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5/15/17 Duodenal bulb, biopsy: Fragments of duodenal mucosa with well differentiated neuroendocrine tumor (carcinoid), extending to the edge of specimen and peptic duodenitis in the submitted tissue. No significant intraepithelial lymphocytosis.

Example: Patient had a 2023 nasopharyngeal mass biopsy showing “Nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type.” 

The Head and Neck Solid Tumor Rules (STRs) do not include an H Rule that instructs us how to code histology when there are two subtypes/variants present for a head and neck primary, nor does the STR define undifferentiated carcinoma as a subtype/variant for 8072.

The WHO Classification of Head and Neck Tumors states non-keratinizing nasopharyngeal carcinoma (non-keratinizing squamous cell carcinoma (SCC) is the most common subtype for nasopharyngeal ca, but that non-keratinizing can be subdivided into undifferentiated and differentiated subtypes.

Should histology be 8020 (undifferentiated carcinoma) or 8072 (non-keratinizing SCC)?

The patient has a history of ganglioglioma, WHO grade I, involving the deep right parietal lobe diagnosed on resection in 07/2012. Tumor recurrence in 2017 was treated with radiation.

The patient then had right temporal tumor biopsy and resection 06/2021 with final diagnosis of anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III. Pathologist notes that the tumor demonstrates a ganglioglioma with frequent mitoses and possible vascular proliferation. Subsequent consult findings support an anaplastic glioneuronal tumor, compatible with progression of the patient's ganglioglioma that is post-irradiation. However, the pleomorphic and epithelioid areas are also reminiscent of pleomorphic xanthoastrocytoma, which may occur in combination with ganglion cell components. There is no related SINQ to code this histology.

The Solid Tumor Rules indicate p16 is a valid test to determine HPV status and can be used to code HPV-associated/-independent.

In this case, we do not know whether the HR-HPV test was done on cytologically malignant cells, or on benign cervical cells. It may be impossible to tell unless 100% of the cytology specimen is malignant, but we will not have access to that information. Also, HR-HPV testing is routine on Pap smears, so this testing does not mean the tumor cells specifically harbor HPV.

The WHO Classification of Hematopoietic Tumors (Blue Book), 5th edition states: “Rare cases of classic follicular lymphoma with cytological features of follicular lymphoma (FL) grade 3A can present with a prominent diffuse pattern. In the previous edition, such cases were defined as diffuse large B-cell lymphoma (DLBCL). Currently, it is uncertain whether such cases should be classified as FL or diffuse large B-cell lymphoma; and in such cases, individual treatment choices should be made in multidisciplinary conference settings taking into consideration clinical, laboratory, and imaging parameters. The presence of diffuse areas composed entirely or predominantly of large cells, however, warrants a diagnosis of diffuse large B-cell lymphoma.”

Our concern is that the Hematopoietic (Heme) Manual and Database do not provide instruction for coding this scenario. We hesitate to interpret the terms “equivalent to” as ambiguous because one could argue it is unambiguous. Barring this argument, the M and H rules would indicate this is a diagnosis of diffuse large B-cell lymphoma. However, the physician does not seem to agree with the pathologist.

In 11/2006 the patient had surgery for a 6cm mass in the RUQ arising in the falciform ligament. The pathologic final diagnosis was: Perivascular epithelioid cell neoplasm (PEComa) of uncertain malignant potential. In 10/2009 a liver biopsy showed metastatic perivascular epithelioid cell neoplasm.

Following the existing Solid Tumor Rules Histology Rules, it would seem this is a single primary with histology 8045 (Combined small cell carcinoma) because there is no indication there are multiple prostate tumors and Table 2 states combined adenocarcinoma and small cell carcinoma is Combined small cell carcinoma (8045).

Conversely, while not an exact match to this case, SINQ 20190083 implies small cell carcinoma and adenocarcinoma of the prostate are separate primaries. In that SINQ case, the patient was simultaneously diagnosed with metastatic small cell carcinoma of the prostate on a liver biopsy and prostate adenocarcinoma on a prostate biopsy. There is no indication that patient had separate tumors in the prostate, however the SINQ instructs to code as separate primaries.

Would the previous SINQ logic apply to synchronous diagnoses in the prostate as well? Or does code 8045 apply to this situation?

The ICD-O-3.2 indicates histology 8051 only applies to diagnoses of condylomatous carcinoma and warty carcinoma made prior to 2018. For penis cases diagnosed 2018 and later, these neoplasms should be coded as 8054. This is consistent with SINQ 20200003.

However, a new Table was added to the Other Sites schema in the 2024 Solid Tumor Rules update. Table 23 lists “Verrucous carcinoma / carcinoma cuniculatum / Warty carcinoma” as histology 8051. While verrucous carcinoma is still listed under histology 8051 in the ICD-O-3.2, warty carcinoma is not.

Does Table 23 need to be updated? Or is this an error in both the ICD-O-3.2 and SINQ 20200003?