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The partial nephrectomy final diagnosis is renal cell neoplasm. The College of American Pathologists (CAP) Summary lists histology as: multilocular clear cell neoplasm of low malignant potential. The diagnosis comment adds: This neoplasm currently termed multilocular clear cell renal cell neoplasm of low malignant potential (WHO 2016), was previously termed cystic renal cell carcinoma.

The patient had a mediastinal mass biopsy showing fibrosclerotic tissue with patchy lymphohistiocytic foci and scattered plasma cells, followed by a retroperitoneal mass biopsy showing fibrohistiocytic infiltrate. Erdheim-Chester disease is not reportable per the Heme Database. However, the physician specifically states this is a malignant disorder.

The article I read (https://www.ncbi.nlm.nih.gov/pubmed/?term=28984674) makes the distinction between mesophrenic adenocarcinoma and mesophrenic-like adenocarcinoma. The authors propose the term mesonephric-like Mullerian adenocarcinoma. So would this be coded as Mullerian adenocarcinoma?

I have searched ICD-O-3 for a histology listing but could not locate. I also searched the SEER Inquiry database for possible answers, but none were found. The patient underwent a pediatric MRI of the brain of which final impression was: 1) Subependymoma nodules, cortical tubers, and SEGAs are seen bilaterally consistent with tuberous sclerosis.

Instruction number 1 under the Coding Multiple Histologies instructions states to code histology when the histology is described as subtype, type or variant. The general rules do indicate we can code the histology identified as type, but when applying the H Rules, it seems an argument could be made for either H1 or H3. H1 applies if you ignore the diagnosis of carcinoma and only code the histologic type: urothelial carcinoma. However, the rules do seem to imply that you take all histologies into account (e.g., code the subtype/variant when there is a not otherwise specified (NOS) and single subtype/variant). Following this logic, Rule H3 seems to be the only rule that fits, and one would code the subtype/variant urothelial carcinoma when the diagnosis is carcinoma NOS, histologic type: urothelial carcinoma.

The problem is that urothelial carcinoma is not a subtype/variant of carcinoma (NOS) per Table 2. The entry for Carcinoma NOS in Table 2 states, Subtypes of carcinoma NOS include adenocarcinoma and all subtypes/variants of adenocarcinoma. To some, urothelial carcinoma is a more specific type of carcinoma; however, urothelial carcinoma is not also listed as a subtype of carcinoma or of adenocarcinoma; only adenocarcinoma is categorized as a subtype of carcinoma. Consistently applying the rules becomes an issue when rules are interpreted in different ways. Should this Table be amended to include urothelial carcinoma as a subtype/variant of carcinoma NOS with the same caveat given to adenocarcinoma in Table 2?

Immunostain results of the malignant cells show strong staining with p63 and negative staining with TTF-1 and Napsin. Rare cells stain with CK7. Findings are most compatible with squamous cell carcinoma. The patient is treated as if he has squamous cell carcinoma. The new histology coding rules say you cannot use ambiguous terms which modify the histology to code the histology. So is this 8010/3?