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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).

Yes, Breast rule M10 applies. This case is a single primary.

Follow the MP/H rules even though the "pathologist specifically states the tumors are morphologically different" so that situations like this are reported consistenty accross cancer registries, regions, and states for consistent national reporting.

Yes, assign diagnostic confirmation code 2 for diagnosis based on smears and cell block from CT guided FNA. This reply pertains to solid tumors.

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.

Code the histology to 8342/3, thyroid oncocytic (oxyphillic) papillary carcinoma.

Typically, the final diagnosis of the reviewing pathologist is the one used to determine whether the case is SEER reportable. If two or more reviewing pathologists disagree as to whether the case should be reportable, determine reportability based on the following priority order:

1) If the patient is treated for cancer, the case is reportable.

2) If the patient is not treated for cancer, use the amended diagnosis on the original pathology report if the hospital pathologist used the reviewing pathologists' opinions in establishing his new diagnosis.

3) If there is not an amended diagnosis for the original hospital pathology report, use the clinician's opinion regarding what the diagnosis is to determine whether the case is reportable.

For tumors diagnosed prior to 2007:

Code the Histology field to 8480/3 [mucinous carcinoma]. This answer assumes the patient presented with a single tumor. There is no combination code that includes these two entities. According to the rules for Coding Complex Morphologic Diagnoses, it would appear that the case should be coded to 8540 [Paget disease] because it is the higher code. However, this combination of histologies represents an exception to that rule. The prognosis for mucinous carcinoma is worse than the prognosis for Paget disease. As a result, it would be more appropriate to the histology to mucinous carcinoma.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.

Thalamic amyloidoma is not reportable. Amyloidoma (tumoral amyloidosis, amyloid tumor) is a tumor-like deposit of amyloid. It is not neoplastic. Amyloid is a protein derived substance deposited in various clinical settings.