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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.

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.

In the old 2007 Multiple Primaries/Histology rules, Lung Equivalent Terms and Definitions section, oat cell carcinoma (8042) was listed as one of the obsolete terms that was no longer recognized for small cell carcinoma.  That note is not in the current 2018 Solid Tumor Manual lung chapter, and ICDO-3.2 lists oat cell carcinoma as the preferred term for code 8042/3.  Would rule H4, Note 2 apply -- only one histology present, if not listed in Table 3 use ICD-O and all updates, to code oat cell carcinoma as 8042/3?

Penile mass excision shows final diagnosis of squamous cell carcinoma, verrucous type. Subsequent partial penectomy has a final diagnosis of squamous cell carcinoma, verrucous type and the summary cancer data lists

Both the final diagnosis and summary cancer data indicate a histology code of 8051/3 (squamous cell carcinoma, verrucous type / verrucous carcinoma). However, this site and histology combination triggers edit IFN4911. Edit documentation indicates that for sites C600-C609 (all penile sites) use histology code 8051 and do not use 8054.

Review of the 2018 ICD-O-3 Histology Updates table does not indicate these terms are synonymous.

March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation.

Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement?

Example: Left thyroid lobectomy with final diagnosis

When the only mention of encapsulation is included in the tumor characteristics of the College of American Pathologists (CAP) summary, not the pathologist's choice of histologic type, what is the preferred histology?

There is an ICD-O histology code for papillary carcinoma, tall cell (8344/3) as well as papillary carcinoma, oxyphilic cell (8342/3). Per SINQ 20150045, the term oncocytic is synonymous with oxyphilic in this context.

The term “variant” can be used for the Other Sites (non-updated STR sites) primaries when the ICD-O-3.2 (or ICD-O-3 for older cases) includes the term “variant” in the histology name. The MPH General Instructions did not include the term “variant” as a term that can be used to code histology.