign meningioma to a borderline meningioma would only be a single primary if the meningioma was a NOS.

This patient has microscopic confirmation of a meningioma showing different subtypes/variants (listed in Column 3, Table 6). Should this be accessioned as multiple primaries based on the transformation and distinctly different histologies?

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.

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.”

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.