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In September 2009 the patient was diagnosed with seminoma, classical type, following an orchiectomy. Testicular mass recurrence in 2014 was treated with chemotherapy.

Then in April 2015 a retroperitoneal dissection of a peri-aortic LN was positive for mixed germ cell tumor with somatic type malignancy (rhabdomyosarcoma) involving 1/11 nodes. Path Comment: major component of tumor is teratoma, rhabdomyosarcoma represents <5% of mass.

Now in October 2016, the patient has a retroperitoneal mass biopsy positive for spindle cell sarcoma with rhabdomyosarcomatous differentiation. The comment section of the pathology report states, "Given the history of a germ cell tumor w/ rhadbomosarcomatous component, the findings are consistent with a recurrence of rhabdomyosarcomatous component of germ cell tumor."

Can a seminoma transform to a mixed germ cell tumor with a somatic type malignancy (see SINQ 20140082 - testicular teratoma with somatic type malignancy)?

The 2016 WHO Classification of Tumours of the Urinary System appears to be moving away from using Fuhrman grading toward using WHO/ISUP grade. These seem like similar 4 grade staging systems; however, the SEER Manual specifically states to not use the Special Grade System table for WHO/ISUP. We are seeing the WHO/ISUP grade being used on 2016 pathology reports.

Examples of new grading for renal cell carcinomas

Histologic type: Clear cell renal cell carcinoma

Histologic grade (WHO/ISUP 2016): Grade 3 in a background of 2 (of 4).

And

Histologic type: Clear cell renal cell carcinoma

Histologic grade (ISUP): Grade 2.

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.

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?

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?

CT-guided liver biopsy, diagnosis: Metastatic neuroendocrine carcinoma. Diagnosis Comment: Cytology of the tumor appears to be low to intermediate grade.

Would this case be coded as an atypical carcinoid tumor (8249/3) based on SINQ 20170033 and the statement of intermediate grade; or should this be 8240/3 (neuroendocrine tumor) per SINQ 20160023 because it is a metastatic site? More clarification is needed on when to code 8249/3 or 8240/3 for a neuroendocrine carcinoma or neoplasm seen in a metastatic specimen only when there is specified grade.