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Patient has a remote history of a right thalamic mass status-post two resections; reported as malignant oligodendroglioma (pathology not received) and chemo/radiation therapy, who recently presented with persistent headaches. Imaging revealed a 3.4 cm heterogeneous lobulated right thalamic mass with coarse calcifications and a probable cystic component.

Pathologist indicates the histologic and immunophenotypic features of this neoplasm are that of relatively circumscribed neuroepithelial tumor without high grade features (mitotic activity, microvascular proliferation, necrosis). Molecularly this neoplasm is characterized by a PATZ1-EWSR1 fusion, which has recently been proposed to be a distinct neuroepithelial tumor entity with a broad histological spectrum.

Pathology reads: 1. FNA left groin lymph node tissue (smears and cell block): B-cell lymphoma, low grade. The concurrent flow cytometry (3-FC-16-288) identifies a monoclonal B cell population with immunophenotype of CD10++, CD5-, CD23-, CD20++ and unusual CD19-. Overall findings favor follicular lymphoma. FNA Specimen Adequacy: Evaluation for specimen adequacy: Immediate cytology smear review for specimen adequacy was performed at the time of the FNA procedure by pathologist. Smears reviewed from 2 passes in one reading. The specimen was adequate cytological evaluation. Surg Path Final Report Special Studies Immunohistochemistry (CD45, MCK, CD20, CD3, CD10, Bcl6, MUM1 \T\ Ki67) was performed on block 1A to confirm the diagnosis. All controls show appropriate reaction. Lymphoma cells are positive for CD45, CD20, CD10 and weakly positive for bcl6(+) and MUM1(+/-), and negative for MCK. CD3 highlights few T lymphocytes. Ki67 labeling index is low, less than 10%. The immunoprofile supports above diagnosis. Chromosomal study for t(14;18) translocation will be performed, and an addendum report will follow. Flow Final Report Comment: The lymphoma appears to be derived from germinal centre B cells. Together with the findings from the lymph node biopsy (3-FN16-416), follicular lymphoma is favored. However, negative CD19 and CD22 are unusual.

This patient had CT scans showing extensive bilateral retroperitoneal lymphadenopathy suspicious for lymphoma and left axillary lymphadenopathy. Thin core biopsies were done of the left axillary lymph nodes and immunohistology pathology was read as malignant neoplasm with extensive necrosis. Flow cytometry analysis of the sample shows no definitive or sufficient CD45+ events for informative analysis. Karyotype analysis could not be performed on this specimen due to inadequate sample. FISH analysis using IGH break apart probe showed no evidence of clonal rearrangement in limited number of cells available for analysis. The physician's diagnosis is probable lymphoma, no further workup felt necessary because patient would not tolerate chemotherapy anyway and hospice was felt most appropriate care for patient.

The definitive diagnostic method for lymphoma, NOS is histologic confirmation, but the only histologic confirmation was of "malignant neoplasm with extensive necrosis." Should the histology and diagnostic confirmation be coded as lymphoma, NOS [9590/3] and imaging without microscopic confirmation [7] or malignancy, NOS [8000/3] and positive histology [1]?

5/15/17 Duodenal bulb, biopsy: Fragments of duodenal mucosa with well differentiated neuroendocrine tumor (carcinoid), extending to the edge of specimen and peptic duodenitis in the submitted tissue. No significant intraepithelial lymphocytosis.

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.