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Report Produced: 02/04/2023 16:50 PM

Report Question ID Question Discussion Answer (Descending)

Solid Tumor Rules (2018/2021)/Histology--Breast:  How is histology coded for a diagnosis of invasive mammary neuroendocrine tumor (NET), grade 2/3? See Discussion.

Table 3 (Breast Equivalent Terms and Definitions) lists “Neuroendocrine tumor, well-differentiated” of the breast as histology 8246/3. There is no entry for a grade 2 neuroendocrine tumor of the breast in Table 3.  

The pathologist did not indicate the neuroendocrine tumor was poorly differentiated (or it would otherwise be considered a small cell carcinoma). The pathologist noted “By current WHO criteria, this tumor is characteristic of a mammary neuroendocrine tumor, grade 2.  These invasive tumors are considered to have similar prognostic and predictive features of invasive ductal carcinoma of the same grade and stage.”

Assign code 8249/3, neuroendocrine tumor, grade 2 based on the pathologist statement of mammary neuroendocrine tumor grade 2.  According to WHO Classification of Tumors of the Breast, 5th edition, neuroendocrine tumor (NET) is an invasive tumor characterized by low/intermediate grade.

If the histology term is not listed in the Solid Tumor rules, the instructions state to also check ICD-O and updates. Per ICD-O, NET, grade 2 is coded 8249/3. Breast Table 3 will be updated for 2023.


Histology/Biliary tract--Ampulla of Vater: What is the histology code for Intra ampullary papillary-tubular neoplasm in association with microinvasion? See discussion.

Patient was diagnosed on 01/2020, and primary site on the pathology report is Ampulla of Vater (C241). Synoptic Report states histology as: Intra ampullary papillary-tubular neoplasm in association with microinvasion.

I have reviewed the ICD-O-3 coding table and found histology Intraductal tubulopapillary neoplasm (C25_) code 8503/2. Based on the Matrix principle (Rule F on the ICD-O-3), I will change the behavior to 3 and code as 8503/3. If I look in ICDO-3, Tubulopapillary adenocarcinoma is coded 8263/3.

Assign code 8163/3. Based on the microinvasion, the correct term for this neoplasm is pancreatobiliary-type carcinoma. Unfortunately, WHO did not provide all synonyms or related terms for some of the new ICD-O codes. Pathologists may continue using non-preferred terminology as well.


MP/H Rules/Histology--Bladder: What is the correct histology code for a diagnosis of urothelial plasmacytoma carcinoma of the bladder per pathology report?

Assign code 8120/3, urothelial carcinoma, NOS, to urothelial plasmacytoma carcinoma of the bladder.  The WHO classification describes plasmacytoid variants of urothelial carcinoma. There is no specific ICD-O-3 code for these variants; however, and 8120/3 must be used.


Solid Tumor Rules/Histology--Head & Neck: What is the histology of human papillomavirus (HPV)--associated multiphenotypic carcinoma? See Discussion.

Histologic Type: HPV-associated multiphenotypic carcinoma. Overall, the morphology, immunohistochemistry, and HPV testing results support the diagnosis of an HPV-related multiphenotypic carcinoma. This entity has been described in the sinonasal region, where it behaves more indolently than its other salivary gland carcinoma counterparts (e.g., adenoid cystic carcinoma), with local recurrence but rare metastases.

Assign code 8072/3 for HPV-associated multiphenotypic carcinoma. WHO Classification of Head and Neck Tumors, 4th edition, lists sinonasal tract HPV-related carcinoma with adenoid cystic-like features as a subtype of non-keratinizing squamous cell carcinoma (NKSCC).Use text fields to record the details.


Solid Tumor Rules (2018/2021)/Histology--Head & Neck:  What is the histology code for a uvula (C052) primary with histology of squamous cell carcinoma, conventional (keratinizing) and p16 result is negative? See Discussion.

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?

Assign code 8071/3 for keratinizing squamous cell carcinoma.  Codes 8085 and 8086 are only valid for the Head and Neck sites listed in Table 5 beginning with cases diagnosed 01/01/2022 and forward.


Histology--Lung: Is there a better code for SMARCA4-deficient malignant neoplasms than 8000/3 that could be used especially given its aggressive nature? This term is not included in the Lung Solid Tumor Rules or ICD-O-3.1 and 3.2. See Discussion.

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.

Assign code 8020/3. SMARCA4-deficient malignant neoplasms are newly identified. WHO has not proposed an ICD-O code as of yet. Our pathology experts suggest coding to undifferentiated carcinoma until they are better classified.


Histology--Brain and CNS: What is the histology code of a primary papillary epithelial tumor of the sella (PPETS)?  See Discussion.

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.”

Assign code 8000/0.  This is an emerging histology and not yet recognized by the World Health Organization. Document the details in text fields. It might also be useful to document this SINQ question in text.


Update to current manual/First course treatment--Neoadjuvant treatment: How are the 2021 neoadjuvant therapy fields coded when neoadjuvant therapy and surgery were part of first course plans but treatment was never completed. See Discussion.

Example: Breast case where first course treatment plan is neoadjuvant therapy and surgery after. The patient was hospitalized during neoadjuvant therapy, elected hospice, and later died, so the neoadjuvant therapy was never completed, surgery not done. How are the 2021 neoadjuvant therapy fields coded in this situation as neoadjuvant therapy and surgery were part of first course plans. I coded neoadjuvant therapy to 2 - started but not completed, but there are no codes to properly explain the clinical response and therapy treatment effect as the patient did not complete neoadjuvant therapy. Should I use code 9 for clinical response and treatment effect or should this be left blank for this particular case?

Assign code 8 for Neoadjuvant Therapy--Clinical Response in this case. We will update the SEER manual to allow code 2, in addition to code 1, in Neoadjuvant therapy when Clinical Response is coded 8. We will also add instructions covering a case such as this one.

Assign code 7 for Neoadjuvant Therapy--Treatment Effect and use text fields to record the details. We will add instructions to the manual for this scenario.


Update to Current Manual/Neoadjuvant Treatment: What codes should be used for Neoadjuvant Therapy--Clinical Response and Neoadjuvant Therapy--Treatment Effect when the neoadjuvant therapy is still in progress at the time the case is initially abstracted as with rapid reporting. There is no code for neoadjuvant therapy still in progress and code 9 generates an edit for Neoadjuvant Therapy--Clinical Response.

Assign code 8 for Neoadjuvant Therapy--Clinical Response and assign a code 9 for Neoadjuvant Therapy--Treatment Effect when the treatment is still in progress. Revise these codes after the treatment has been completed.

We will update the manual to include these instructions.

20081095 Race, ethnicity/Spanish surname or origin: If birthplace is Brazil or Portugal, patient's last name is on the Spanish Surname list, and there is no text to further clarify ethnicity, what is the correct Spanish Ethnicity code: 0 or 7? See Discussion. See also SINQ 20081075.

Assign code 7 [Spanish surname only] when the last name is on the Spanish Surname list. This includes cases for which the birthplace is Brazil, Portugal or the Philippines and there is no text to further clarify ethnicity.

The instruction to use code 0 [Non-Spanish/Non-Hispanic] in the SEER manual on page 51 (#2) applies when the only information available is the birthplace or a statement of "Portuguese," "Brazilian" or "Filipino."