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The ICD-O-3.2 Implementation Guidelines and ICD-O-3.2 Coding Table indicate that insulinoma, NOS has changed behavior from /0 to /3 for cases diagnosed 2021 and later. However, the ICD-O-3.2 Implementation Guidelines do not indicate whether this change applies to tumors described as above. Insulinomas are generally neuroendocrine tumors/neoplasms, so it seems any neuroendocrine tumor described as an insulinoma should be collected as 8151/3, but does that apply to an epithelioid tumor/neoplasm also described as insulinoma?

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

Bone marrow, right iliac crest (aspirate smear, touch preparation, clot section and core biopsy): Hypercellular marrow (40-50%) with plasma cell neoplasm (see Comment): " No evidence of metastatic carcinoma. " Adequate iron storage.

Comment: CBC data shows normocytic anemia. Flow cytometric analysis of bone marrow detects a kappa restricted plasma cell population that expresses CD138 and CD38. CD56 is positive. CD19 and CD20 are negative. T lymphocytes are immunophenotypically unremarkable. Polyclonal B lymphocytes are detected. Blast gate is not significantly increased. Immunohistochemical stains are performed on the biopsy core and clot section for greater sensitivity and further architectural assessment with adequate controls. CD138 positive plasma cells comprise > 70% of the total cellularity. AE1/AE3 is negative. Taken together, the morphologic and immunophenotypic findings are consistent with a diagnosis of plasma cell neoplasm. Trilineage hematopoietic activity as are seen.

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.

11/18/2019 Left 1st Digit/Thumb Biopsy: Atypical Melanocytic Proliferation consistent with Early Acral Lentiginous Melanoma in situ. Margins Positive. (Not a reportable diagnosis for 2019.)

12/5/2019 Left 1st Digit Shave Biopsies: Malignant Melanoma in situ. Margins Positive.

1/15/2020 Started Aldara (treatment plan: use for ~3 months then Mohs/excision, but due to COVID could not get resection until 7/2020).

7/29/2020 Left Thumb Excision: Residual Melanoma in situ. Margins Positive. Treatment Plan: re-excision.

8/6/2020 Left Thumb Re-Excision: Atypical Lentiginous Melanocytic Proliferation at the 12-2 margin may represent the advancing edge of melanoma in situ. (8/19/2020 Plan to treat the 12-2 margin as positive with in situ; plan for re-excision).

8/20/2020 Left Thumb Re-Excision & Left Nail Plate Excision: Malignant Acral Lentiginous Melanoma with extensive melanoma in situ. Breslow 1.3mm. Margins Positive. Nail plate & bed epithelium with hemorrhage and a mild increase in melanocyte density likely represent melanoma in situ.

9/4/2020 Left thumb partial amputation & Left axillary Sentinel Lymph Node Excision: Residual Malignant Melanoma in situ. 0/3 sentinel nodes positive.

Example 1: 5/2013 diagnosis of HCC in segment 4B (single tumor), treated with microwave ablation in 7/2013. CT scan in 11/2017 with new 23mm hypodensity in liver segment 4 suspicious for recurrent disease. Clinical assessment in 1/2018: New enlarging lesion in liver most consistent with progression of HCC. Treated with RFA in 2/2018. Is the 2018 occurrence a new primary as imaging stated this was a new lesion?

Example 2: 7/2017 diagnosis of HCC in right liver; 2.5 cm lesion in segment 5/6 with a couple of satellites and 12mm lesion in segment 6, treated with Y90 radioembolization. Follow-up note in 11/2017: complete response of treated cluster of lesions in segment 5/6 and lesion in segment 6, increase in size of caudate lesion not amenable for treatment (this lesion was stated to be indeterminate on 7/2017 imaging). Caudate lesion finally stated as LI-RADS5 on 3/2018 imaging and was treated with chemoembolization 6/2018. 7/2018 and 10/2018 Follow-up imaging states LR-TR nonviable lesion in caudate lobe. 8/2019 CT shows caudate lobe with arterial enhancement, new compared to prior imaging, LR-TR viable. MD note states patient has small local HCC recurrence in segment 1 (caudate lobe) with plan to repeat TACE. Is this 8/2019 HCC a new primary as the patient was disease free for greater than 1 year, or is it the same tumor and a single primary?

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.

The ICD-O-3.2 Coding Table includes hybrid oncocytic chromophobe tumor as a related term for histology code 8317 (Renal cell carcinoma, chromophobe type). However, this related term is not discussed in the implementation guidelines as being a new term/reportable tumor. The Solid Tumor Rules do not indicate a hybrid oncocytic chromophobe tumor is reportable; however, if a registrar only looked at the ICD-O-3.2 Coding Table, it may seem as though this histology should be collected.

The term hybrid oncocytic chromophobe tumor was not included in the Solid Tumor Rules as a subtype/variant of RCC, or as an equivalent term for chromophobe RCC. There is a SINQ (20180047) that states not to report renal hybrid oncocytic tumor, despite the fact these tumors exhibit mixed features of both oncocytoma and chromophobe RCC. For cases diagnosed 2021 and later, should the clarification in the SINQ apply? Or should the ICD-O-3.2 Coding Table be used which indicates this is a reportable diagnosis? If the standard setters decided not to implement use of hybrid oncocytic chromophobe tumor for 2021, can clarification be added to the Solid Tumor Rules or Implementation Guidelines?

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

Example: 3/22/2017-26 year old white female seen in the emergency room with abdominal pain. Patient was diagnosed about a month ago with breast cancer. Impression: menstrual pain. In this example the patient is newly diagnosed with breast cancer, but the second hospital does not treat or diagnose the patient; pain management for a separate condition is received only. Is this patient reported due to the history of active disease?