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A physician stated the patient likely had secondary polycythemia vera due to cardiac and pulmonary conditions but that a polycythemia vera could not be ruled out. A JAK2 was ordered that was positive for JAK2 V617F mutation. The patient was treated with hydrea.

According to SEER SINQ 20120049, secondary polycythemia vera is not reportable. However, in this case, the patient was positive for JAK2 V617F mutation. Therefore, is this reportable? We looked for guidance in the Hematopoietic and Lymphoid Neoplasms Database and found it confusing that secondary polycythemia vera was not mentioned or discussed under polycythemia vera in the database. The only thing we could find was secondary polycythemia NOS that was discussed under polycythemia.

Ampulla, biopsy: High grade dysplasia with focal intramucosal carcinoma in a background of ulceration with acute and chronic inflammation.

Surgery pathology: Head of pancreas, duodenum, and distal stomach, pancreaticoduodenectomy-Ampulla, Adenocarcinoma, intestinal type, intra-ampullary and peri-ampullary (mixed type). Grade moderately differentiated, 1.5cm. Tumor invades into duodenal submucosa. Lymphovascular Invasion: Foci suspicious for lymphovascular invasion identified. Perineural Invasion: Present.

Synoptic report: Tumor Site Intra-ampullary and peri-ampullary (mixed type). Histologic Type Adenocarcinoma, intestinal type.

There is not enough information regarding site in radiology reports or operative report. CT-A/P/C:The patient's known ampullary mass is not well visualized on this exam. No significant intrahepatic or extrahepatic biliary ductal dilation is identified. The pancreatic duct is normal caliber.

The SEER Manual states to assign Tumor Size--Pathological code 000 when EOD Primary Tumor is coded to 800 (No evidence of primary tumor) for any schema. However, the definition of Tumor Size--Pathologic states that it records the size of a solid primary tumor that has been resected.

If the primary site has not been resected (does not meet the pathologic staging criteria), then it seems that Tumor Size Pathologic should be 999 when EOD Primary Tumor is coded as 800.

HL-7 e-path report, Final Diagnosis

High normocellular marrow with maturing trilineage hematopoiesis, multilineage dyspoiesis, compatible with MDS-MLD and involvement by plasma cell neoplasm/myeloma, IgA kappa positive, approximately 20-25% of total cellularity present. See comment.

Comments

Correlation with other relevant laboratory (amount and type of serum and urine paraprotein levels, renal function tests, serum calcium level, and anemia) and radiologic (lytic bone lesions) findings is recommended for complete interpretation.  Dyspoiesis of all lineages is seen and the findings are compatible with low grade myelodysplastic syndrome (MDS-MLD), assuming that other possible causes are excluded. Correlation with cytogenetic and molecular studies is recommended for complete characterization

The Solid Tumor histology coding guideline #3 for Lung states that an ambiguous histology can only be coded over an NOS when a physician clinically confirms it or the patient receives treatment based on the ambiguous histology; similar instructions exist in rules H3 and H12.  We are in a central registry and don't typically have access to physician notes or treatment plans; unfortunately our hospital abstracts rarely document physician confirmation of ambiguous histology and we are uncertain if we should accept their coding of the more specific histology, assuming they did find clinical confirmation that was not documented.  If not, our understanding of the Solid Tumor rules is that the histology in such a case would have to be coded as malignancy NOS (8000/3) per the non-ambiguous final diagnosis, and that we cannot use the more specific but ambiguous squamous cell carcinoma since we don't have definite clinical confirmation.  We also have a fair number of cytology-only lung cases without any hospital information to clinically confirm an ambiguous histology.

Patient was diagnosed with carcinosarcoma of Mullerian origin on omental/pelvic biopsies in March 2021. First course treatment was neoadjuvant chemotherapy followed by July 2021 resection showing residual primary endometrial carcinosarcoma with cervical stromal invasion and involvement of bilateral tubes/ovaries, omentum, and mesenteric nodule. Additional findings included endometrium with background endometroid intraepithelial neoplasia (EIN).

The Breast EOD Regional Nodes notes contain new clarification regarding the clinical assessment vs. pathological assessment codes, but the new Note 2 does not specifically indicate an exception for neoadjuvant therapy. However, if the pre-treatment lymph node core biopsy proved cN1 disease, and the post-treatment resection proved ypN0 disease, should the clinical assessment code (code 150) have priority over any pathological assessment code (including 200) since the involved lymph node was only clinically positive and not pathologically positive? Should an exception be added to Note 2 to address cases where neoadjuvant therapy is given, but the clinical assessment is greater than the pathological assessment?