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Table 6 (Tumors of the Oropharynx, Base of Tongue, Tonsils, Adenoids) in the Head and Neck Equivalent Terms and Definitions lists both squamous cell carcinoma HPV-positive and squamous cell carcinoma HPV-negative as subtypes/variants of squamous cell carcinoma (the NOS histology, 8070). Squamous cell carcinoma HPV-positive and squamous cell carcinoma HPV-negative are also listed in the 2018 ICD-O-3 update table.

Previous clarification from the standard setters regarding the 2018 ICD-O-3 Update table indicated that histology codes 8085 and 8086 (HPV-positive and HPV-negative squamous cell carcinoma, respectively) included p16+ and p16- squamous cell carcinoma, respectively. Presumably, this clarification was made because p16 is a surrogate marker for HPV, and capturing whether a tumor is HPV-related or not has implications for staging for 2018 and later diagnoses. However, this clarification was not added to the 2018 ICD-O-3 Update table via errata, nor do the Head and Neck Equivalent Terms and Definitions or Histology Coding Rules address this.

Is a diagnosis of p16-positive squamous cell carcinoma equivalent to a diagnosis of squamous cell carcinoma HPV-positive (8085)? If so, will this clarification be added to the Head and Neck Solid Tumor Rules?

Table 4 (Changes in reportable terminology), 2021 ICD-O-3.2 Update, does confirm that the term malignant no longer needs to be used to describe a paraganglioma, but Table 4 includes the histology for extra-adrenal paraganglioma, NOS (8693/3) as the new preferred term for paraganglioma. Paraganglioma, NOS is histology code 8680/3. Which code is correct?

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

Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors.

While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin.

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 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 have similar prognostic and predictive features of invasive ductal carcinoma of the same grade and stage.”

Table 19: Vulva Histologies of the Other Sites Solid Tumor Rules does not include entries for either keratinizing or nonkeratinizing squamous cell carcinoma in the “Squamous cell carcinoma, NOS” row. However, these are two distinctly different histologies per the ICD-O-3.2.

All other Solid Tumor Rules schemas include an M Rule instructing one to abstract multiple primaries when separate/non-contiguous tumors are two or more different subtypes/variants in Column 3 of the Specific Histologies, NOS, and Subtype/Variants Table for the schema (e.g., Rule M6 for Lung). The timing of these tumors is stated to be irrelevant. The Notes confirm the tumors may be subtypes/variants of the same or different NOS histologies and tumors in column 3 are all distinctly different histologies (even if they are in the same row). However, the 2023 Other Sites schema appears to be missing this rule.

Should these distinctly different histologies be accessioned as separate primaries? Is an M Rule missing from the Other Sites schema to address distinctly different histologies?

Table 1 of the 2018 Kidney Solid Tumor Rules (STR) lists succinate dehydrogenase-deficient renal cell carcinoma as histology code 8312, but in the ICD-O-3.2 Coding Table it is listed as histology code 8311.

No changes were made in the Kidney STR. As a result, the histology change described in the ICD-O-3.2 Coding Table conflicts with Table 1. Succinate dehydrogenase-deficient renal cell carcinoma (SDHD) is listed in Table 1 as a synonym for renal cell carcinoma, NOS (8312). However, the ICD-O-3.2 Coding Table lists this as a related term for histology code 8311/3. This related term was not discussed in the Implementation Guidelines, and no change was noted in the STR.

While it seems we should continue to follow the STR, without clarification as to why this histology change was not implemented in STR, achieving consistency will be problematic if registrars jump straight to the ICD-O-3.2 Coding Table to code histology for cases diagnosed 2021 and later. If this code cannot be used for cases diagnosed prior to 2021, should that clarification be included in the STR?

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

Surgery text indicates TAH-BSO with bilateral pelvic and paraaortic lymph node dissection. The pathology report indicates the specimen includes: Uterine corpus, cervix, bilateral ovaries and fallopian tubes, bilateral parametria. The Gross Description also indicates: Representative sections submitted in 16 cassettes as follows: A1: Anterior cervix A2: Posterior cervix A3: Full thickness anterior lower uterine segment A4: Full thickness posterior lower uterine segment A5: Tumor A6-A7: Full thickness anterior endomyometrium to include tumor A8-A10: Full thickness posterior endomyometrium with tumor A11: Representative sections of right fallopian tube and fimbria A12: Representative sections of right ovary A13: Representative sections of left fallopian tube and fimbria A14: Representative sections of left ovary A15: Right parametrial tissue A16: Left parametrial tissue A17-23: Remainder of cervix.

Starting for cases diagnosed in 2024, the SPCSM manual no longer requires the data items for clinical and pathologic tumor size.  Instead, it appears to align with the CoC data item of Tumor Size Summary.  The two manuals contradict each other when it comes to coding tumor size summary for neoadjuvant chemotherapy (NAC) treated cancers.  

STORE states: "If neoadjuvant therapy followed by surgery, do not record the size from the pathologic specimen. Code the largest size of the tumor prior to neoadjuvant treatment; if unknown code size as 999."  

2024 SPCSM states "If neoadjuvant therapy followed by surgery, do not record the size from the pathologic specimen. Code the largest size of the tumor prior to neoadjuvant treatment; if unknown code size as 999." It continues to state     

 12. Assign code 000 when…. (a) no residual tumor is found…(i) Neoadjuvant therapy has been administered and the resection shows no residual tumor &

14. Assign code 999 when...(d) Neoadjuvant therapy has been administered and resection was performed. Do not use a post-neoadjuvant size to code pathologic tumor size; however, you may use the clinical tumor size if available

It seems that we will lose the value of the tumor size summary if we code 000 when NAC is administered and there is no residual disease. 

Example: Patient has a 90 mm triple positive breast tumor and is treated with neoadjuvant TCHP (docetaxel/carboplatin/ trastuzumab/pertuzumab). After completing neoadjuvant therapy, the patient has a mastectomy with no residual disease noted on the final pathology report. 

Using the 2024 SPCSM instructions, code 000 for Tumor Size Summary instead of 090 for the clinical tumor size of 90 mm tumor noted before NAC was administered. This has the potential to affect data analysis, research, and clinical trial accrual.

Spindled cell lesion of smooth muscle origin (desmin and SMA are positive, CD34, S100, pancytokeratin, Pax8, MDM2 and CDK4 are negative). Many of the cells have hyperchromatic, bizarre-shaped nuclei. Mitotic activity is inconspicuous. There are no areas of necrosis. The overall findings in this biopsy is best classified as a "STUMP"; however, a leiomyosarcoma cannot be excluded.

Specimen A is labeled vocal cord biopsy. Specimen B is labeled left true vocal cord nodule. For specimen B the gross portion of the pathology report describes a .5 cm tissue portion. Is the term "nodule" enough information to code this as an excision? Can we code site specific surgery to code 20 or 27?