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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 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 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 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?

The 10/2023 TURBT final diagnosis is “Urothelial carcinoma with mixed histologic appearances, see synoptic summary below for details.” The synoptic report includes, “Histologic Type Comment: Invasive carcinoma percentages: Micropapillary 60-70%, high grade or poorly differentiated urothelial 20-30%, squamous 10-20%.” The squamous component is stated to be “Urothelial carcinoma with squamous differentiation.”

It appears there are two specific urothelial carcinoma subtypes to consider: Urothelial carcinoma, micropapillary variant (8131/3) and poorly differentiated carcinoma (8020/3). The squamous component would not be considered because there is no specific histology for “squamous differentiation.”

The micropapillary component is the predominant histology (60-70%) in this case, and it does seem like this is important to capture. However, the WHO Blue Book indicates poorly differentiated carcinoma of the bladder has a poor prognosis.

The 11/2023 lower gum tumor is a separate tumor occurring after a disease-free interval, so we know the Head and Neck Multiple Tumors Module applies. However, our staff is having difficulty applying the rules to this particular scenario with consistent results.

Is the 11/2023 SCC a non-reportable recurrence per M12, since M4 is ignored due to patient’s prior 2017 C031 (lower gum) primary, and then M6 is ignored due to patient’s prior 05/2022 C023 primary?

Or is the 11/2023 SCC a new primary per M4, since the last diagnosis was in a site differing at the third character (C03 vs C02)? If M4 does not apply due to patient's previous C03 primary, then does M6 apply since it has been more than 5 years since the previous C03 primary?

The 2007 SEER Manual (page 3) states that cases diagnosed clinically are reportable. Exception 2 states if enough time has passed that it is reasonable to assume the physician has seen the negative pathology report, but the clinician continues to call this a reportable disease, accession the case.

SEER reporting guidelines state that severe dysplasia is not reportable, however, many clinicians regard it to be equivalent to carcinoma in situ.

Example 1: In 09-2007 the pathology report for excisional biopsy of right floor of mouth states the final diagnosis is severe dysplasia. At the time, the case is not accessioned based on non-reportable pathology. Patient is subsequently admitted in 3-09. According to the clinical history the patient was diagnosed with squamous cell carcinoma in 2007 and treated with laser. Is this reportable? If yes, how is behavior to be coded? How is "Ambiguous Terminology at Diagnosis" to be coded?

Example 2: In 2-08, the pathology report for a punch biopsy of a skin lesion states the final diagnosis is atypical melanocytic hyperplasia. In 3-08, patient is admitted for re-excision. The clinical diagnosis states re-excision being done for melanoma in situ.

Reference: SINQ 20061123

The 2011 pathology report: Spine at L3 biopsy: metastatic adenocarcinoma. Per addendum: Prior total abdominal hysterectomy specimen from 2003 was reviewed and showed an ovarian mucinous cystic tumor of borderline malignancy which has a similar morphology to the invasive adenocarcinoma seen on current specimen.

Abdominal tissue and omental biopsy: invasive and non-invasive glandular implants compatible with origin from ovarian mucinous borderline tumor.

The final diagnosis per radiation oncologist was, "recurrent ovarian cancer."

The 2016 WHO Classification of Tumours of the Urinary System appears to be moving away from using Fuhrman grading toward using WHO/ISUP grade. These seem like similar 4 grade staging systems; however, the SEER Manual specifically states to not use the Special Grade System table for WHO/ISUP. We are seeing the WHO/ISUP grade being used on 2016 pathology reports.

Examples of new grading for renal cell carcinomas

Histologic type: Clear cell renal cell carcinoma

Histologic grade (WHO/ISUP 2016): Grade 3 in a background of 2 (of 4).

And

Histologic type: Clear cell renal cell carcinoma

Histologic grade (ISUP): Grade 2.

The 2018 Breast Solid Tumor Rules Equivalent Terms and Definitions - Changes from 2007 Multiple Primaries/Histology Rules states: Mammary carcinoma is a synonym for carcinoma no special type (NST)/duct carcinoma not otherwise specified (NOS) 8500. It will no longer be coded as carcinoma NOS 8010. Should metastatic carcinomas of breast origin be 8500, or is code 8010 (carcinoma NOS) more applicable because histology coding from metastatic sites is not as reliable?