Report | Question ID | Question | Discussion | Answer | Year |
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20230022 | Solid Tumor Rules/Multiple Primaries: What M Rule of the updated Solid Tumor Rules, Other Sites, applies to a 2022 diagnosis of endometrial cancer, followed greater than one year later by a 2023 diagnosis of esophageal cancer with no interim evidence of tumor recurrence? See Discussion. |
These diagnoses were made greater than one year apart with a disease-free interval and M12 seems to be the first rule that applies. This rule does not specifically state the tumors diagnosed greater than 1 year apart must be in the same primary site but Note 1 could be interpreted as implying this. Note 1 states, “Clinically disease-free means that there was no evidence of recurrence in the same site on follow-up.” Does Other Sites Rule M12 (the timing rule) apply to tumors in different primary sites? It would be helpful if the notes specified this clarification, such as “Clinically disease-free means that there was no evidence of recurrence in the same site (same second and third character CXX.X) on follow-up.” |
Abstract multiple primaries using the Solid Tumor Rules, Other Sites, Rule M13. The topography differs at the second and third characters (C54.1 Endometrium; C15 Esophagus). Rule M12 refers to being disease-free vs. recurrence of a tumor, where Note 1 states that clinically disease-free means no evidence of recurrence in the same site on follow up. A note can be added to clarify that M12 applies to new tumors in the SAME site. |
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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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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20230037 | Reportability/Histology--Gallbladder: Is intracholecystic papillary-tubular neoplasm (ICPN) with extensive high grade dysplasia of the gallbladder reportable? |
Report intracholecystic papillary neoplasm (ICPN) with high-grade dysplasia (8503/2) of the gallbladder. |
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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20230032 | Reportability/Histology--Thyroid: Is a diagnosis of papillary carcinoma, follicular variant, encapsulated/well demarcated, non-invasive reportable? See Discussion. |
The final diagnosis for a left thyroid lobectomy was Papillary thyroid microcarcinoma, further stated to be Histologic Type: Papillary carcinoma, follicular variant, encapsulated/well demarcated, non-invasive. The diagnosis comment states there is a small follicular pattern papillary microcarcinoma. Is the designation of “non-invasive” for this papillary follicular tumor equivalent to a non-reportable diagnosis of Non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP), 8349/1? Or should this be accessioned as either a reportable in situ (non-invasive) papillary follicular thyroid carcinoma or a papillary microcarcinoma per the diagnosis comment? |
Your case is equivalent to encapsulated follicular variant of papillary thyroid carcinoma, non-invasive (non-invasive EFVPTC) and is not reportable for cases diagnosed in 2021 or later even though it says "carcinoma." That is because the WHO assigned a behavior code of /1 to this entity (8349/1). NIFTP is assigned to the same histology and behavior code. |
2023 |
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20240041 | Reportability--Brain and CNS: Is an optic nerve meningioma reportable if stated to arise in the “intraorbital segment” of the optic nerve meninges? See Discussion. |
Patient was diagnosed on imaging with enhancement along the right optic nerve intraorbital segment, displacing the optic nerve, most consistent with optic nerve sheath meningioma. Extracranial meningiomas are rare, however SINQ 20230052 does contain an exception for reportability in a different head and neck site because it is not an intracranial location. It is unclear if this portion of the meninges surrounding the intraorbital optic nerve is still “intracranial” and thus reportable. |
Report optic nerve sheath meningioma arising in the intraorbital segment. The optic nerve contains four segments, of which intraorbital is one. The WHO Classification of Eye Tumors, 4th edition, defines meningioma as a neoplasm originating from the meningothelial cells of the optic nerve leptomeninges. According to the Table 3 of the Non-malignant Solid Tumor Rules, all portions of the optic are reportable and meningiomas arising in the dura/meninges of an intracranial nerve are coded to cerebral meninges C700. |
2024 |
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20240023 | Solid Tumor Rules/Histology--Penis: Why is warty carcinoma listed in Other Sites, Table 23 (Penis and Scrotum Histologies) as 8051 when the ICD-O-3.2 and SINQ 20200003 indicate the correct histology is 8054 for this neoplasm? See Discussion. |
The ICD-O-3.2 indicates histology 8051 only applies to diagnoses of condylomatous carcinoma and warty carcinoma made prior to 2018. For penis cases diagnosed 2018 and later, these neoplasms should be coded as 8054. This is consistent with SINQ 20200003. However, a new Table was added to the Other Sites schema in the 2024 Solid Tumor Rules update. Table 23 lists “Verrucous carcinoma / carcinoma cuniculatum / Warty carcinoma” as histology 8051. While verrucous carcinoma is still listed under histology 8051 in the ICD-O-3.2, warty carcinoma is not. Does Table 23 need to be updated? Or is this an error in both the ICD-O-3.2 and SINQ 20200003? |
Assign histology code 8054/3 for warty carcinoma. Assign 8051/3 for verrucous carcinoma and carcinoma cuniulatum. The WHO Classification of Urinary and Male Genital Tumors, 5th edition (2022) revised the terminology for squamous cell carcinoma groupings from "non-HPV-related" to "HPV-independent" and from "HPV-related to "HPV-associated". Warty carcinoma is defined as a "morphologically distinct HPV-associated verruciform neoplasm that shares histological features with a giant condyloma but has definitive cytological atypia and a malignant infiltrative architecture." Verrucous carcinoma (including carcinoma cuniculatum) is defined as an HPV-independent squamous cell carcinoma, and is correctly coded to 8051/3. The 2024 Solid Tumor Rules, Table 23, Penis and Scrotum Histologies will be updated to reflect this revised terminology and coding. |
2024 |
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20240015 | Solid Tumor Rules/Histology--Breast: Is ductal carcinoma in situ (DCIS), solid type coded as 8500/2 or 8230/2? See Discussion. |
In the NAACCR Coding Pitfalls 2023 webinar, the example of DCIS, solid type is given. The webinar advised us to code 8230/2 (ductal carcinoma in situ, solid type). When going through the beginning of the solid tumor rules in the Changes from 2007 MPH Rules section it states "DCIS/Carcinoma NST in situ has a major classification change. Subtypes/variant, architecture, pattern, and features ARE NOT CODED. The majority of in situ tumors will be coded to DCIS 8500/2." In the equivalent or equal terms section it lists "Type, subtype, variant" can be used interchangeably. Since the example has it listed as as ductal carcinoma in situ, solid "type," would we code 8500/2 or 8230/2? |
Assign 8230/2 (ductal carcinoma in situ, solid type/intraductal carcinoma, solid type) using Breast Solid Tumor Rules Table 3 as instructed in Rule H2 for in situ tumors. The carcinoma, NST row lists this histology in the subtype/variant column 3. Coding histology for in situ breast tumor differs from invasive. While the majority of in situ breast primaries will be coded to DCIS 8500/2, there are others that are listed in Table 3 that should be coded according to the specific histology. Some codes have the word subtype or type as part of their histologic term so these can be coded based on the histologic term as listed in the table. We suggest you routinely review the histology tables to see if a term is listed. |
2024 |
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20240056 | Reportability/Histology--Heme & Lymphoid Neoplasms: How should this unusual 2023 pathology-only case be reported and coded for leukemia cutis? See Discussion. |
10/25/2023: Patient presents to dermatology office with a questionable drug eruption having 3 weeks of papular eruptions of Trunk (Left Chest). Punch biopsies were taken that came back as immature hemopoietic infiltrate with monocytic differentiation. Comment: Myelodysplastic syndrome and leukemia cutis are possibilities. Addendum Report: Additional stains were prepared. ERG is strongly positive. CD1a and S100 do not stain the atypical cells.The controls stain appropriately. CD123 perform with appropriate control is also negative. The pattern is that of so-called "leukemia cutis" which could be seen in the clinical setting of myelodysplasia, chronic myelomonocytic leukemia (CMML) or precursor to acute myelomonocytic leukemia (AMML). Recommend work up. The only available information at present is a diagnosis of leukemia cutis, and that there was no prior history of a hematological malignancy in this patient. |
Report this case of leukemia cutis and code to bone marrow (C421) and leukemia NOS (9800/3) based on the information provided. Update the abstract if new information becomes available. Leukemia cutis is the rare infiltration of neoplastic leukocytes into the epidermis, dermis, or subcutis from an existing leukemia that results in clinically identifiable cutaneous lesions. Leukemia cutis may precede, follow, or occur concurrently with the diagnosis of systemic leukemia. It is an advanced phase of the leukemia having a poor prognosis that also strongly correlates with additional sites of extramedullary involvement. This can alter the appropriate treatment regimen for a patient. It is a type of "metastasis" or spread of the leukemia cells. The "conventional" definition for leukemia cutis is the infiltration of skin from a bone marrow primary. It is most often diagnosed via skin biopsy—punch, shave, etc., utilizing IHC/biomarker testing and is commonly associated with CMML and acute myeloid leukemia (AML). As such, it a reportable condition especially when preceding a confirmed systemic leukemia diagnosis. In this situation, the diagnosis date would be the date of the positive leukemia cutis skin bx—punch, shave, etc. The case should be coded to C421; 9800/3 Leukemia NOS until the official systemic leukemia diagnosis is rendered. If possible, follow back should be conducted to determine the specific systemic leukemia histology (CMML; AML) and the treatment received. If the leukemia cutis follows or occurs concurrently with the diagnosis of a systemic leukemia, it is NOT a separate primary but merely an advanced stage of the systemic leukemia diagnosis. |
2024 |