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Pituitary blastoma was not added to Table 3 (Specific Histologies, NOS, and Subtypes/Variants) of the 2018 Malignant CNS and Peripheral Nerves Solid Tumor Rules as part of the December 2020 update. This is a new malignant CNS histology for 2021 and later. Not including this histology in Table 3 results in the registrars being required to check another source to correctly code this histology. If this histology cannot be used for cases diagnosed prior to 2021, should that diagnosis year 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.

Per Mayo consulting pathologist, the final diagnosis on this right lung biopsy is: SMARCA4-deficient malignant neoplasm (see Comment). Comment: Sections show a poorly-differentiated malignant neoplasm without any apparent glandular, squamous, or stromal differentiation. The tumor near totally replaces the underlying lung tissue without recognizable underlying alveolar parenchyma. Immunohistochemical stains performed at Mayo Clinic (Oscar keratin, INSM1, NUT, S100, desmin and BRG1 protein encoded by SMARCA4 gene) demonstrate that the malignant cells are positive for Oscar keratin (rare cells only), synaptophysin (weak/patchy) and p63 (focal) while negative for the remaining antibodies tested. Of note, SMARCA4 stain is negative in the tumor cells. Thus, this tumor can be categorized as a SMARCA4-deficient malignant neoplasm, which is known to be an aggressive malignancy, likely represent a SMARCA4-deficient thoracic sarcoma, a recently described entity. SMARCA4-deficient carcinomas in the lung have been reported to be mostly adenocarcinomas or squamous cell carcinomas, which would not fit for this case. Please refer to a paper published by our group (Sauter JL et al. Mod Pathol 2017;30:1422-32.

We received an edit error in SEER*DMS on the following site/histology (Orbit, NOS (C696)/9699/3) that involved an incorrect staging code being assigned to SS2018. The staging language is identical in AJCC, EOD and SS2018. SEER*RSA notes that SS2018 should be coded distant, but in the SS2018 manual, this language is noted Regional. Staging language is: Orbital adnexal lymphoma AND extraorbital lymphoma extending beyond the orbit to adjacent structures--Bone, Brain, Maxillofacial sinuses

Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification.

Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable.

Insulinoma, NOS is reportable for cases diagnosed 2021 and later. However, the diagnosis of insulinoma is most frequently made with clinical correlation of the patient's clinical syndrome and serum hormone levels. Despite a pathological diagnosis of NET, this will clinically be stated as insulinoma based on the functional type of tumor. At the largest facility in our area, all pathology reports with a diagnosis of insulinoma over the last year only provide a pathological Final Diagnosis of NET (either G1 or G2), but elsewhere specify, Functional Type: Pancreatic neuroendocrine tumor, functional. Correlation with Clinical Syndrome and Elevated Serum Levels of Hormone Product: Insulin-producing (Insulinoma).

For 2021 and later, it seems this should be accessioned as insulinoma (8151/3), but one cannot arrive at that histology using the current Other Sites (MP/H) H Rules. Following the existing rules, one would code the histology to NET, G1 or NET, G2 (8240 or 8249) per Rule H6. There are technically two specific histologies to consider: NET (either 8240 or 8249) and insulinoma, NOS (8151). Following the H Rules, Rule H6 instructs one to code the histology with the numerically higher ICD-O-3 code (8240 or 8249).

Coding this histology to NET (8240 or 8249) does not seem to reflect the most accurate classification of this tumor, but applying the current rules, this is the only histology that can be coded. There is no current guideline in the Other Sites schema or the ICD-O-3.2 Implementation Guidelines instructing us to ignore the pathological diagnosis of a NET for these tumors (even though insulinomas are NETs). The only SINQ that currently exists (SINQ 20150019) states the histology can be coded as either a NET or an insulinoma in these cases. How are registrars to consistently code histology for these tumors without a rule clarification?

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

Recognizing the addition of the behavior requirement into this rule is an attempt to stop non-synchronous ductal carcinoma and lobular carcinomas from being accessioned as a single primary (SINQ 20200022), the issue with using behavior rather than timing is that now, synchronous separate/non-contiguous tumors that are invasive carcinoma NST (8500/3) and lobular carcinoma in situ (8520/2) (or vice versa) are separate primaries per M14.

Lobular and carcinoma, NST are separate rows in Table 3, so we cannot stop at M10 and code the mixed histology because there are two separate histologies with different behaviors. There is no rule that states we can just ignore the in situ tumors for the purpose of applying the M Rules. (We are instructed to ignore the in situ when coding histology only in certain circumstances.) The problem with Rule M10 appears to be related to timing.

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

The patient was diagnosed with an EBV-positive plasmablastic lymphoma involving the left testis on radical orchiectomy in April 2019.

In September 2019, a plasmacytoma was found on a right mandibular mass biopsy. Imaging at that time revealed diffuse disease involving the thoracic spine and sinus involvement. The patient then underwent a resection of the T8 spinal/epidural tumor that also proved plasmacytoma.

Subsequently, the right mandibular mass and testis slides were reviewed (at an outside facility) and both were stated to be, The T8/epidural tumor pathology was not reviewed, so it is unclear if this is also assumed to be the same disease process as the right mandibular mass or still a separate, solitary plasmacytoma.

Additionally, some chart notes indicate the patient has plasmablastic lymphoma with a secondary diagnosis of plasmacytoma, while other chart notes state this is stage IV plasmablastic lymphoma involving all documented sites. Although the plasmablastic lymphoma and at least the plasmacytoma of T8 have different ICD-O-3 histology codes, the physicians do seem to be treating this as a single disease process.

Per the site validation list: https://seer.cancer.gov/icd-o-3/sitetype.icdo3.20190618.pdf#search=site%20validation, ill-defined sites (ILL-DEFINED C760-C768) does not include 8070- Squamous cell carcinoma as a valid histology. Therefore when a Cervical Lymph Node and Unknown Primary Tumor of the Head and Neck is submitted with a C760 and 8070/3, it requires an override be set.