Primary Site--Sarcoma: What is the best primary site code for an undifferentiated sarcoma of the pulmonary artery? See discussion.
Consolidation of the case: The operating hospital stated: SOFT TISSUE: Resection: Procedure: Radical resection Other: Pneumonectomy Tumor Site: Right pulmonary artery - They used code C383 (mediastinum NOS). The consulting hospital stated: Lung, right, pneumonectomy: High grade sarcoma consistent with intimal sarcoma; tumor involves pulmonary artery. They used code C449 (other soft tissue NOS). Would C493 (soft tissue thorax) be correct?
Code the primary site to pulmonary artery, C493. According to the WHO classification of tumors, intimal sarcomas are malignant mesenchymal tumors arising in large blood vessels. They show mostly intraluminal growth with obstruction of the vessel. They may occur in the pulmonary vessels or, less often, in the aorta.
MP/H Rules/Multiple Primaries: Is this counted as one or two primaries?
Patient is diagnosed with SCC esophageal cancer. Work-up reveals a lung nodule. Lung FNA (cytology) is read by the pathologist as SCC, favor metastatic esophageal SCC. However, the managing physicians are treating the patient as two separate primaries.
If the patient is being managed and treated as a case of primary lung cancer, report the lung diagnosis as a separate primary.
Primary Site--Liver: What is the topography code for combined hepatocellular carcinoma/cholangiocarcinoma (M-8180/3) especially when there is no documentation that intrahepatic bile duct is the tumor site? Reports usually just indicate a liver mass(es) but since the intrahepatic ducts are within the liver, is the code C221 due to the cholangiocarcinoma component, thus making the case stageable?
If there is no further information about where the cancer originated, assign C220. Use ICD-O-3 as the source for coding topography. The topography code associated with combined hepatocellular and cholangiocarcinoma (8180/3) is C220 when there is no other information available, according to ICD-O-3.
Reportability--Brain and CNS: Is pseudotumor cerebri reportable?
Pseudotumor cerebri is not reportable. It is not a neoplasm. The pressure inside the skull is increased and the brain is affected in a way that appears to be a tumor, but it is not a tumor.
MP/H Rules/Histology--Kidney: What is the correct histology for this diagnosis? See discussion.
Procedure: Nephrectomy
Laterality: Left
Tumor type: SOLID VARIANT RENAL CELL CARCINOMA
Nuclear grade: High grade (3/4)
Histologic grade: Poorly differentiated
Pattern of growth: Solid
Tumor size: 5x4.5x4cm
Local invasion: Present
Renal vein invasion: None
Surgical margins: Negative
Non-neoplastic kidney: Unremarkable
Adrenal gland: Not submitted
Lymph nodes: Not present
Pathologic stage: T1b
There are solid sheets of tumor cells without papillary structure. The tumor stains positive for Pax-2, negative for Ecadherin, P63 and CK7, consistent with renal cell carcinoma, solid variant.
Assign histology code 8312, renal cell ca, NOS. There is no specific code for the solid variant of renal cell carcinoma.
Multiple primaries--Heme & Lymphoid Neoplasms: Is this 2 primaries? In 2011, a patient had a spinal mass biopsied positive for DLBCL and follicular lymphoma. The heme rules make this one primary coded as DLBCL. Patient had 2 rounds of chemo, but in 2014, he had a recurrent tumor in the same location. The 2014 biopsy was follicular lymphoma. Is this a new primary -- conversion of acute to chronic after treatment? Or is it the same, since FL was diagnosed in the original specimen?
Rule M13 applies, abstract as two primaries. Since both DLBCL and FL were present in 2011, rule M2 does not fit -- not a single histology. Rule M13 reflects the situation in this case much better: an acute neoplasm which was treated and a chronic neoplasm diagnosed later.
Reportability/MP/H Rules: Where can I find documentation on how to accession malignant tumors in transplanted organs? See discussion.
A patient was diagnosed with hepatocellular cancer (HCC) in 2010, and underwent a hepatectomy, and then received a donor liver. In 2014, HCC was discovered in the liver once again. This likely is a new primary, but there are no specific rules to cover this. There are many odd situations involving transplanted organs, many of which pose reportability and multiple primary problems.
Accession the new tumor in the transplanted organ as you would any other new/second primary. As transplants have become more common especially for liver, lung, and kidney, we are seeing more of these types of cases. We are adding instructions to the revised MP/H rules on coding subsequent primaries when they occur in a transplanted organ. We are also looking at adding a data field that will identify cancers/tumors which arose in a transplanted organ. We feel this is important to track for analysis. Until the revised MP/H rules are implemented, we will look at adding general coding instructions to the SEER Program Manual for transplants.
Reportability/MP/H Rules/Histology: Is malignant perivascular epithelioid cell tumor (PEComa) reportable, and if so, what is the histology code?
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).
MP/H Rules/Histology--Thyroid: When is 8341/3, papillary microcarcinoma coded? The code description in ICD-O-3 is followed by (C739), yet there are two SINQ answers that tell us specifically to not use this code for thyroid primaries. Even the first revision of ICD-O-3 still carries the (C739) as part of this code, which goes against SINQ 20110027 and 20081127.
Per the WHO Tumors of Endocrine Organs, for thyroid primaries/cancer only, the term micropapillary does not refer to a specific histologic type. It means that the papillary portion of the tumor is minimal or occult (1cm or less in diameter) and was found incidentally. WHO does not recognize the code 8341 and classifies papillary microcarcinoma of the thyroid as a variant of papillary thyroid and thereby should be coded to 8260. If the primary is thyroid and the pathology states papillary microcarcinoma or micropapillary carcinoma, code 8260 is correct. This information will be included in the upcoming revisions to the MP/H manual.
MP/H Rules/Histology--Head & Neck: Please clarify rule H3. The first statement is "Do not code terms that do not appear in the histology description". The second statement is "Do not code...unless the words...appear in the final diagnosis"
One of our pathology labs frequently will state "keratinizing squamous cell" in the microscopic description (histologic description), but only state "squamous cell carcinoma" in the final diagnosis. May we code from the histologic description if it's not in the final diagnosis?
Follow rule H3 and code squamous cell carcinoma for these cases unless you can obtain confirmation that these cases should be coded keratinizing squamous cell carcinoma from the lab and/or pathologist. Document this confirmation in your policies and procedures.
The MP/H rules were written with input from leading pathologists in each specialty area. Based on their expert opinion, we instruct registrars to code histology based on the information in the final diagnosis. The microscopic description may contain other terms, but the pathologist lists only the pertinent terms in the final diagnosis.