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12/3/2020 Pathology: AML: Blasts 40% of nucleated cells. CD45 positive, CD34 negative, CD 117+,

CD13 positive, CD33 positive in 59.6% and HLA-DR was dim and myeloperoxidase was dim.

Cytogenetics normal karyotype. The next generation sequencing detected IDH 2p.(R172K)c515>A.

Because this was AML NOS, we consulted with the physician. The physician stated the patient had AML with recurrent genetic abnormalities"and the basis for the diagnosis was the IDH-2 mutation identified on Next Generation Sequencing. We assigned 9896/3, based on the physician's interpretation of the pathology. This histology is being questioned.

Disease History

2018 - Lymphomatoid papulosis (non-reportable)

2020 - Transform to CTCL (and called Mycosis Fungoides specifically) (CTCL/MF same primary)

2021 - Transform to CTCL with large cell transformation

Example: Bone marrow biopsy diagnosis is chronic myelogenous leukemia, chronic phase, and the RT-PCR test result proved, BCR-ABL1 p210 (Major Breakpoint) - Detected, 3.3659%.

Even though the p210 fusion transcript was less than 5%, it was detected. The presence of BCR-ABL1 does define whether or not patients are treated with tyrosine kinase therapies. Therefore, it seems likely that the presence of any BCR-ABL1 would be captured using the more specific histology code 9875/3, instead of the non-specific CML, NOS histology code 9863/3.

Are there minimum threshold requirements for these quantitative studies in order to code the histology to the more specific type of CML?

Left mandible resection: Malignant tumor, favor high grade sarcomatoid carcinoma. Please see comment.

Comment: Considering the focal stain with P63 and the consult from Mayo Clinic done on the previous biopsy, the diagnosis of sarcomatoid carcinoma is more likely.

Gross: left mandible resection...sectioning reveals a...mass that has replaced the majority of the mandibular bone and is at the medial, anterior lateral and posterior soft tissue margins and comes to within 2.4 cm of the anterior boney resection margin and 1.9 cm of the smooth articular temporal mandibular joint surface.

The combination of C411 and 8033/3 is impossible (with no override available).

We are confused by SINQs 20180097, 20150001, and 20140051.  The latter two indicate that “well-differentiated neuroendocrine neoplasms” of the duodenum and appendix are reportable because they’re synonymous with neuroendocrine tumor (NET).  Yet 20180097 states “primary hepatic neuroendocrine neoplasm” is NOT reportable unless there is documentation that it’s being used as a synonym for Primary Hepatic Neuroendocrine Tumor (PHNET).  In addition, we see in the 2021 ICDO-3.2 update that only “poorly differentiated neuroendocrine neoplasm” is listed with behavior code /3 and noted to be reportable for 2021+ on the companion annotated histology list.  Does reportability of neuroendocrine neoplasms depend on primary site, differentiation terminology within the histology name, or something else?  Our casefinding staff is hoping for a general reportability guideline to follow when they come across “neuroendocrine neoplasms” NOS.  For example, we have a 2020 pathology report for a core biopsy of a soft tissue pelvic mass with final diagnosis of low grade neuroendocrine neoplasm; there is no further clarification as to whether it is felt to be primary or metastatic, and we have no other associated records for this patient in our central registry.

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

Pathology report states, "Histologic sections reveal lymph node metastases, consisting predominantly of mature teratoma. In addition, there are cells scattered through the fibrous stroma which exhibit mild cytologic atypia but have low N:C ratios. The largest metastasis grossly measures 10cm. In addition extracapsular extension is identified. Diagnosis: Lymph Nodes--Metastatic Testicular Carcinoma Involving Multiple Lymph Nodes." The morphology code for mature teratoma is 9080/0. The pathologist does not classify this as an immature teratoma (9080/3). Is this reportable?

In 11/2006 the patient had surgery for a 6cm mass in the RUQ arising in the falciform ligament. The pathologic final diagnosis was: Perivascular epithelioid cell neoplasm (PEComa) of uncertain malignant potential. In 10/2009 a liver biopsy showed metastatic perivascular epithelioid cell neoplasm.