| Report | Question ID | Question | Discussion | Answer | Year |
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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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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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20230027 | Solid Tumor Rules/Multiple Primaries--Peripheral Nerves: How many primaries should be abstracted, and which M Rule applies, when a malignant peripheral nerve sheath tumor (MPNST) in the right arm (C471) is followed greater than one year later by a separate malignant peripheral nerve sheath tumor of the thoracic chest wall (C473)? See Discussion. |
Since the peripheral nerves are included in the Malignant CNS schema of the Solid Tumor Rules, neither the differences in subsite nor timing indicate these are separate primaries (Rule M10 indicates a single primary). However, these are separate MPNSTs in different sites and the tumors are not stated to be metastasis. Additionally, these are treated as separate primaries by the managing physician. While the malignant CNS tumors do not take timing into account, is this correct for these peripheral nerve tumors that are often treated similarly to soft tissue tumors? Should Rule M8 be updated to include tumors in different peripheral nerve subsites? |
Abstract a single primary using Solid Tumor Rules, Malignant CNS and Peripheral Nerves, Rule M10 based on the information provided. Rule M10 applies as both non-contiguous tumors are of the same histology; i.e., on the same row in Table 3. As MPNST can arise in many sites, look for information about the precise location and tissue type in which the tumor arose. For example, if the tumors are stated to arise in soft tissue, follow the Multiple Primary Rules for Other Sites. Both WHO Classification of Central Nervous System Tumors and WHO Classification of Soft Tissue and Bone Tumors state that MPNST is a malignant spindle cell tumor often arising from a peripheral nerve, from a pre-existing benign nerve sheath tumor, or in a patient with neurofibromatosis type 1 (NF1). Future updates will move C470-C479 from CNS to other sites module. |
2023 |
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20230054 | Reportability/Histology--Pancreas: According to SINQ 20140058, solid pseudopapillary neoplasm of the pancreas is reportable (as of 2014). However, per ICD-O-3.2, this histology is not reportable until 2021+. Please clarify which is correct and clearly state the timeframe that it was reportable or not reportable. |
Solid pseudopapillary neoplasm of the pancreas is reportable for cases diagnosed in 2014 and later. Report solid pseudopapillary neoplasm of the pancreas (8452/3) as the guidance in SINQ 20140058 is still in effect. The 4th and 5th editions of the WHO Classification of Tumors of the digestive system define solid pseudopapillary neoplasm of the pancreas as a low-grade malignant pancreatic tumor. |
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 |
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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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20230029 | Primary Site--Skin: Are perianal skin primaries within 5 cm of the anus coded as perianal skin (C44.5) or anus (C21.0). See Discussion. |
ICD-O-3 tells us that perianal skin is C445 and we do not capture basal or squamous cell skin cancers in our registry. The AJCC manual stages perianal skin cancers within 5 cm of the anus with the anus chapter. We cannot AJCC stage them as an anus if we are not capturing them as C445. I realize we do not code a site in order to stage. We have been following the reportability rules and not capturing. Is this correct? I do not see this addressed in the new Other Sites Solid Tumor Rules. |
Code primary site based on the site of origin as determined by the physicians. If the physicians state the site of origin is anus, code anus; the same as with skin. As you state, squamous cell cancer of sites coded to C44 is not reportable. The AJCC instruction for physicians to stage perianal neoplasms within 5 cm of the anus using the Anus chapter does not change cancer registry instructions for coding primary site, nor does it affect cancer registry reportability instructions. |
2023 |
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20230001 | Solid Tumor Rules/Multiple Primaries--Lung: How many primaries should be reported when two separate squamous cell carcinoma (SCC) tumors, one in the left upper lobe (LUL) and one in the right lower lobe (RLL), are diagnosed? The tumors are separated by an interval occurring right hilar lymph node biopsy proving metastatic pulmonary adenocarcinoma without a clear description of a corresponding interval occurring lung tumor. See Discussion. |
The patient was diagnosed with a biopsy-proven 12/2020 LUL SCC treated with radiation only, followed by a right hilar lymph node biopsy in 07/2022, that proved “metastatic pulmonary adenocarcinoma” per pathology and treated with radiation, followed by a biopsy-proven 12/2022 RLL SCC to be treated with immunotherapy only. The imaging never definitively identified a lung tumor that can be assumed to be a primary adenocarcinoma tumor. In 06/2022, a PET scan only described a “strongly PET positive Rt inferior hilar LN vs infrahilar pulmonary mass,” as well as the subsequently biopsy-proven SCC in the RLL (12/2022 SCC primary). The biopsy path indicates this was a right hilar lymph node metastasis and does not indicate this is an infrahilar pulmonary mass. No other PET positive pulmonary lesions were seen at the time. The oncologist’s assessment indicates the right hilar node was the only positive finding on the biopsy, and it was unclear if this right hilar node metastasis was from the left lung or if the primary was “not detectable.” The oncologist summarized this as a LUL lung lesion radiated for SCC, a right hilar lesion radiated for adenocarcinoma, and a RLL lung lesion on pathology found to be SCC. Should the interval occurring metastatic adenocarcinoma be accessioned as a separate lung, NOS primary based on the histology difference? While the Solid Tumor Rules do not apply to metastasis, the oncologist did treat these three malignancies separately and does not indicate the hilar lymph node metastasis was felt to be from either SCC primary. |
Abstract three primaries based on this scenario. 1 – 2020, SCC LUL lung 2 – 2022, Adenocarcinoma lung, described as metastatic pulmonary, based on biopsy of right hilar node (Rule M8) 3 – 2022, SCC RLL lung (Rule M11) |
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). |
2023 |
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20230004 | SEER Manual/Laterality--Kaposi Sarcoma: If both arms are involved with Kaposi sarcoma and no other sites, how is laterality coded? See Discussion. |
Per Solid Tumor Manual Other Sites Rule M6, despite the number of areas of involvement, any presentation of Kaposi sarcoma is always a single primary. The primary site is skin using the Kaposi Sarcoma for All Sites Coding Guidelines (Appendix C, 2023 SEER Manual). Does SEER Program Coding and Staging Manual Laterality Coding Instruction #4 preclude the use of code 4 [Bilateral involvement at time of diagnosis...] if a patient presents with KS involvement of only both arms or only both sides of the face? |
Assign Laterality code 4 (Bilateral involvement at time of diagnosis, lateral origin unknown for a single primary) in the situations you describe. Skin of upper limb and shoulder and Skin of other and unspecific parts of the face are listed as paired organs in the table Sites for Which Laterality Must Be Recorded In the 2023 SEER Manual. |
2023 |
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