SEER Inquiry System - Search

Procedure: Wedge, resection specimen, Laterality: Right, Tumor site: Right upper lobe, Tumor size: 1.0 cm in greatest dimension, Histologic type: Adenocarcinoma in-situ, mucinous, Histologic grade: N/A, Visceral pleura invasion: Not identified, Tumor extension: N/A, Margins: Uninvolved, Lymphocytosis.

The patient has one tumor each in the left parietal and left medial temporal lobe. The tumors were excised. The final diagnosis for the left parietal tumor is glioblastoma, IDH-wild type. he final diagnosis of the left medial temporal tumor is, at least anaplastic astrocytoma, WHO grade III; see comment. The comment states: There is a single focus of vascular hyperplasia, separate from neoplastic cells. No necrosis is identified. These findings on their own would warrant a diagnosis of anaplastic astrocytoma, WHO grade III. However, in the context of the patient's glioblastoma in the left parietal lobe, and imaging showing ring-enhancing lesions of the parietal and temporal lobes, this specimen is favored to be an un-sampled glioblastoma, WHO grade IV. The Solid Tumor Rules indicate we may no longer use terms like favor(s) to code the histology, leaving the final diagnosis as the priority source for coding histology per the Histology coding rules.

The tumor board review confirmed that, despite the anaplastic astrocytoma on pathology, they felt strongly that this is a multifocal glioblastoma and not an anaplastic astrocytoma. Both the pathologist's comment and the tumor board's assessment indicate this patient does not have two primaries. However, the Solid Tumor Rules do not give priority to the tumor board's assessment over the pathology, and registrars are not to use ambiguous terms to code histology thus leaving the two histologies to consider. Per the Solid Tumor Rules, one tumor that is glioblastoma and one tumor that is anaplastic astrocytoma are multiple primaries per M11 (Abstract multiple primaries when separate, non-contiguous tumors are on different rows in Table 3 in the Equivalent Terms and Definitions. Timing is irrelevant).

As a central registry, we cannot ask the pathologist or attending physician for clarification as suggested in Section 3 of the Malignant CNS and Peripheral Nerves Equivalent Terms and Definitions. We can only follow the current Solid Tumor Rules. In doing so, we would have to ignore both the pathologist's and tumor board's assessment that this patient has multifocal glioblastoma. Is there any concern that this will lead to over-reporting?

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?

According to this article, PRRT treatment lutetium Lu 177 dotatate was approved earlier this year by the United States Food and Drug Administration for adult use. PRRT is a nuclear medicine therapy that travels throughout the body looking for a certain receptor within neuroendocrine tumors. These include pancreatic and small neuroendocrine tumors in the gastrointestinal tract. Once absorbed into the tumor, the radioactive material starts to break down tumor cells, killing them. It is the first radioactive drug approved for the targeted treatment of gastroenteropancreatic neuroendocrine tumors.

(http://cinj.org/rutgers-cancer-institute-new-jersey-and-robert-wood-johnson-university-hospital-are-first-state ).

A recent molecular study of PNET tumors at NCI (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5139621) seems to indicate the discovery of four new CNS tumor entities, of which HGNET-BCOR is one. The article suggests that these are not primitive neuroectodermal tumors tumors (PNET), but something different.