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In the 2018 ICD-O-3 Update Table, undifferentiated high-grade pleomorphic sarcoma and undifferentiated high-grade pleomorphic sarcoma of bone (C40_) were both listed as a New Term for histology 8830/3. There was no site restriction for a diagnosis of undifferentiated high-grade pleomorphic sarcoma. Therefore, it appears the diagnosis could easily be applied to a soft tissue tumor. This histology is used by pathologists in our region for soft tissue tumors as well as bone tumors. However, in the ICD-O-3.2 Table an entry (or synonym) was not provided for a tumor outside the bone.

The ICD-O-3.2 Table only lists undifferentiated high-grade pleomorphic sarcoma of bone for site codes C40_ and C41_ as a synonym for histology 8830/3. This also is not listed in the ICD-O-3.2 Implementation Guidelines. As a result, it is unclear whether a diagnosis of undifferentiated high-grade pleomorphic sarcoma of the soft tissue can be coded to 8830/3 and/or can be a synonym for the preferred term (8830/3, Malignant fibrous histiocytoma). Can a diagnosis of undifferentiated high-grade pleomorphic sarcoma of the soft tissue be coded to 8830/3, C49_ as it was per the 2018 ICD-O-3 Update Table?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Examples:

1) New tumor at the ileocolic anastomosis, described as a, Circumferential centrally ulcerated mass with raised borders. Tumor extension: Tumor invades through muscularis propria into subserosal adipose tissue, no involvement of the serosal surface identified. The only mention of mucosa on the resection is the uninvolved enteric mucosa or uninvolved colonic mucosa in the otherwise uninvolved portions of the ileum/colon. If neither bullet 1 or 2 apply, is this a new primary per M7 bullet 3?

2) Right colon with anastomosis site. Tumor site: Anastomosis. Tumor extension: Tumor invades through the muscularis propria. Gross description does not describe mucosa, only noting, at the central area of anastomosis is an ill-defined, slightly raised, tan-brown to purple mass measuring 2.2 x 2 cm, which is nearly circumferential, causing obstruction at the site of anastomosis. If neither bullet 1 or 2 apply, is this a new primary per M7 bullet 3?

3) Polyp at ileocolonic anastomosis, polyp biopsy final diagnosis was, Invasive moderately differentiated colonic adenocarcinoma in association with adenoma. No mention of mucosa on the biopsy final diagnosis or gross description. Clinical info indicates, There is an ulcerated 5 cm mass at the ileo-colonic anastomosis that was biopsied. If neither bullet 1 or 2 apply, is this a new primary per M7 bullet 3?

Example 1: 5/2013 diagnosis of HCC in segment 4B (single tumor), treated with microwave ablation in 7/2013. CT scan in 11/2017 with new 23mm hypodensity in liver segment 4 suspicious for recurrent disease. Clinical assessment in 1/2018: New enlarging lesion in liver most consistent with progression of HCC. Treated with RFA in 2/2018. Is the 2018 occurrence a new primary as imaging stated this was a new lesion?

Example 2: 7/2017 diagnosis of HCC in right liver; 2.5 cm lesion in segment 5/6 with a couple of satellites and 12mm lesion in segment 6, treated with Y90 radioembolization. Follow-up note in 11/2017: complete response of treated cluster of lesions in segment 5/6 and lesion in segment 6, increase in size of caudate lesion not amenable for treatment (this lesion was stated to be indeterminate on 7/2017 imaging). Caudate lesion finally stated as LI-RADS5 on 3/2018 imaging and was treated with chemoembolization 6/2018. 7/2018 and 10/2018 Follow-up imaging states LR-TR nonviable lesion in caudate lobe. 8/2019 CT shows caudate lobe with arterial enhancement, new compared to prior imaging, LR-TR viable. MD note states patient has small local HCC recurrence in segment 1 (caudate lobe) with plan to repeat TACE. Is this 8/2019 HCC a new primary as the patient was disease free for greater than 1 year, or is it the same tumor and a single primary?

In Table 3 (Specific Histologies, NOS/ NST, and Subtypes/Variants), the entry for papillary carcinoma, NOS includes a change in column 3 of the 2018 Breast Solid Tumor Rules that conflicts with the H Rules. It is not accounted for in the change log. No explanation is offered as to why this change was made. This is a major change because encapsulated papillary carcinoma is frequently associated with carcinoma NST, and we have not been collecting these as such.

Encapsulated papillary carcinoma (8504) in column 3 now includes an indented entry, with invasive carcinoma, NST/invasive duct carcinoma 8504/3. However, most encapsulated papillary carcinomas are in situ or there is no definitive statement of invasive encapsulated papillary carcinoma, so when in situ and invasive tumors are present, we are instructed to code the invasive histology (which would be the invasive carcinoma (NST), 8500/3). How are registrars to arrive at the correct histology without a new H rule or a clarification regarding this update being documented in the change log?

Does the same change/addition apply to solid papillary carcinoma? These are often also associated with carcinoma, NST. Again, without an explanation regarding the change mentioned above, it is difficult to understand why the change was made.

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

SINQ 20200022 indicates that cases originally diagnosed as in situ do not have a new primary when a new invasive tumor with a different histology is diagnosed within 5 years. Should histology and/or behavior get updated for the in situ breast primary?

SINQ 20130140 indicates a histologic diagnosis that is characteristic of a specified malignancy is reportable because this is equivalent to the term, diagnostic of. Does the same logic apply to a clinical diagnosis that is strongly characteristic of a malignancy on imaging?

SINQ 20180104 indicates the term, almost certainly, is not a reportable ambiguous term. If a radiologist notes a mass was most certainly malignant, is this adequate to accession this as reportable? Is a clinically certain diagnosis equivalent to diagnostic of? Or are the modifiers almost and most irrelevant because the terms certainly and certain are not on the ambiguous terminology list?

11/18/2019 Left 1st Digit/Thumb Biopsy: Atypical Melanocytic Proliferation consistent with Early Acral Lentiginous Melanoma in situ. Margins Positive. (Not a reportable diagnosis for 2019.)

12/5/2019 Left 1st Digit Shave Biopsies: Malignant Melanoma in situ. Margins Positive.

1/15/2020 Started Aldara (treatment plan: use for ~3 months then Mohs/excision, but due to COVID could not get resection until 7/2020).

7/29/2020 Left Thumb Excision: Residual Melanoma in situ. Margins Positive. Treatment Plan: re-excision.

8/6/2020 Left Thumb Re-Excision: Atypical Lentiginous Melanocytic Proliferation at the 12-2 margin may represent the advancing edge of melanoma in situ. (8/19/2020 Plan to treat the 12-2 margin as positive with in situ; plan for re-excision).

8/20/2020 Left Thumb Re-Excision & Left Nail Plate Excision: Malignant Acral Lentiginous Melanoma with extensive melanoma in situ. Breslow 1.3mm. Margins Positive. Nail plate & bed epithelium with hemorrhage and a mild increase in melanocyte density likely represent melanoma in situ.

9/4/2020 Left thumb partial amputation & Left axillary Sentinel Lymph Node Excision: Residual Malignant Melanoma in situ. 0/3 sentinel nodes positive.

Per Mayo consulting pathologist, the final diagnosis on this right lung biopsy is: SMARCA4-deficient malignant neoplasm (see Comment). Comment: Sections show a poorly-differentiated malignant neoplasm without any apparent glandular, squamous, or stromal differentiation. The tumor near totally replaces the underlying lung tissue without recognizable underlying alveolar parenchyma. Immunohistochemical stains performed at Mayo Clinic (Oscar keratin, INSM1, NUT, S100, desmin and BRG1 protein encoded by SMARCA4 gene) demonstrate that the malignant cells are positive for Oscar keratin (rare cells only), synaptophysin (weak/patchy) and p63 (focal) while negative for the remaining antibodies tested. Of note, SMARCA4 stain is negative in the tumor cells. Thus, this tumor can be categorized as a SMARCA4-deficient malignant neoplasm, which is known to be an aggressive malignancy, likely represent a SMARCA4-deficient thoracic sarcoma, a recently described entity. SMARCA4-deficient carcinomas in the lung have been reported to be mostly adenocarcinomas or squamous cell carcinomas, which would not fit for this case. Please refer to a paper published by our group (Sauter JL et al. Mod Pathol 2017;30:1422-32.

Example: Diagnosis is made on liver core biopsy path showing Metastatic carcinoma, poorly-differentiated, consistent with lung primary. Diagnosis Comment notes: Carcinoma cells are positive for CK7 and TTF-1, negative for CK20.

Subsequent immunohistochemistry report for PD-L1 testing states Liver: Metastatic adenocarcinoma consistent with lung primary. Interpretation: no PD-L1 expression.

IHC/Biomarker testing is often performed to determine treatment type, but it seems like some of the biomarkers for treatment planning are also histology specific. The Solid Tumor Rules do not address the use of biomarkers reports in the histology coding instructions.

Left Eye Conjunctiva, biopsy (01/23/2018): Conjunctival intraepithelial neoplasia moderate to severe. Is intraepithelial neoplasia moderate to severe the same as coding 8077/2?