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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).

Code AML with monocytic differentiation as acute myeloid leukemia, NOS (9861/3) per consultation with our expert hematopathologist.

Acute monoblastic and monocytic leukemia (9891/3) and acute myelomonocytic leukemia (9867/3) are distinct entities according to the WHO. "AML with monocytic differentiation" is a descriptive diagnosis, whereas, "Acute monoblastic and monocytic leukemia" are specific diagnoses. In the WHO Classification of Tumours, Central nervous system tumours (4th Ed) in 2016, WHO began integrating information on molecular alterations that provide significant prognostic implications and/or a therapeutic target into the histology code/term itself. As a result it is also important to look at the molecular testing because acute myeloid leukemias can have different molecular mutations that could result in coding to a different histology code. In this case, there was no other information regarding additional immunophenotyping, so that is why AML, NOS was assigned.  Acute myeloid leukemia with monocytic differentiation has been added to the Hematopoietic and Lymphoid Neoplasm Database as an alternate name for 9861/3.

Rule H8 applies, code the histology with the numerically higher ICD-O-3 code which is papillary transitional cell carcinoma, 8130.

Based on the information provided, there is a single bladder tumor, papillary urothelial carcinoma with squamous cell differentiation. Urinary sites rule H12 does not apply because this is a single tumor, not multiple tumors. In the single tumor H rules, H3 does not apply as this rule does not include papillary transitional cell carcinoma. Rule H4 is papillary carcinoma or papillary transitional cell carcinoma and refers you to Table 1. Table 1 does not list papillary urothelial carcinoma with squamous cell differentiation because there is no ICD-O-3 code for this histology. Table 1 does list transitional cell carcinoma with squamous differentiation as code 8120, however, the papillary transitional cell carcinoma is the higher code, 8130. We will review this situation for the next version of the rules.

For tumors diagnosed prior to 2007:

Abstract the July 2004 diagnosis as a new endocervical primary. Abstract an invasive cancer in the same site more than two months after an in situ cancer as a new primary. Residual cervical tissue is present following a hysterectomy.

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.

For cases diagnosed 2007 or later:

As long as this is NOT urothelial carcinoma extending to the prostatic ducts, code histology to 8140/3 [adenocarcinoma] using rule H10.

Do not code histologies designated as "focal."

For cases diagnosed 1998-2003:

Code the EOD-Extension field to 55 [Invasion of/through serosa and adjacent connective tissue]. The description states the tumor extended through the serosa into periserosal tissue. The periserosal tissue in this case refers to adjacent connective tissue lying exterior to the intestinal wall and not the (sub)serosal tissue that lies exterior to the muscularis but inferior to the serosa. Analyze each case individually since pathologists are not consistent when using the above terminology.

For tumors diagnosed prior to 2007:

The best histology code for this breast case is 8503/2 [Noninfiltrating intraductal papillary carcinoma]. According to the WHO Classification of Tumors for Breast, Papillary carcinoma, non-invasive is a synonym for Intraductal papillary carcinoma. Further, code a more specific histologic type when found in the microscopic description, according to the SEER Program Code manual.

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.

For cases diagnosed 1998-2003:

For Primary Site and EOD, CT information has higher priority than laryngoscopy. The CT scan gives a better picture of the involvement of the deeper tissues. A laryngoscopy falls into the "physical exam" category more than the "operative" category. The laryngoscopy report is not an "operative" report like those generated from a surgical procedure.

Code the Grade, Differentiation field to 9 [cell type not determined, not stated or not applicable]. For lymphomas, do not code the descriptions "high grade," "low grade," and "intermediate grade" in the Grade, Differentiation field. These terms refer to categories in the Working Formulation and not to histologic grade for lymphoma histologies.

Generally, for histologies other than Non-Hodgkin lymphoma, the Grade, Differentiation field is coded to 2 [low grade], 3 [intermediate grade] and 4 [high grade] for most cancers.