Report | Question ID | Question | Discussion | Answer | Year |
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20230021 | Histology--Soft Tissue: How is histology coded for malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement diagnosed on left shoulder excision? See Discussion. |
March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation. Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement? |
NTRK-rearranged spindle cell neoplasm is a newly identified variant of sarcoma; however, WHO has not yet proposed a specific ICD-O code for this rare neoplasm. Code to spindle cell sarcoma (8801/3). WHO defines NTRK-rearranged spindle cell neoplasm as an emerging group of molecularly defined rare soft tissue tumors that span a wide group of morphologies and histological grades, and are most often characterized by a spindle cell phenotype among other characteristics. |
2023 |
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20230013 | Reportability/Histology--Skin: Is dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous overgrowth, DFSP with fibrosarcomatous component Grade 2, or DFSP with focal myxoid features (2022) reportable for 2021-2022 diagnoses? |
Yes. DFSP with fibrosarcomatous overgrowth and DFSP with fibrosarcomatous component Grade 2 are synonymous with fibrosarcomatous DFSP (8832/3). Our expert pathologist also advises that DFSP with focal myxoid features is the same as DFSP, myxoid (8832/3). |
2023 | |
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20230039 | Histology/Hematopoietic and Lymphoid Neoplasms--AML: What is the histology code for Acute Myelogenous Leukemia (AML) with monocytic differentiation, 9891/3: acute monoblastic and monocytic leukemia or 9867/3: Acute myelomonocytic leukemia? |
Code AML with monocytic differentiation as acute myeloid leukemia, NOS (9861/3) per consultation with our expert hematopathologist. Acute monoblastic and monocytic leukemia (9891/3) and acute myelomonocytic leukemia (9867/3) are distinct entities according to the WHO. "AML with monocytic differentiation" is a descriptive diagnosis, whereas, "Acute monoblastic and monocytic leukemia" are specific diagnoses. In the WHO Classification of Tumours, Central nervous system tumours (4th Ed) in 2016, WHO began integrating information on molecular alterations that provide significant prognostic implications and/or a therapeutic target into the histology code/term itself. As a result it is also important to look at the molecular testing because acute myeloid leukemias can have different molecular mutations that could result in coding to a different histology code. In this case, there was no other information regarding additional immunophenotyping, so that is why AML, NOS was assigned. Acute myeloid leukemia with monocytic differentiation has been added to the Hematopoietic and Lymphoid Neoplasm Database as an alternate name for 9861/3. |
2023 | |
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20230016 | Solid Tumor Rules/Histology--Brain: How is histology coded for an anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III, diagnosed following a right temporal lobe resection in 2021? See Discussion. |
The patient has a history of ganglioglioma, WHO grade I, involving the deep right parietal lobe diagnosed on resection in 07/2012. Tumor recurrence in 2017 was treated with radiation. The patient then had right temporal tumor biopsy and resection 06/2021 with final diagnosis of anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III. Pathologist notes that the tumor demonstrates a ganglioglioma with frequent mitoses and possible vascular proliferation. Subsequent consult findings support an anaplastic glioneuronal tumor, compatible with progression of the patient's ganglioglioma that is post-irradiation. However, the pleomorphic and epithelioid areas are also reminiscent of pleomorphic xanthoastrocytoma, which may occur in combination with ganglion cell components. There is no related SINQ to code this histology. |
Assign histology as 9505/3. WHO Classification of Central Nervous System (CNS) Tumors describe ganglioglioma as a well-diffferentiated and slow-growing glioneuronal neoplasm. While WHO does not recognize the histology/behavior combination 9505/3, the 2021 CNS Solid Tumor Rules identify non-malignant tumors that have the potential of transforming to a malignant tumor (new primary). Ganglioglioma (9505/1) is listed with the transformed histology and instructs us to code as anaplastic ganglioglioma (9505/3). |
2023 |
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20230008 | SEER Manual/Surgery of Primary Site 2023--Breast: What instructions should be followed when the 2023 SEER Manual Appendix C 2023 Breast Surgery Codes advise to code 1 in Surgical Procedure of Other Site for a simple bilateral mastectomy but the 2023 STORE Manual does not. See Discussion. |
The 2023 SEER Manual, Appendix C 2023 Breast Surgery Codes, note reads: SEER Note: Assign code A760 for a more extensive bilateral mastectomy. Assign code 0 in Surgical Procedure of Other Site (NAACCR #1294). For a simple bilateral mastectomy, assign code A410 with code 1 in Surgical Procedure of Other Site (NAACCR #1294). In the 2023 STORE Manual, these notes are not mentioned and we are instructed not to code surgery to other site. Other education related to 2023 breast coding provided by NAACCR states to not code surgery to other site. |
Assign code 1 in Surgical Procedure of Other Site (NAACCR #1294) when a simple bilateral mastectomy is performed for a single tumor involving both breasts. This statement was inadvertently omitted from the STORE manual and will be added back in: For single primaries only, code removal of contralateral breast under the data item Surgical Procedure/Other Site (NAACCR Item #1294) or Surgical Procedure/Other Site at This Facility (NAACCR Item #674). The information presented by NAACCR was intended to be consistent with what is in the SEER manual. It may have been misuderstood. |
2023 |
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20230069 | First Course Treatment/Immunotherapy--Colon: Is infliximab cancer directed treatment? See Discussion. |
While SEER*Rx does indicate infliximab should be coded as biological response modifier (BRM)/Immunotherapy, the manufacturer website for this medication indicates it is given for: Crohn’s disease, ulcerative colitis, rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and plaque psoriasis. In addition, SEER*Rx does not indicate which primary sites this treatment may be given for. If it is indeed cancer directed treatment, can the typical primary sites be added for clarity? Case example: Patient is diagnosed with colorectal cancer and also has an existing diagnosis of Crohn’s disease; received surgery and FOLFOX6, as well as infliximab. There was no statement of what disease the infliximab was given to treat. |
infliximab is not cancer-directed treatment. This drug was last updated by the FDA 2/22/2023 with additional information on its approval to treat non-malignant neoplasms. To date, the FDA has not approved it for use in colon cancer. This drug was intially developed to treat colon cancer; however, found to be ineffective treating cancer. |
2023 |
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20230014 | Reportability--Thyroid: Is a case with thyroid fine needle aspirate (FNA) cytology with nodule 1 Bethesda category 5 and nodule 2 Bethesda 6, reportable in 2021? Does the Bethesda category 5 or 6 have any bearing on reportability? |
In the absence of information to the contrary, thyroid FNAs designated as Bethesda classification category VI are reportable. Thyroid FNAs designated as Bethesda classification category V are not reportable unless there is additional information confirming a reportable diagnosis. For both Bethesda V and VI, NCCN Guidelines recommend total thyroidectomy or lobectomy (depending on tumor size and nodal involvement) for the purposes of definitive diagnosis/treatment, so additional information should be available. We will add this to the next version of the SEER manual. In your example, nodule 1 Bethesda V is not reportable. Nodule 2 Bethesda VI is reportable. |
2023 | |
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20230025 | Histology--Cervix: Can human papilloma virus (HPV) or p16 testing results from a non-reportable high-grade squamous intraepithelial lesion (HSIL)/cervical intraepithelial neoplasia (CIN 3) pathology report be used to code histology as squamous cell carcinoma (SCC), HPV-positive (8085), if subsequent excision/resection identifies invasive SCC and no further HPV or p16 testing is done on the invasive specimen? See Discussion. |
Example #1: Cervix loop electrocautery excision procedure (LEEP) pathology: Histologic Type: Squamous cell carcinoma, HPV-associated. Histologic Type Comments: High-risk HPV testing on previous Pap test sample reported as positive for high-risk HPV. The prior Pap diagnosis was HSIL only with molecular results positive for high-risk HPV. Example #2: Cervix endocervical curettage and biopsy with CIN 3, p16 diffusely positive. Subsequent LEEP with superficially invasive squamous carcinoma (no HPV or p16 testing done). This was followed by an additional cone excision that was negative for residual malignancy and p16 testing was also negative. |
Use the histology codes SCC, HPV-associated (8085/3) and SCC, HPV-independent (8086/3) only when HPV testing is done on that specimen. Do not use previous HPV tests to code the histology. Code as SCC, NOS (8070/3) in both examples as no HPV testing was performed on the LEEP procedure specimens that identified the SCC. |
2023 |
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20230070 | Solid Tumor Rules/Multiple Primaries--Breast: How many primaries should be accessioned for a diagnosis of invasive carcinoma of the left breast (8500/3) in 2020 followed by a 2023 diagnosis of dedifferentiated carcinoma in the left breast (8020/3)? See Discussion. |
The WHO Blue Books do not include dedifferentiated carcinoma as a valid histology for the breast. However, there is known to be progression of ductal carcinoma that is essentially dedifferentiation of an estrogen receptor, progesterone receptor, and HER2 breast carcinoma to a triple negative "dedifferentiated" carcinoma which it appears this patient has. Whether we should accession this as a separate 8020/3 primary per M14 is unclear and the Solid Tumor Manual does not address this scenario. |
Abstract a single primary using Breast Solid Tumor Rules, Rule M18, as none of the previous rules apply. Undifferentiated carcinoma is a malignant epithelial tumour lacking overt evidence of a specific line of differentiation. Dedifferentiated carcinoma is composed of an undifferentiated carcinoma and a differentiated component. Dedifferentiated carcinoma (8020/3) as a morphology is associated with cancer of the endometrium and ovary rather than the breast. Breast cancer shows a broad spectrum of morphology with extensive variation in histological type and grade, related to the complexity of carcinogenesis. This includes initial genetic changes in the cell of origin, subsequent genetic and epigenetic alterations, and reprogramming that occur at various stages of development along with interaction of other factors that influence the process of differentiation. This scenario likely represents the process of phenotypic change of a carcinoma at a later stage, better known as transdifferentiation. |
2023 |
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20230022 | Solid Tumor Rules/Multiple Primaries: What M Rule of the updated Solid Tumor Rules, Other Sites, applies to a 2022 diagnosis of endometrial cancer, followed greater than one year later by a 2023 diagnosis of esophageal cancer with no interim evidence of tumor recurrence? See Discussion. |
These diagnoses were made greater than one year apart with a disease-free interval and M12 seems to be the first rule that applies. This rule does not specifically state the tumors diagnosed greater than 1 year apart must be in the same primary site but Note 1 could be interpreted as implying this. Note 1 states, “Clinically disease-free means that there was no evidence of recurrence in the same site on follow-up.” Does Other Sites Rule M12 (the timing rule) apply to tumors in different primary sites? It would be helpful if the notes specified this clarification, such as “Clinically disease-free means that there was no evidence of recurrence in the same site (same second and third character CXX.X) on follow-up.” |
Abstract multiple primaries using the Solid Tumor Rules, Other Sites, Rule M13. The topography differs at the second and third characters (C54.1 Endometrium; C15 Esophagus). Rule M12 refers to being disease-free vs. recurrence of a tumor, where Note 1 states that clinically disease-free means no evidence of recurrence in the same site on follow up. A note can be added to clarify that M12 applies to new tumors in the SAME site. |
2023 |