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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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20230047 | Reportability/Histology--Head & Neck: Is a 2023 mandibular biopsy showing “severe squamous dysplasia with microscopic focus suspicious for superficial invasion” reportable? See Discussion. |
Patient had a mandibular mucosal lesion resected in June of 2023, with a diagnosis of “atypical squamous proliferation” and case was forwarded to an expert in oral pathology for best classification. Subsequent slide review final diagnosis was “moderate to severe squamous dysplasia.” That slide review diagnosis goes on to state “microscopic focus suspicious for superficial invasion.” Currently there is no ICD-O code for severe squamous dysplasia, however it is unclear if this terminology is equivalent to high grade squamous dysplasia (histology code 8077/2). |
Report as squamous cell carcinoma (8070/3) on the basis of “microscopic focus suspicious for superficial invasion.” "Severe dysplasia" is equivalent to "high grade dysplasia" in the Head and neck. As such, "severe squamous dysplasia" would be coded to 8077/2. However, in combination with the statement of "with microscopic focus suspicious for superficial invasion,” report as squamous cell carcinoma (8070/3) based on “microscopic focus suspicious for superficial invasion.” The 2023 SEER Manual instructs us to code the behavior as malignant (/3) if any portion of the primary tumor is invasive no matter how limited, i.e., microinvasion. Use text fields to record the details. |
2023 |
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20230068 | Solid Tumor Rules/Histology--Thyroid: What is the histology code for a diagnosis of poorly differentiated thyroid carcinoma arising in a background of solid papillary thyroid carcinoma? See Discussion. |
Patient had a hemithyroidectomy with the final diagnosis above. There does not appear to be an Other Sites H rule or table that addresses this combination of histologies for thyroid primaries. |
Code to poorly differentiated thyroid carcinoma, 8337/3. In this case the tumor is comrpised of two difffernat thyroid histologies: poorly differentiated carcinoma 8337/3 and papillary thyroid carcinoma 8260/3. WHO does not have a code for this combination. Per our endocrine pathology expert, the poorly differentiated carcinoma is the more agressive histology and will determine treatment and progrnosis. |
2023 |
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20230056 | Reportability/Histology--Heme and Lymphoid Neoplasms: What is the histology code for nodular lymphocyte predominant B cell lymphoma that is never called Hodgkin lymphoma? Is it acceptable to record the histology code for nodular lymphocyte predominant Hodgkin lymphoma, (9659/3)? See Discussion. |
Patient has a history of human immunodeficiency virus and diffuse large B cell lymphoma diagnosed in 2012, and is status/post systemic therapy and in remission since completing first course treatment. In 2022, the patient has imaging suspicious for recurrence. A biopsy of a deep left cervical lymph node showed atypical lymphoid infiltrate with the comment: “This is a challenging case. The constellation of findings is most in keeping with early / focal and subtle involvement by a nodular lymphocyte predominant B-cell lymphoma. We find no evidence of involvement by a diffuse large B-cell lymphoma.” The managing physician later states, “Cervical lymph node biopsy (06/2022) was consistent with nodular lymphocyte predominant B cell lymphoma.” |
According to the 5th edition WHO Blue Book for Hematopoietic Neoplasms, Beta Version, (not released yet), nodular lymphocyte predominant B-cell lymphoma is an alternate name for 9659/3. We will update the Heme database once the 5th edition is released in print. |
2023 |
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20230012 | Solid Tumor Rules/Multiple Primaries--Prostate: How many primaries are accessioned when a 06/2022 diagnosis of prostate adenocarcinoma is followed less than one year later by a 01/2023 diagnosis of small cell carcinoma (SmCC)? See Discussion. |
Rule M4 was added to the Other Sites M Rules to address diagnoses of small cell carcinoma following prostate adenocarcinoma, but Rule M4 states the diagnoses must be greater than one year apart. In this situation, the diagnoses were less than one year apart and one must continue through the M Rules. The next M Rule that applies Rule M19: “Abstract multiple primaries when separate/non-contiguous tumors are on multiple rows in Table 2-21 in the Equivalent Terms and Definitions. Timing is irrelevant.” If one were to STOP at the first rule that applies, one would stop at Rule M19 which confirms the prostatic adenocarcinoma and small cell carcinoma are separate primaries, regardless of timing. If these are not to be accessioned as multiple primaries, does an Exception need to be added to M19? |
Assuming the smal cell is a seperate tumor, accession two primaries, adenocarcinoma (8140/3) of the prostate and SmCC (8041/3) of the prostate using Rule M19 of the current Other Sites Solid Tumor Rules. As these two tumors are less than a year apart, Rule M4 does not apply; however, Rule 19 does apply as these are two distinct histology types. It takes time for an acinar tumor to transform into the small cell and it is usually triggered by hormone and/or radiation treatment. |
2023 |
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20230003 | SEER Manual/Reportability--Ambiguous Terminology: Please clarify the reportability and relevant date ranges of the following ambiguous terminology: almost certainly, most certainly, and malignant until proven otherwise. See Discussion. |
SINQ 20180104 indicates, in the absence of further info, the terms “almost certainly” and “until proven otherwise” are NOT reportable. There is no date range provided for this answer. SINQ 20200027 indicates, in the absence of further info, the term “most certainly” IS reportable. There is no date range provided for this answer. SEER Program Coding and Staging Manual 2022 indicates, in the absence of further info, the terms “until proven otherwise” and “most certainly” ARE reportable. Essentially, we are hoping for an update of SINQ 20180104 due to 2022 reportability change. Clarification to the equivalence of “almost certainly” and “most certainly” would also be helpful. |
Use the ambiguous terminology list as a guide in the absence of additional information after reviewing all available information and consulting the physician who diagnosed and/or staged the tumor. Equivalent to "Diagnostic for" malignancy or reportable diagnosis
Not Equivalent to "Diagnostic for" malignancy or reportable diagnosis
We will update SINQ 20180104. |
2023 |
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20230074 | Extent of Disease/EOD Regional Nodes--Small Intestine: For an ileal/jejunal neuroendocrine primary, how should mesenteric soft tissue deposits (less than 2 cm) be collected in Extent of Disease (EOD) Staging? See Discussion. |
Example: Patient is diagnosed with grade 1 well-differentiated neuroendocrine tumor of the ileum, confirmed on ileocolic resection in 2023. The final diagnosis is a 2.8 cm ileal mass, with focal lymph-vascular invasion and a single 0.6 cm tumor deposit within mesenteric fat; primary tumor completely resected with widely negative margins and 10 regional nodes negative for malignancy. According to AJCC, mesenteric masses less than 2 cm should be stated in the pathology report as being present and collected by registrars but do not affect stage. EOD Regional Nodes has a code for large mesenteric masses greater than 2 cm only. How should we record these smaller tumor deposits if they are not supposed to affect stage? |
Do not code 500 for involvement of the mesentery unless the mesentery is specifically stated to be involved (and we don't have that information). We need more information on this case to assign EOD primary tumor. EOD Regional Nodes would be 000 per AJCC. |
2023 |
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20230059 | Histology--Heme and Lymphoid Neoplasms: How is histology coded for a diagnosis stated as MDS/AML (myelodysplastic syndrome/acute myeloid leukemia) per the international consensus classification (ICC)? See Discussion. |
The final diagnosis on bone marrow biopsy was high grade myeloid stem cell neoplasm, 17% blasts by differential count. The pathologist further states that this could be classified as “MDS with increased blasts (MDS-IB2) per the WHO 5th edition classification, or MDS/AML per the international consensus classification (ICC).” FISH and cytogenetics revealed a loss of 7q, but no other AML-related genetic abnormalities. The physician confirms the patient has MDS/AML. |
Updated Answer July 2024 Code histology as myelodysplastic neoplasm with increased blasts (9983/3) based on the WHO Classification of Hematolymphoid Tumors, 5th edition, Beta version 2. WHO lists MDS with increased blasts-2 (MDS-IB2) as a subtype of 9983/3. Terms coded to 9983/3 include
When differences exist between WHO and ICC, assign the histology based on the WHO Classification. |
2023 |
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20230009 | Solid Tumor Rules/Multiple Primaries--Vulva: How many primaries are accessioned when a 2023 diagnosis of keratinizing squamous cell carcinoma (SCC) (8071/3) of the vulva follows a previous diagnosis of nonkeratinizing SCC (8072/3) of the vulva and the timing rule (M12) does not apply? See Discussion. |
Table 19: Vulva Histologies of the Other Sites Solid Tumor Rules does not include entries for either keratinizing or nonkeratinizing squamous cell carcinoma in the “Squamous cell carcinoma, NOS” row. However, these are two distinctly different histologies per the ICD-O-3.2. All other Solid Tumor Rules schemas include an M Rule instructing one to abstract multiple primaries when separate/non-contiguous tumors are two or more different subtypes/variants in Column 3 of the Specific Histologies, NOS, and Subtype/Variants Table for the schema (e.g., Rule M6 for Lung). The timing of these tumors is stated to be irrelevant. The Notes confirm the tumors may be subtypes/variants of the same or different NOS histologies and tumors in column 3 are all distinctly different histologies (even if they are in the same row). However, the 2023 Other Sites schema appears to be missing this rule. Should these distinctly different histologies be accessioned as separate primaries? Is an M Rule missing from the Other Sites schema to address distinctly different histologies? |
Table 19 is based on WHO 5th Ed Tumors of vulva and squamous cell variants, keratinizing and non-keratinizing, are no longer recommended and are excluded from the 5th Ed. HPV related terminology is now preferred for these neoplasms. Per consultation with our GYN expert pathologist, based on the information provided, this is likely a single tumor that was not completely excised in the original biopsy. A new tumor in the same site would not appear within 8 months. If you cannot confirm two separate/non-contiguous tumors were present, abstract a single primary per M1. As for histology, the tumor showed both keratinizing and non-keratinizing features and HPV status is unclear. Per our expert, code to SCC 8070/3—keratinization or lack of does not change treatment or prognosis. Even If there is proof of separate/non-contiguous tumors, our expert still feels this is a single primary coded to SCC 8070/3. Treatment does not differ by keratinization or HPV status. Coding two primaries would be incorrect and inflate incidence rates. Per our expert, this is an unusual occurrence. The rules cover 85% of cases but there will always be situations that do not fit a rule. This case is an example of that. A new GYN specific Solid Tumor Rules module is under development and a rule to address this situation could be included. |
2023 |
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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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