Report | Question ID | Question | Discussion | Answer | Year |
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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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20230079 | Solid Tumor Rules/Histology--Cutaneous Melanoma: How is histology coded for a 2023 diagnosis of “early lentiginous melanoma in situ” of the skin? See Discussion. |
Previous SINQ 20091100 has a similar scenario and the instruction was to code as lentigo maligna (8742/2); however, it does not appear to be applicable to cases diagnosed after 2020. The WHO Blue Book does not list melanoma, lentiginous type or lentiginous melanoma in situ as an alternate term for lentigo maligna and neither do the STR or the ICD-O-3.2. |
Assign code 8742/2 (lentigo maligna) for “early lentiginous melanoma in situ.” ICD-O-3.2 lists the preferred term for 8742/2 as lentigo maligna (C44._). |
2023 |
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20230052 | Reportability/Primary Site--Brain and CNS: What is the primary site of a meningioma arising from the jugular bulb/petrous aspect of the temporal bone? See Discussion. |
Example July 2022, Brain CT describes a mass appearing to be centered on the petrous aspect of the temporal bone with intracranial and extracranial extension. July 2022, Brain MRI describes an extra-axial mass centered in the right jugular bulb with intracranial and intraosseous extension as well as extension within the internal jugular vein. September 2022, Resection operative report surgical findings are of a calcified mass filling middle ear, abutting stapes and appearing to enter the stapes obturator foramen, debulked. Final diagnosis is right middle ear meningioma, WHO grade I of III. Is this a reportable intraosseous meningioma of the temporal bone/skull base, or a non-reportable meningioma arising in a meningocele within the middle ear? |
Do not report cases of meningioma originating in the jugular bulb or petrous aspect of temporal bone or middle ear. These are not intracranial locations. This is a non-reportable meningioma arising in a meningocele within the middle ear. The jugular bulb is the confluence of the lateral venous sinuses situated in the jugular fossa. The precise location of this structure within the temporal bone is variable.The jugular bulb, petrous aspect of temporal bone, and middle ear are not intracranial locations, and therefore meningiomas arising in these areas are not reportable. |
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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20230020 | First Course Treatment/Reason for No Surgery of Primary Site: How should Reason for No Surgery of Primary Site be coded for cases when surgery was planned but aborted due to extent of disease seen during planned procedure? See Discussion. |
Lung abnormality on imaging prompted diagnosis on subsequent biopsy and clinical staging was documented as cT1b N0 M0. There was an attempt at resection, but the patient was found to have chest wall involvement and the procedure was aborted. How would Reason for No Surgery of Primary Site be coded in these types of scenarios when the surgery is aborted and the treatment plan changes due to the extension seen during surgery? |
For the example provided: For 2023 cases and forward, if no part of the surgery was performed, code Surgery of Primary Site 2023 (NAACCR Item #1291) as code A000 or B000 (no surgical procedure of the primary site). Code Reason for No Surgery of Primary Site (NAACCR Item #1340) as code 2 (surgery of the primary site was not recommended/performed because it was contraindicated due to patient risk factors (comorbid conditions, advanced age, progression of tumor prior to planned surgery, etc.). In contrast, if any part of the surgery was performed, assign the Surgery of Primary Site 2023 (NAACCR Item #1291) code that best reflects the extent of the surgery performed. Code Reason for No Surgery of Primary Site (NAACCR Item #1340) as code 0 (surgery of the primary site was performed). Use text fields to record the details. For cases prior to 2023, apply the same approach using Surgery of Primary Site (NAACCR Item #1290) instead of Surgery of Primary Site 2023 (NAACCR Item #1291). |
2023 |
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20230036 | Reportability/Histology--Vulva: Is angiomyxoma (8841/1), such as aggressive angiomyxoma of vulva diagnosed in 2022, reportable? |
Do not report superficial angiomyxoma (8841/0) or aggressive angiomyxoma (8841/0). WHO Classification of Female Genital Tumors, 5th edition, defines deep (aggressive) angiomyoma as a benign, infiltrative, myxoid spindle cell neoplasm that occurs in deep soft tissue of the pelviperineal region. |
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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20230017 | Solid Tumor Rules/Multiple Primaries--Rectum/Anal Canal: How many primaries are accessioned and how should histology be coded for a 2021 abdominoperineal resection showing invasive adenocarcinoma of distal rectum and associated Paget disease of the anal mucosa and perianal skin? See Discussion. |
The synoptic report calls this “Invasive adenocarcinoma with secondary Paget disease of anal mucosa and perianal skin.” The tumor size is listed as “2.1 x 1.7 x 0.7 cm, including associated advanced adenoma; size does not include the extent of the associated Paget disease, which extends for at least 2 cm distally.” Clinically this is called an incidentally discovered Paget’s disease. It is unclear if this is a collision tumor that should be abstracted as separate primaries, or if this is a single tumor with underlying Paget’s disease (similar to that described in Other Sites Rule H26). If this is a single rectal tumor, there does not appear to be an H rule for this scenario. |
Abstract two primaries using rule M4 of the Colon rules or rule M13 of Other Sites: 1. Invasive adenocarcinoma of distal rectum and 2. Paget disease of the anal mucosa / perianal skin (determine site of origin and code primary site accordingly). The rectum and the anus are separate sites and the histologies differ in each site. The WHO Classification of Digestive System Tumors, 5th edition, states that in addition to secondary anal Paget disease arising from anal canal adenocarcinoma, or rarely, adenoma without documented invasive disease, secondary Paget cells may be contiguous with the underlying neoplasm or manifest at different at sites distinctly away from it (with skip lesions). Document the details in the appropriate text fields. |
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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20230050 | Reportability/Histology--Soft Tissue: Is a diagnosis of Myofibroblastoma with sarcomatous transformation a reportable malignancy? See Discussion. |
Patient was diagnosed in September 2022 via excision of a 12 cm pelvic mass with final diagnosis of Myofibroblastoma with sarcomatous transformation. Diagnosis comment states, “Most of the tumor is composed of conventional features of myofibroblastoma. However, a focal area demonstrates increased cellularity, fascicular growth and increased mitotic activity (up to 11 per 10 hpf), consistent with sarcomatous transformation (morphologically low to intermediate grade).” Is this sarcomatous transformation describing a malignant transformation from an otherwise benign histology? If so, how should histology be coded in this case? |
Do not report the case. The histology is 8825/0 based on the example provided and not reportable. Myofibroblastoma with sarcomatous transformation is a rare, benign condition, sometimes referred to as sarcomatous features. A malignant tumor would be referred to as a myofibroblastic sarcoma. |
2023 |