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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20230019 | Solid Tumor Rules/Multiple Primaries--Pancreas: How many primaries, and what M Rule applies, when a pancreatectomy identified an invasive adenocarcinoma in one pancreatic head tumor, but multiple separate pancreatic neuroendocrine tumors (PanNETs), WHO grade 1, in the pancreatic body? See Discussion. |
There was a 3.5 cm invasive adenocarcinoma tumor in the pancreatic head. There were four separate, sized pancreatic neuroendocrine tumors measuring 0.9, 0.7, 0.5 and 0.2 cm in the pancreatic body. There are multiple tumors with distinctly different histologies. However, Table 11 (Pancreas Histologies) does not include any entries for neuroendocrine tumors of the pancreas (e.g., pancreatic NET, WHO grade 1, histology 8240). While it would seem Rule M19 should apply as they’re distinctly different histologies, because PanNETs are not included in Table 11, it is not clear which M Rule applies to these multiple tumors. If Rule M19 does not apply, we are left with Rule M21 (Abstract a single primary when there are multiple tumors that do not meet any of the above criteria). Are these separate tumors with distinctly different histologies really a single primary? Pancreatic neuroendocrine tumors are not an uncommon histology, is there a reason these were not included in Table 11? |
Abstract two primaries using the 2023 Solid Tumor Rules, Other Sites, Rule M19, as adenocarcinoma and pancreatic neuroendocrine tumors are two distinct histologies. The WHO Classification of Digestive Tumors, 5th ed., Chapter 10-Tumors of the Pancreas, lists both epithelial tumors and neuroendocrine neoplasm as separate entities. The Solid Tumor Rules histology-specific tables contain histologies that commonly occur in the 19 site-specific histology tables; therefore, not all histologies are listed in the rules. Further, the adenocarcinoma would be staged in the Pancreas Schema, while the neuroendocrine tumor would be staged in the NET Pancreas schema. We will consider adding PanNETs to Table 11 in a future release of the Solid Tumor Rules. |
2023 |
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20230023 | Solid Tumor Rules/Multiple Primaries—Brain and CNS: How many primaries are accessioned, and which M Rule applies, to a 2018 pituitary adenoma (8272/0) that was partially resected followed by a 2023 resection of residual disease proving pituitary adenoma/pituitary neuroendocrine tumor (8727/3)? See Discussion. |
The patient had residual tumor following the 2018 transsphenoidal resection and underwent an additional surgery after the residual tumor increased in size. Since pituitary adenoma/pituitary neuroendocrine tumor (PitNET) is a new malignant neoplasm for cases diagnosed 2023 and later, should this be a new primary per M5? Or do we disregard the change in behavior and apply rule M2 (single tumor is a single primary) for this scenario? |
This case does not fall into the standard rules. WHO criteria for diagnosing pituitary adenoma have recently changed (per 5th Ed WHO CNS book) and we will likely see more PitNET’ s than pituitary adenomas in the future. PitNET may be invasive or non-invasive but the likelihood of the pathologists providing this information is low. Since we don’t know if the 2018 adenoma was a PitNET based on current criteria or if it transformed to the malignant neoplasm, err on the side of caution and abstract a second primary per M5. This issue is new, and we’ve received numerous questions concerning pathologist reviewing older cases of pituitary adenoma and reclassifying them as PitNET using the new criteria. |
2023 |
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20230014 | Reportability--Thyroid: Is a case with thyroid fine needle aspirate (FNA) cytology with nodule 1 Bethesda category 5 and nodule 2 Bethesda 6, reportable in 2021? Does the Bethesda category 5 or 6 have any bearing on reportability? |
In the absence of information to the contrary, thyroid FNAs designated as Bethesda classification category VI are reportable. Thyroid FNAs designated as Bethesda classification category V are not reportable unless there is additional information confirming a reportable diagnosis. For both Bethesda V and VI, NCCN Guidelines recommend total thyroidectomy or lobectomy (depending on tumor size and nodal involvement) for the purposes of definitive diagnosis/treatment, so additional information should be available. We will add this to the next version of the SEER manual. In your example, nodule 1 Bethesda V is not reportable. Nodule 2 Bethesda VI is reportable. |
2023 | |
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20230042 | First Course Treatment/Surgery of Primary Site--Rectum: What surgery code should be used for laparoscopic C/T open low anterior resection with colorectal anastomosis, loop ileostomy in diagnosis year 2020, code 30 or 40? See Discussion. |
Can you provide clarification on Rectum primary surgical code 40 Pull through WITH sphincter preservation (colo-anal anastomosis)? Would this be code 30 or 40 due to the colorectal anastomosis? |
Assign code 40, Pull through WITH sphincter preservation (colo-anal anastomosis). The National Cancer Institute Dictionary of Cancer Terms defines coloanal anastomosis as a surgical procedure in which the colon is attached to the anus after the rectum has been removed. It is also called coloanal pull-through. |
2023 |
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20230053 | Reportability/Histology--Ovary/Testis: Is serous borderline tumor-micropapillary variant (8460/2) of the ovary or testis reportable? If so, what dates are applicable to the reportability changes? See Discussion. |
Serous borderline tumor–micropapillary variant (8460/2, C569) was included in the ICD-O-3 Behavior Code/term updates effective 1/1/2018 but marked as Not Reportable for 2018. There have been multiple additional updates to the ICD-O but no further clarification as to the reportability of this histology. ICD-O-3.2 currently lists serous borderline tumor, micropapillary variant (C569) as 8460/2 with no mention of reportability and no information provided in Includes/Excludes. SINQ 20220032 instructs capturing this histology as reportable when diagnosed 1/1/2021 or later and occurring in the testis. The answer indicates this is reportable due to the /2 behavior code in ICD-O-3.2, but it does not specify that it is limited to specific sites. Is serous borderline tumor, micropapillary variant reportable for ovary? If so, what dates apply? Is serous borderline tumor, micropapillary variant of the testis diagnosed after 1/1/2021 reportable? |
Do not report serous borderline tumor–micropapillary variant of the ovary (8460/2, C569) as borderline ovarian tumors are not reportable. This applies to cases 2018 and later. Do report serous borderline tumor–micropapillary variant of the testis as stated in SINQ 20220032. It is reportable for cases diagnosed Jan 1, 2021 and later. |
2023 |
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20230078 | Primary Site/Heme & Lymphoid Neoplasms--CLL/SLL: Should the primary site be coded C421 (bone marrow) for a diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) when the managing physician provides a Rai stage? See Discussion. |
The patient has adenopathy and a lymph node biopsy proved CLL/SLL. The patient underwent a peripheral blood smear, but the final diagnosis only indicated there is an abnormal CLL panel, positive for monoallelic or biallelic deletion of 13q. The pathologist noted a CLL related clone was detected, but there was no definitive diagnosis of CLL on the peripheral blood. No bone marrow biopsy was performed. However, the managing physician noted this was Rai Stage I CLL/SLL with adenopathy in the neck. The SSDI Manual notes, “Rai stage is only applicable for CLL, in which the bone marrow and/or peripheral blood are involved (primary site C421 for bone marrow, see Hematopoietic Manual, Module 3: PH 5, 6).” Should primary site default to C421 if the physician provides a Rai Stage in the absence of definitive peripheral blood or bone marrow involvement documented in the medical record? |
Assign primary site C421. The Site-Specific Data item (SSDI) Manual, Rai Classification section, states: Per confirmation from medical oncologists, Rai stage is only recorded for patients who have bone marrow and/or peripheral blood involvement. Per the Hematopoietic Rules, primary site would be C421 (See Hematopoietic Manual, Module 3: Rules PH 5, 6). A new code has been added to the 5 SSDIs (code 5) to use when primary site is not C421. |
2023 |
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20230048 | Solid Tumor Rules/Histology--Uterine Corpus: How is histology coded for an epithelioid and myxoid leiomyosarcoma of the myometrium? See Discussion. |
Patient had a total abdominal hysterectomy-bilateral salpingo-oophorectomy performed in January 2023 with final diagnosis of myxoid and epithelioid leiomyosarcoma. Diagnosis comment states: The tumor is 15 cm per report. It grows in nests and poorly formed interanastomosing trabeculae and cords that are separated by abundant myxoid background. The cells have an epithelioid morphology with eosinophilic cytoplasm, large nuclei, and very prominent nucleoli. The mitotic activity is overall low ranging from 1 to 3/10 HPFs. Immunohistochemical stains performed at the outside hospital showed diffuse positivity for SMA, desmin, caldesmon, and PR. They are negative for CD10, claudin-4, calretinin, HBM45, MART1 (rare weakly positive cells), PANCK, and SOX10. This immunohistochemical profile supports a smooth muscle derivation of this neoplasm. As this tumor is extensively myxoid, diagnostic criteria differ from the spindle cell leiomyosarcoma. Per Solid Tumor Rules Other Sites, Table 16: Uterine Corpus Histologies, Epithelioid Leiomyosarcoma (8891/3) and Myxoid Leiomyosarcoma (8896/3) are both subtypes of Sarcoma, NOS (8800/3). Per Rule H21, use a combination code when there are multiple specific histologies AND the combination is listed in Table 2 OR there are coding instructions for the combination in the applicable histology Tables 3-21 OR you receive a combination code from Ask A SEER Registrar. Since there is no combination listed in Table 2 and there is no instruction for the combination in Table 16, how should the histology be coded for this tumor? |
Assign code 8891/3 (epithelioid leiomyosarcoma) as cells were described as have an epithelioid morphology; whereas, myxoid was used as a descriptive term and not a specific histologic type. |
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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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 |