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.
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.
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.
Reportability--Brain and CNS: Is this diagnosis reportable? If this neoplasm originated in the spinal cord, it is reportable, correct?
Specimen is described as a 'spinal cord mass.' The final diagnosis is 'fragments of adipose tissue demonstrating vascular proliferations consistent with angiolipoma. No histologic evidence of malignancy.' The microscopic description says: Sections of the spinal mass reveal bone, cartilage, fibrous tissue and adipose tissue. The adipose tissue demonstrates increased vascularity with thin walled blood vessels seen with islands of delicate fibrous stroma. The histologic findings are compatible with fragments of angiolipoma.
The neoplasm is reportable if it originated in the spinal cord or is intradural (within the spinal dura; spinal nerve roots are intradural). If there is not enough information to determine the exact site of origin, do not report the case.
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.
Primary Site--Skin: Should cutaneous leiomyosarcoma be coded to primary skin of site (C44_) or soft tissue (C49_)?
Code cutanteous leiomyosarcoma to skin. Leiomyosarcoma can originate in the smooth muscle of the dermis. The WHO classification designates this as cutaneous leiomyosarcoma. The major portion of the tumor is in the dermis, although subcutaneous extension is present in some cases.
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.
Reportability--Brain and CNS: Is schwannoma of the extracranial part of a cranial nerve reportable? Some cranial nerves, like facial nerve, have intracranial and extracranial branches.
An extracranial schwannoma is not reportable. The schwannoma must arise on the intracranial part of the nerve to be reportable.
Reportability: Is penile intraepithelial neoplasia, differentiated type, reportable? See discussion.
Foreskin circumcision shows: Penile intraepithelial neoplasia, differentiated type (differentiated PeIN). If reportable, how would the histology and behavior be coded? Is this behavior /2?
For cases diagnosed 2018 and later
Differentiated penile intraepithelial neoplasia (differentiated PeIN), is reportable (8071/2).
Please note: Penile intraepithelial neoplasia, grade 3 (PeIN 3) is also reportable to SEER (C600-C609, 8077/2).
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.
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