SEER Inquiry System - Search

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.

This patient was diagnosed in 2016 with renal cell clear cell and was coded to that. In 2018, the patient's liver lesion was resected and pathology revealed epithelioid angiomyolipoma perivascular epithelioid cell tumor (PEComa) (8714/3), a new term as of 2018. This was compared to the kidney slides and it was determined to be metastatic PEComa from the kidney. The physician's note states: The patient had a nephrectomy for a kidney tumor in 2016, excision of cutaneous melanomas, and resection of liver mass in 2018. These three cases were sent in consultation. The diagnosis of cutaneous melanoma was confirmed by a dermatopathologist of our department, (a separate report had been already issued). The kidney tumor is poorly differentiated composed of sheets of discohesive cells with markedly pleomorphic cells with frequent giant and bizarre cells. Most of the cells have abundant eosinophilic to clear cytoplasm. The nuclei are enlarged and pleomorphic. Multinucleated cells are numerous. Some cells have markedly enlarged nucleoli. Multifocal tumor necrosis is noted. Extensive lymphovascular invasion is observed. There are foci at the periphery of the tumor consisting of a proliferation of spindle cells with entrapped adipocytes consistent with minor element of unusual angiomyolipoma (see block A18). The liver tumor has histologic features that are similar to the poorly differentiated component of the kidney tumor.

The hospital registrar reports that the case was identified at the hospital because of the referral for chemotherapy for the metastatic GIST. The records from the out-of-state hospital mentioned a history of a small bowel resection in 2003 for a borderline tumor. The registrar went back through the hospital's old records and found the surgery was done for GIST of low malignant potential at her facility. The question is whether to report the case or not, and if reported, is 2003 the diagnosis date.

The rules say to change the behavior and backdate the diagnosiswhen a tumor is presumed benign and islater diagnosed as malignant. Another problem for this case is that the out-of-state hospital did not review the slides from the 2003 surgery.

10/27/2017 partial nephrectomy final path diagnosis: renal oncocytic neoplasm, favor hybrid oncocytic tumor. Comment:

2015 Rt kidney nephrectomy pathology states: congenital cellular mesoblastic nephroma, tumor sz 5.9cm, tumor limited to kidney, extension into pelvicalyceal system, margin not applicable, LVI negative. Per PubMed.gov: (In newborns) among the low-grade malignant tumors, congenital mesoblastic nephromas can be successfully treated with simple nephrectomy. Per ScienceDirect: ...currently thought that cellular mesoblastic nephroma is actually a renal variant of infantile fibrosarcoma.

From a number of definitions reviewed, it seems unclear if it's part of the appendix or the cecum of the colon. For example: The cecum is usually located in the right iliac fossa. In the pole of the cecum, there is often the appearance of fusion of the three teniae coli around the appendix, giving rise to the tri-radiate fold (Mercedes Benz sign), but the anatomy can be variable. The most reliable landmarks of the cecum are the appendiceal orifice and ileocecal valve. The appendiceal orifice is usually an unimpressive slit, often crescentic in shape. The ileocecal valve is made up of the superior and inferior lips (usually not seen en face) and is the gateway leading into the terminal ileum. It is located on the prominent ileocecal fold encircling the cecum, between 3 and 5 cm distal to the cecal pole. (https://www.sciencedirect.com/science/article/pii/S2212097113701730)

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?

This will be a tough one for registrars to recognize as non-reportable since the terminology contains sarcoma, so we just want to double check.

Glioblastoma multiforme is listed as a synonym for the preferred term glioblastoma, NOS (9440) per Table 3 Column 2. Therefore, it seems reasonable to assume that a diagnosis of glioblastoma, NOS would be a new primary if it followed a glial or astrocytic tumor. However, in general, the Solid Tumor Rules use the preferred terminology and/or indicate when a specific rule also includes any tumor diagnosed as a subtype/variant. Rule M6 does not explicitly include a diagnosis of glioblastoma, NOS or any of its subtypes/variants (e.g., glioblastoma IDH-mutant or gliosarcoma). Does Rule M6 apply to any diagnosis of glioblastoma, NOS and any of its synonyms or subtypes/variants?