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Based on the information provided, code the primary site to urachus (C677). Primary adenocarcinoma of the bladder accounts for less than 1% of all bladder malignancies. Of these, 20–39% are urachal in origin.

Kidney rule H5 applies, code the more specific histology which is clear cell renal cell carcinoma (8310/3). Per the WHO Tumors of the Urinary System, clear cell renal cell carcinoma contains both clear and eosinophilic cytoplasm. Eosinophilic is not a type or variant of renal cell carcinoma.

Carcinoma ex pleomorphic adenoma (Ca-ex-PA) is reportable. Assign 8941/3. The WHO classification of head and neck tumors defines Ca-ex-PA as an epithelial malignancy arising in a benign pleomorphic adenoma. Most of these originate in the parotid gland but can also arise in other salivary glands.

Do not report a case based on UroVysion test results alone. Report a case when there is positive histology, a physician statement of malignancy, and/or the patient was treated for cancer.

1. Do not report the case.

2. Report the case based on the positive biopsy.

Do not report primary acquired melanosis with atypia.

According to our expert pathologist consultant, "There has been a lot of debate in the literature about the diagnostic criteria, terminology, and natural history of primary acquired melanosis [PAM]. Your case comes down squarely on the main issue, which is whether PAM with atypia should be regarded as melanoma in situ. In most studies it appears that PAMs with no atypia or mild atypia do not progress to melanoma, and only a small percentage of those with severe atypia do so."  "PAM, even with atypia, is not melanoma in situ, and should not be reported."

For further information, see this article for a review of a large number of patients: Shields, Jerry A, Shields, Carol L, et al. Primary Acquired Melanosis of the Conjunctiva: Experience with 311 Eyes. Trans. Am Ophthalmol Soc 105:61-72, Dec 2007.

Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.

We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.

Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).

Apply the Multiple Primaries/Histology, Breast Rule H3: DCIS and a more specific in situ are coded to the more specific histology term which in this case is solid. Code the histology to ductal carcinoma in situ, solid type (8230/2). Based on the information provided, there is no invasive component. The term "microca ++" means micro-calcifications are present, not micro carcinoma.

Assign grade code 4 using the sarcoma table. Nottingham or Bloom-Richardson (BR) Score/Grade does not apply to angiosarcomas. This is a good question and points out needed clarification of the grade rules.

Sequence the brain tumor in the 60-87 series when you do not know the behavior. If you have reason to believe the brain tumor was malignant, sequence it in the 00-59 series.

Assign 8441/2. This is based on the WHO classification for female reproductive system tumors.