| Report | Question ID | Question | Discussion | Answer | Year |
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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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20230031 | Solid Tumor Rules/Multiple Primaries--Lung: How many primaries and what M Rule applies to a 2022 diagnosis of right upper lobe non-small cell lung carcinoma (NSCLC) when the patient has a history acinar adenocarcinoma in the right lower lobe of the lung in 2020, followed by squamous cell carcinoma in the right middle lobe of the lung in 2021? See Discussion. |
The patient was not synchronously diagnosed with multiple tumors, but three separate tumors with three different histologies were diagnosed at different times and no more specific histology was provided for the NSCLC. The timing rules do not apply to this case (the tumors were not greater than 3 years apart and they were not synchronously/simultaneously diagnosed). While NSCLC is a NOS histology for both adenocarcinoma and squamous cell carcinoma, it is unclear if Rule M8 should apply because NSCLC is not listed in Table 3 (Table 3 is not an exhaustive list). In some situations, Rule M8 would apply if the tumors were different histologies and one of the histologies was not listed in the Table. Does that logic still apply if one of the tumors is NSCLC? If NSCLC is excluded from Rule M8, is Rule M14 the appropriate M Rule for the 2022 NSCLC diagnosis? |
The patient's previous acinar adenocarcinoma in the right lower lobe of the lung in 2020 and squamous cell carcinoma in the right middle lobe of the lung in 2021 were correctly abstracted as two primaries per rule M8 as they are in different rows in Table 3. The NSCLC, RUL (8046) diagnosed in 2022 would not be abstracted as a third primary because NSCLC is a broad category which includes all histologies in Table 3 (except for small cell carcinoma/neuroendocrine tumors (NET Tumors) 8041 and all subtypes), and because it was diagnosed less than 3 years after the 2021 squamous cell carcinoma, RML (8070). |
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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20230072 | Solid Tumor Rules/Multiple Primaries--Bladder: How many primaries and what M Rule applies to a diagnosis of non-invasive urothelial carcinoma of the bladder in 1996, followed by multifocal non-invasive papillary urothelial carcinoma involving bladder, prostatic urethra, and left ureter in 2022? See Discussion. |
An argument could be made to apply Rule M10 (timing rule which may result in reporting the case as an additional primary) because the 2022 primary included multiple non-invasive urothelial carcinoma tumors in both the bladder and other urinary sites (coded to site C689, not C679) following a long disease-free interval. While Rule M10 excludes multiple bladder tumors, does that also apply when new, multifocal urothelial tumors arise in both bladder and other urinary sites? Does the presence of any subsequent bladder tumor rule out the use of M10 and one must use M11 that indicates reporting this disease process is a single primary? |
Abstract as a new primary per rule M10, as the subsequent tumors are not limited to the bladder. Code the primary site to C689, per Instructions for Coding Primary Site, #4: "Code Urinary System NOS C689 when there are multiple non-contiguous tumors in multiple organs within the urinary system", and following Note: "The physician subject matter experts (SME) discussed the issue of coding primary site for multifocal/multicentric urinary tract carcinoma. Although the SMEs understood and acknowledged the importance of coding a specific primary site, there is no literature or criteria for determining the organ of origin for multiple tumors involving multiple urinary sites". |
2023 |
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20230038 | Histology/Heme & Lymphoid Neoplasms--Mycosis Fungoides: What is the histology code for lymphomatoid papulosis that transforms initially to mycosis fungoides (MF)/cutaneous T-cell lymphoma (CTCL) and subsequently to CTCL with large cell transformation, and is it a new primary? See Discussion. |
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 |
Abstract a single primary and assign code 9700/3 for MF. According to our subject matter expert, this is all MF. When MF progresses, there can be large cd30 positive T cells. This is not the same as anaplastic large cell lymphoma. |
2023 |
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20230075 | EOD/Summary Stage--Eye: How is stage coded for a patient with extranodal non-Hodgkin lymphoma involving bilateral choroids (single focus, both sites) and no lymph node involvement? Since the eyes are a paired site, is this two separate extranodal sites? If so, there are no Summary Stage or EOD tumor codes that best fit this scenario. |
Assign as Stage IV as recommended by our expert hematological oncologist. This is a rare occurrence and this type of presentation does not fit the definition of intraocular extension. Stage IV is probably the best stage for this type of presentation, since there are two extranodal organs involved, even though they involve a bilateral site. EOD Primary Tumor: 700 SS: 7 (Distant) |
2023 | |
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20230067 | First Course Treatment/Scope of Regional Lymph Node Surgery--Breast: How is Scope of Regional Lymph Node Surgery coded when initially there is a sentinel lymph node biopsy (SLNBx) and an intramammary node removed followed a month later by an axillary dissection for a right breast primary? See Discussion. |
Patient has a diagnosis of invasive carcinoma of the right breast from a core biopsy on 04/2023. Subsequent bilateral mastectomy and sentinel node biopsy proves one positive sentinel node and one negative intramammary node. One month later there is a completion axillary node dissection with 15 nodes negative for malignancy. Per previous SINQ 20190074, the initial mastectomy and sentinel node excision with intramammary node removal should be coded as Scope of Regional Lymph Node Surgery 6. It is unclear how the resulting axillary dissection should be recorded in Scope of Regional Lymph Node Surgery. There is no code for sentinel node biopsy and 3, 4, or 5 at same time (code 6) PLUS an additional subsequent axillary dissection. Please provide coding instructions for Sentinel Lymph Nodes Positive, Sentinel Lymph Nodes Examined, and Scope of Regional Lymph Node Surgery in this scenario. |
Scope of Regional Lymph Node Surgery: Assign code 7, Sentinel node biopsy and code 3, 4, or 5 at different times. In this case, the SLNBx (code 2) preceded the regional node dissection (code 5: 4 or more regional lymph nodes removed), i.e., procedures performed in separate surgical events. Sentinel Lymph Nodes Examined: Assign code 98, Sentinel lymph nodes were biopsied, but the number is unknown. In this case, only the results were provided. Sentinel Lymph Nodes Positive: Assign code 01, Sentinel nodes are positive (code exact number of nodes positive). In this case, there was one positive sentinel node. |
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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20230018 | SEER Manual/First Course Treatment--Chemotherapy: Does the First Course of Treatment end when subcategories change for treatments such as hormone therapy or immunotherapy or is that instruction specific to chemotherapy? See Discussion. |
Treatment for estrogen receptor positive (ER+) breast cancer started with tamoxifen (non-steroidal estrogen subcategory) and switched to letrozole (non-steroidal aromatase inhibitor subcategory). Patient being treated with immunotherapy, Avastin (cytostatic agent-antiangiogenesis agent subcategory), and then changed to Atezolizumab (monoclonal antibody subcategory). Is Atezolizumab a new course of therapy because it is a different subcategory? |
Answer updated April 2025 A change in the subcategory for a hormone drug does not indicate the end of First Course of Treatment because different hormone therapies generally achieve the same result. For example, some forms of breast cancer are estrogen-dependent and the various subcategories of hormone drugs used to treat them, such as gonadotropin-releasing factor agonists, aromatase inhibitors and estrogen antagonists, all achieve the same result - to block estradiol effects in these tumors. Similarly, a change in immunotherapy is not a new course of treatment. However, if a change to hormone therapy or immunotherapy is due to a change in the patient's ER, PR, or Her2 status, this could signify a new course of treatment. The instruction in the SEER Manual is specific to chemotherapy. Chemotherapy is the only systemic treatment for which a change in the subcategory of a drug indicates the end of First Course of Treatment, due to the fact that different chemical agents damage cancer cells in different ways and at different phases in the cell cycle. |
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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