Report | Question ID | Question | Discussion | Answer | Year |
---|---|---|---|---|---|
|
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 |
|
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 |
|
20230059 | Histology--Heme and Lymphoid Neoplasms: How is histology coded for a diagnosis stated as MDS/AML (myelodysplastic syndrome/acute myeloid leukemia) per the international consensus classification (ICC)? See Discussion. |
The final diagnosis on bone marrow biopsy was high grade myeloid stem cell neoplasm, 17% blasts by differential count. The pathologist further states that this could be classified as “MDS with increased blasts (MDS-IB2) per the WHO 5th edition classification, or MDS/AML per the international consensus classification (ICC).” FISH and cytogenetics revealed a loss of 7q, but no other AML-related genetic abnormalities. The physician confirms the patient has MDS/AML. |
Updated Answer July 2024 Code histology as myelodysplastic neoplasm with increased blasts (9983/3) based on the WHO Classification of Hematolymphoid Tumors, 5th edition, Beta version 2. WHO lists MDS with increased blasts-2 (MDS-IB2) as a subtype of 9983/3. Terms coded to 9983/3 include
When differences exist between WHO and ICC, assign the histology based on the WHO Classification. |
2023 |
|
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 |
|
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. |
2023 |
|
20230023 | Solid Tumor Rules/Multiple Primaries—Brain and CNS: How many primaries are accessioned, and which M Rule applies, to a 2018 of 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 |
|
20230061 | EOD (2018)/EOD Primary Tumor--Prostate: How is Extent of Disease (EOD) Prostate Pathologic Extension coded when no residual cancer is found? See Discussion. |
Patient was diagnosed with a pT1c prostate cancer in 2022. Patient was then treated with radical prostatectomy. No residual disease was found. Would the correct EOD prostate path extension code be 999 based on Note 8 (code 999 when radical prostatectomy is performed, but there is no information on the extension); or, would we use code 300 (confined to prostate) because the data item "…is used to assign pT category for prostate cancer based on radical prostatectomy specimens" and we know it was limited to the prostate because no residual was found? |
Assign code 300 for EOD Prostate Pathologic Extension. In this scenario, the patient has a localized cancer confirmed by radical prostatectomy; the needle core biopsies likely removed all the cancer. Unlike prostate, other sites’ extension information is collected in EOD Primary Tumor, as seen commonly with breast tumors where the results from the surgical resection are recorded with tumor confined to primary site. |
2023 |
|
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 |
|
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.” |
2023 |
|
20230057 | EOD (2018)/EOD Regional Nodes--Thyroid: How is Extent of Disease (EOD) Regional Nodes coded for thyroid primary with cervical lymph nodes containing psammomatous calcifications (psammoma bodies) but negative for metastatic tumor cells? See Discussion. |
The AJCC 8th edition confirms that the identification of psammomatous calcifications within a cervical lymph node is metastatic disease. Example: Patient had a thyroid lobectomy and level VI neck node excision in August 2022. The final diagnosis is multifocal papillary carcinoma of the thyroid, as well as rare psammomatous calcifications only in the resected node. The pathologist notes that “psammoma bodies only” in lymph nodes is not well defined, and while indolent, they do indicate capacity for lymphatic spread and are pN1a. Should thyroid primaries with cervical node psammomatous calcifications get captured in EOD Regional Nodes category as it is in the AJCC pN staging? |
Assign EOD Regional Nodes code 300 for Psammoma bodies within a cervical lymph node that are microscopically confirmed. A clarifying note for the Thyroid Schema will be included in the 2025 EOD updates. |
2023 |