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Patient diagnosed with multinodular and vacuolating neuronal tumor of the cerebrum. My research shows: Multinodular and vacuolating neuronal tumor of the cerebrum is a recently reported benign, mixed glial neuronal lesion that is included in the 2016 updated World Health Organization classification of brain neoplasms as a unique cytoarchitectural pattern of gangliocytoma. There is no code in ICD-O-3 for it, so do I report it and use 9492/0 or not ?

There is a group of patients diagnosed within the past few years with ovarian cancers who are now enrolled in a clinical trial and are being screened as potential patients for a particular protocol. The screening for these particular cases is being done by a pathologist who has a particular interest in GYN pathology. As the pathologist is screening the cases, there are some which the pathologist is reclassifying as being fallopian tube primaries rather than ovarian primaries. This is apparently due to newly emerging findings and literature. The problem for me is that these cases have been entered into the registry as ovarian primaries, which was correct as of the time of the initial diagnosis. Should the abstracts remain as they were initially coded, since the diagnosis was ovarian cancer at the time they were diagnosed, or should these cases be updated to reflect the current pathologist's interpretation that these are fallopian tube primaries?

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.

Code 9920/3 is not included under rule M3. However, disease process knowledge would indicate that because the patient has an underlying AML subtype, the leukemia cutis is due to the AML cells that have migrated into the skin tissue. This appears to be a single advanced disease process essentially diagnosed simultaneously.

The 2018 Solid Tumor (ST) Rules General Rules state: For those sites/histologies which have recognized biomarkers, the biomarkers frequently identify the histologic type. Currently there are clinical trials being conducted to determine whether these biomarkers can be used to identify multiple primaries. Follow the Multiple Primary Rules; do not code multiple primaries based on biomarkers.

Additionally, Biomarkers is at the top of the priority order to identify histology in several sections (it appears to be excluded from only Colon, Melanoma and Other sections). In the sections that include this rule, there is not much additional information on using biomarkers. Can you please provide further explanation for prioritizing biomarkers in the histology coding rules? For example, will the ST manual be updated when we need to look for specific biomarkers in a diagnosis?

Pathology--Right breast, lumpectomy with needle localization: Encapsulated papillary carcinoma of the breast. A separate focus of ductal carcinoma in situ is present. Sentinel lymph node, right breast, biopsy: One lymph node, negative for malignancy. No metastatic carcinoma is seen on slides stained with immunostain for cytokeratin (AE1/AE3). Specimen laterality: Right. Tumor size: 1.2 cm. Histologic type: Encapsulated papillary carcinoma. Nuclear grade: Grade 1 (low). Mitotic rate: Score 1. Ductal carcinoma in situ (DCIS): DCIS is present. Estimated size (extent) of DCIS: 3 mm. Architectural patterns: Cribriform and papillary. Nuclear grade: grade 1 (low). Necrosis: Not identified. Margins: Margins uninvolved by encapsulated papillary carcinoma. Distance from closest margin: 8 mm, superior Margins uninvolved by DCIS. Distance from closest margin: 11 mm, superior Lymph nodes: Total number of lymph nodes examined (sentinel and nonsentinel): 1. Number of sentinel lymph nodes examined: 1. Number of lymph nodes with tumor cells: 0. Pathologic staging: Primary tumor: See comment. Regional lymph nodes: pN0(i-). Comment: In the WHO Classification of Tumours of the Breast (2012), it is stated that "there is no universal agreement on how to stage encapsulated papillary carcinomas. In the absence of conventional invasive carcinoma, the consensus of the WHO Working Group was that such lesions should be staged and managed as Tis disease."