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The 2021 ICD-O-3.2 Update includes papillary microcarcinoma (8341/3) as the preferred term for thyroid primaries (C739). However, there are multiple SINQ entries instructing registrars not to use code 8341/3 for diagnoses of micropapillary carcinoma of the thyroid (including SINQ 20071076, 20081127, 20110027, 20150023, and 20180008).

SINQ 20150023 specifically indicates: Per the WHO Tumors of Endocrine Organs, for thyroid primaries/cancer only, the term micropapillary does not refer to a specific histologic type. It means that the papillary portion of the tumor is minimal or occult (1 cm or less in diameter) and was found incidentally. WHO does not recognize the code 8341 and classifies papillary microcarcinoma of the thyroid as a variant of papillary thyroid carcinoma and codes histology to 8260. If the primary is thyroid and the pathology states papillary microcarcinoma or micropapillary carcinoma, code 8260 is correct.

Does this clarification apply to cases diagnosed 2021 and later? If WHO feels the term micropapillary still does not refer to a specific histologic type for the thyroid, why is 8341/3 listed as a preferred term for this morphology/site combination? For cases 2021 and later, should a diagnosis of Incidental papillary thyroid microcarcinoma (3 mm) in left lower pole, be coded as 8341/3 per the ICD-O-3.2, or as 8260/3 per clarification in multiple SINQ entries?

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

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.

Transurethral resection: Microscopic Diagnosis: Bladder, transurethral resection: Low-grade papillary urothelial carcinoma Gross Description: Received in formalin labeled with the patient's name and bladder tumor is a 3.0 x 2.0 1.0 cm aggregate of friable tan tissue biopsies. The specimen is submitted in toto, cassettes

This is all the information there is on this path report. Extent of Disease (EOD) instructions state inferred description of noninvasive: No statement of invasion (microscopic description present) SEER 2018 Appendix C Bladder Coding Guidelines state code behavior 3 if the only surgery performed is a transurethral resection of the bladder (TURB) documenting that depth of invasion cannot be measured because there is no muscle in the specimen OR the pathology report does not mention whether the submucosa is free of tumor or has been invaded by tumor.

12-19-19 Transurethral resection of bladder tumor pathology revealed high-grade papillary urothelial carcinoma with focal glandular and neuroendocrine features; Pathology Overread: High-grade papillary urothelial carcinoma with focal glandular and neuroendocrine differentiation. Carcinoma invades muscularis propria pT2.

Histology 8130

01/20/20 to 07/01/20, completed 6 cycles of gemcitabine/cisplatin.

07/30/20 Robotic radical cystoprostatectomy with bilateral pelvic lymph node dissection, open ileal conduit pathology revealed carcinosarcoma, invading perivesical fat, no lymphovascular invasion, negative margins. ypT3bN0M0 disease; Pathology Overread: Carcinosarcoma arising in association with high-grade papillary urothelial carcinoma.

Histology 8980/3 or is there another histology that should be used?

Pathology report dated 10/17/2019: Final Diagnosis: Fallopian tubes and gonads, right and left, excision: Dysgenetic gonadal tissue with nests and tubules of atypical germ cells suspicious for gonadoblastoma and at least germ cell neoplasia in situ; and segments of fallopian tube (pending expert consultation).

We think this should be a single primary because the Solid Tumor rules do not apply to metastases. However, we are not sure whether or not the instructions outlined for prostate (SINQ 20180088, 20130221), that indicate we are to accession a new metastatic tumor only with a small cell neuroendocrine histology after an adenocarcinoma, also applies to lung primaries.

We are aware of a phenomenon in which lung adenocarcinoma cases treated with Erlotinib can transform to small cell, but do not know whether it impacts the number of reportable primaries.

Bone marrow, right iliac crest (aspirate smear, touch preparation, clot section and core biopsy): Hypercellular marrow (40-50%) with plasma cell neoplasm (see Comment): " No evidence of metastatic carcinoma. " Adequate iron storage.

Comment: CBC data shows normocytic anemia. Flow cytometric analysis of bone marrow detects a kappa restricted plasma cell population that expresses CD138 and CD38. CD56 is positive. CD19 and CD20 are negative. T lymphocytes are immunophenotypically unremarkable. Polyclonal B lymphocytes are detected. Blast gate is not significantly increased. Immunohistochemical stains are performed on the biopsy core and clot section for greater sensitivity and further architectural assessment with adequate controls. CD138 positive plasma cells comprise > 70% of the total cellularity. AE1/AE3 is negative. Taken together, the morphologic and immunophenotypic findings are consistent with a diagnosis of plasma cell neoplasm. Trilineage hematopoietic activity as are seen.

Lung Rule M14 appears to be the first rule that applies to this case and instructs the user to abstract a single primary. However, we were hoping for confirmation that a cancer (NOS) or malignancy (NOS) would not be a distinctly different histology that may qualify for Lung Rule M8. Currently, these histologic terms are not included in the Table 3 options or mentioned in the preceding notes.

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?