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Solid pseudopapillary neoplasm of the pancreas is reportable. According to the WHO classification, it is a "low-grade malignant neoplasm…[which] frequently undergoes hemorrhagic-cystic degeneration and occurs predominantly in young women."

Assign topography code C25 with the appropriate 4th digit. Code the histology as 8452/3.

For tumors diagnosed prior to 2007:

The presence of numerous/multiple polyps is not necessarily adenomatous polyposis coli. Adenomatous polyposis is an extreme condition usually characterized by the presence of hundreds of polyps and should be identified as such either clinically or pathologically.

Look for the term "Familial adenomatous polyposis," FAP or one of its synonyms:

Adenomatosis of the colon and rectum [ACR]

Familial adenomatous colon polyposis

Familial colonic polyposis

Multiple familial polyposis

In the absence of these terms, the following probably indicate a diagnosis of FAP:

Hundreds of adenomatous polyps throughout large intestines, and at times, throughout the digestive system

Development of polyps as early as ten years of age, but more commonly at puberty

History of colectomy

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.

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, those modalities should be counted as part of first course of cancer-directed treatment if the patient continues with the planned first course. For example, if patient has the originally planned type of surgery, radiation, or drug protocol, then code the given treatment as first course.

Caution: It is not a change in the treatment plan if the drugs are changed but the action of the drugs remains the same. This is still first course. However, if the treatment is changed from a chemotherapy drug to a hormonal drug following the discovery of the mets, do not code the hormonal therapy as first course.

Do not use the date of a positive Cologuard test as the date of diagnosis.

The primary site is C482 [Peritoneum, NOS]. "Paracaval" means alongside or near the vena cava.

Code the site in which the primary tumor originated.

For cases diagnosed 2007 or later, assign histology code 8503 [Papillary adenocarcinoma]. Rule H14 applies.

ICD-O-3 code 8050 does not apply in this case. Refer to the numeric listing in ICD-O-3. 8050 is a squamous cell neoplasm. Papillary carcinoma of the breast is NOT a squamous cell neoplasm. It is a neoplasm of the breast parenchyma - ducts, lobules or connective tissue. 8503 is the correct code in this case.