| Report | Question ID | Question | Discussion | Answer | Cancer Site Category | Data Item Category | Year |
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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". |
Bladder | Primary site | 2023 |
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20230071 | Solid Tumor Rules/Histology--Cervix: How is histology coded for a 2023 endocervical adenocarcinoma negative for high-risk human papilloma virus (HR-HPV) on Pap smear and strongly positive for p16 on biopsy? See Discussion. |
The Solid Tumor Rules indicate p16 is a valid test to determine HPV status and can be used to code HPV-associated/-independent. In this case, we do not know whether the HR-HPV test was done on cytologically malignant cells, or on benign cervical cells. It may be impossible to tell unless 100% of the cytology specimen is malignant, but we will not have access to that information. Also, HR-HPV testing is routine on Pap smears, so this testing does not mean the tumor cells specifically harbor HPV. |
Assign histology as adenocarcinoma, HPV-associated (8483/3) as designated in Table 17, Uterine Cervix Histologies, of the Other Sites Solid Tumor Rules. The WHO Classification of Female Genital Tumors, 5th edition, states that p16 immunohistochemistry is an effective (yet flawed) indirect test for HR-HPV infection, in line with the STRs that state p16 is a valid test to determine HPV status and can be used to code HPV-associated and HPV-independent histologies. In this scenario, "negative for high-risk human papilloma virus (HR-HPV) on Pap smear" would be cytology-based, and may have missed cytologically malignant cells. A subsequent, more definitive biopsy was performed and was found to be strongly positive for p16, therefore, the tumor should be coded as 8483/3. |
Cervix Uteri | Histology | 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. |
Breast | Histology | 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. |
Colon | First course treatment, Immunotherapy | 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. |
Thyroid | Histology | 2023 |
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20230060 | Histology--Urinary: How is histology coded for a diagnosis of bladder carcinoma with a mix of different urothelial carcinoma subtypes? See Discussion. |
The 10/2023 TURBT final diagnosis is “Urothelial carcinoma with mixed histologic appearances, see synoptic summary below for details.” The synoptic report includes, “Histologic Type Comment: Invasive carcinoma percentages: Micropapillary 60-70%, high grade or poorly differentiated urothelial 20-30%, squamous 10-20%.” The squamous component is stated to be “Urothelial carcinoma with squamous differentiation.” It appears there are two specific urothelial carcinoma subtypes to consider: Urothelial carcinoma, micropapillary variant (8131/3) and poorly differentiated carcinoma (8020/3). The squamous component would not be considered because there is no specific histology for “squamous differentiation.” The micropapillary component is the predominant histology (60-70%) in this case, and it does seem like this is important to capture. However, the WHO Blue Book indicates poorly differentiated carcinoma of the bladder has a poor prognosis. |
Code histology as urothelial carcinoma, NOS (8120/3). Our subject matter expert advises that WHO Classification of Urinary and Male Genital Tumors, 5th edition, does not recognize mixed urinary histologies; therefore, has not assigned an ICD-O code for urothelial mixed with multiple variants. Only pure variants are coded as they have a different prognosis from those that are mixed. According to WHO, invasive urothelial carcinoma is remarkable for its diversity of morphological appearances and a single lesion can display an admixture of conventional urothelial and various well-defined histological subtypes. |
Bladder | Histology | 2023 |
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20230064 | Primary Site--Cervix Uteri: When no other information is available regarding the origin of the tumor, can an overlapping cervical adenocarcinoma (C538, 8140/3) be coded to the endocervix (C530) based on the histology? See Discussion. |
Adenocarcinoma is a glandular tumor and the endocervix is generally the origin of glandular tissue for the cervix. However, if the only available information is pathology proving a single tumor overlapping the endocervix and exocervix, can we code the site to C530 instead of C538? Applying the current primary site coding instructions, primary site would be coded as C538 because there is no specific statement of the tumor origin; the primary site coding instructions state the tumor is coded to an overlapping site in the absence of a specific statement of origin and there is no existing SINQ confirming the site can be assumed to be the endocervix based on the histology. |
Code Primary Site as Overlapping lesion of cervix uteri (C538). The 2023 SEER Program Coding and Staging Manual Primary Site Coding Instructions for Solid Tumors #4 says to code the last digit of the primary site code to ‘8’ when a single tumor overlaps an adjacent subsite(s) of an organ and the point of origin cannot be determined. This is also supported by the ICD-O-3, 3rd edition, note in the Topography section that states: In categories C00 to C809, neoplasms should be assigned to the subcategory that includes the point of origin of the tumor. A tumor that overlaps the boundaries of two or more subcategories and whose point of origin cannot be determined should be classified to subcategory ‘8.” |
Cervix Uteri | Primary site | 2023 |
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20230077 | EOD 2018/ Primary Site/Heme & Lymphoid Neoplasms--CLL/SLL: How are Primary Site and Extent of Disease (EOD) Primary Tumor coded when a lymph node biopsy proved chronic lymphocytic leukemia (CLL), and the peripheral blood is involved with an “abnormal CD5-positive B-cell population”? See Discussion. |
The patient has adenopathy in multiple lymph node regions above and below the diaphragm and a lymph node biopsy pathology proved CLL/small lymphocytic lymphoma (SLL). Further work-up with peripheral blood proved an abnormal CD5-positive B-cell population comprising only a small percentage of the white blood cells (WBCs). The pathologist noted this neoplastic B-cell population comprises “3.5% of white blood cells and has an immunophenotype characteristic of CLL/SLL and is similar to the recent lymph node biopsy in this patient.” The managing physician indicated this was a Lugano Stage III SLL. The registrar coded the peripheral blood involvement in EOD Primary Tumor. If this small percentage of WBCs with an abnormal B-cell population is included in EOD Primary Tumor as peripheral blood involvement, then this would indicate peripheral blood/bone marrow involvement and primary site would need to be coded to C421 per Rule PH5. Rules PH5 and PH6 confirm primary site must be coded C421 if peripheral blood or bone marrow are involved. Is there a cutoff value for these abnormal B-cell populations in the peripheral blood? Or should these abnormal B-cell populations be ignored unless the pathologist states the abnormal B-cell population is consistent with CLL/SLL (not just immunophenotypically characteristic of CLL/SLL)? |
Primary site would be C421 based on Hematopoietic and Lymphoid Neoplasm Manual, Module 3, Rule PH 5. Assign EOD Primary Tumor to code 800 (peripheral blood involvement WITH other involvement). Per consultation with an expert hematologist oncologist, this is a Stage IV CLL/SLL since the peripheral blood is involved. There is no cutoff value for the abnormal B-cell populations in the peripheral blood when the cells are consistent with CLL/SLL. If the peripheral blood is involved, even only slightly, it is a Stage IV CLL/SLL. Our expert stated that the physician's staging was wrong (this is not a Lugano, Stage III). |
CLL/SLL | EOD Primary Tumor, Primary site | 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._). |
Melanoma, Skin | Histology | 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. |
Brain and CNS | Primary site | 2023 |
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