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Rectal mass biopsy final diagnosis: High grade neuroendocrine tumor (WHO Grade 3). Neither SINQ 20170033 nor 20160023 address coding histology or grade for neuroendocrine tumors that are designated as high grade and/or WHO grade 3.

This terminology is not specifically included in either alternate names list for myelodysplastic syndrome, NOS (9989/3) or refractory anemia with excess blasts (9983/3).

Example: Bone Marrow Biopsy, Final Diagnosis: Consistent with involvement by myelodysplastic syndrome with excess blasts-2 (MDS EB-2).

The clinical documentation clearly identifies residual tumor after the 2011 craniotomy. Scans demonstrated slow enlargement of the tumor over the years, which resulted in a repeat craniotomy. The pathologist noted in the diagnosis comment section of the pathology report that

Is this a single primary per MP/H Rule M3 (A single tumor is always a single primary), or an additional brain malignancy per MP/H Rule M8 (Tumors with ICD-O-3 histology codes on different branches in Chart 1 or Chart 2 are multiple primaries)?

Partial nephrectomy showed carcinoma, histologic type: succinate dehydrogenase-deficient renal cell carcinoma. This is not a term in the ICD-O, and is not a histology covered in the Kidney MPH rules. However, a recent web search indicates this is a specific type of RCC that was added to the 2016 WHO classification of RCC (per abstract: https://www.ncbi.nlm.nih.gov/pubmed/27179267) and makes up 0.05-0.2% of RCC cases (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4229399/).

This specific histology (cribriform-morula variant of papillary thyroid carcinoma) is not found in the ICD-O and is not mentioned in the 2007 MP/H Manual. However, per a web search it appears that this is a distinct type of papillary thyroid carcinoma (http://erc.endocrinology-journals.org/content/24/4/R109.full).

Example: Right lobectomy shows thyroid epithelial neoplasm, pending consultation.

Consultation: Thyroid gland, right lobe: papillary thyroid carcinoma, favor cribriform-morula variant.

Consultation Comment: IHC stains argue against medullary carcinoma. The histologic features of growth patterns and cytologic atypia (with rare grooves and pseudoinclusions) and the immunohistochemical profile support a diagnosis of papillary thyroid carcinoma, favoring the cribriform-morula variant. It is important to note that a significant number of patients with this variant of papillary thyroid carcinoma have been associated with familial adenomatous polyposis syndrome.

Per Rule M8, this is a single primary as there are multiple urothelial tumors as outlined in Table 1 (papillary urothelial carcinoma [8130] and sarcomatoid urothelial carcinoma [8122]) simultaneously present in multiple urinary organs (bladder, ureter and renal pelvis). As Rule M8 indicates these are a single primary, despite the histologies differing at the third digit (8130 vs 8122), then Rule H14 (Code the histology of the most invasive tumor) seems to be the most applicable histology rule. Following Rule H14 (in the Text version of the MP/H Rules), the histology would be coded as 8122 (sarcomatoid urothelial carcinoma) since the renal pelvis tumor was the most invasive tumor present.

However, in both the Matrix and Flowchart versions of the MP/H Rules, Rule H14 contains a note (missing from the Text version) that states that this rule should only be used when the first three numbers of the histology codes are identical (This is a single primary). Rule M8 clearly tells us these are a single primary, despite the differences at the third digit of the histology. Further defaulting to Rule H15 (Code the numerically higher histology code) in this case would ignore the histology of the tumor with the worse prognosis (the most invasive tumor). Was this note included in the Matrix and Flowchart versions in error?

Since acellular dermal matrix/AlloDerm comes from human tissue donors with cells removed and sterilized to promote regenesis and decrease rejection, is Alloderm coded as "Tissue' as it also "provides an additional layer of tissue between the skin and the implant?

The patient has a history of CLL/SLL dating back to 2007, but has had progressive disease with development of a new left frontal brain tumor. The brain tumor resection proved CLL/SLL with large cell transformation, but neither the pathologist nor the managing physician called this a Richter transformation, Richter syndrome or provided a diagnosis of DLBCL. However, a large cell transformation of CLL/SLL is a Richter transformation. Can this be accessioned as a new acute neoplasm per Rule M10?

Pathology report diagnosis: Urethral lesion: Intraductal carcinoma of the prostate, see microscopic. Clinical Information: Urethral Lesion/Hematura. Microscopic Description: The biopsy shows dilated ductal structures filled with anaplastic epithelium showing areas of comedo-type necrosis. The tumor cells have enlarged nuclei prominent nucleoli and mitoses are identified. Surrounding benign prostatic tissue is also present. Immunostains show that the tumor cells stain for PSA, PSAP, P504s but are negative for GATA-3. The other components of the PIN 4 stain CK5/14 and P63 stain the basal cells surrounding the tumor confirming the intraductal nature of the process. Intraductal carcinoma should not be confused with high grade PIN as the former is usually associated with high grade invasive tumor. Is this C619 and 8500/2?