Report | Question ID | Question | Discussion | Answer | Year |
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20210024 | Primary Site--Vulva: What is the primary site of patient with an excision of a left vulvar cystic mass showing focal mammary-type ductal carcinoma in situ (DCIS) on 11/06/2020? See Discussion. |
Final Pathologic Diagnosis: Vulvar cyst, excision: Focal mammary-type ductal carcinoma in situ, intermediate grade, arising within cystically dilated duct (See Comment) Size of DCIS: 0.7 CM. Margins: Negative. Comment Sections demonstrate a cystically dilated duct. Focally, at the periphery of the duct, there is a neoplastic monomorphic proliferation of ductal cells with intermediate grade nuclei. No associated necrosis is identified. Immunostains for GATA-3 and estrogen receptor are strongly positive within the neoplastic cells, supporting origin from mammary-like epithelium. Immunostain for p63 demonstrates preservation of a basal layer around the dilated duct, including the region involved by DCIS. Immunostain for cytokeratin 5/6 shows loss of expression within the DCIS. No stromal invasion is identified. The cyst appears to be completely excised. 12/01/2020 post op visit with surgeon: Ductal carcinoma in situ (DCIS) of the left vulva in an excised cystic lesion. PLAN: I reviewed the pathologic findings from the excision of the left vulvar cyst. This appears to be a cystic lesion in the mammary line with focal DCIS. It was excised completely with negative margins. It would not warrant any additional treatment except expectant management. |
Code the primary site to vulva. Use text fields to record the details. According to the WHO classification, several types of primary vulvar mammary-like carcinoma have been reported. It is rare and is thought to arise from specialized anogenital mammary-like glands within the vulva. It does not arise from ectopic breast tissue and is does not represent metastatic breast carcinoma. |
2021 |
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20210008 | Solid Tumor Rules (2018, 2021)/Histology--Melanoma: In what situation will Rule H6 be used to code the histology to lentigo maligna melanoma? See Discussion. |
Rule H6 states: Code 8742/3 (Lentigo maligna melanoma) when the diagnosis is lentigo maligna melanoma with no other histologic types. However, if the diagnosis was strictly lentigo maligna or lentigo maligna melanoma, the first rule that applies is Rule H1 because lentigo maligna melanoma is a single, specific histologic type and Rule H1 states, Code the histology when only one histologic type is identified. Following the current rules, one would never arrive at Rule H6. Should the H Rules be reordered? Or should an example of when one would use Rule H6 be added to clarify when to use this rule? |
Solid Tumor rule H6 is the same as MP/H rule H8. We found registrars have problems understanding reportable terminology and the corresponding ICD-O-3 histology code for lentigo maligna melanoma. It is included in H6 to capture cases where the registrar may not stop at H1. We will add another note to H1 instructing users to continue through the rules if the diagnosis is lentigo maligna melanoma. |
2021 |
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20210078 | Solid Tumor Rules (2018/2021)/Multiple Primaries--Skin Cancer: How many primaries are assigned for sebaceous carcinomas using the Solid Tumor/Multiple Primaries/Histology Rules? Does this scenario represent eight separate primaries? See Discussion. |
Details 4/15/2018: Right abdominal wall mass excision: infiltrating sebaceous carcinoma. Noted to have a history of Muir-Torre/Lynch syndrome. 1/21/2019: Two left upper back mass excisions and two lower back (laterality not specified) mass excisions: infiltrating sebaceous carcinomas 8/7/2019: Excision of multiple sebaceous carcinomas from the right posterior back, left posterior thigh, left anterior abdominal wall, left anterior thigh, right scrotum, right lower abdominal fold, all positive for sebaceous carcinoma on pathology report 9/30/2020: Right gluteal mass, left gluteal mass, back (NOS) excisions: sebaceous carcinomas. 10/14/2020: Right back excision: sebaceous carcinoma. Op note: History of Lynch syndrome with multiple sebaceous carcinomas, recurrent back mass, site of prior mass resection. 10/18/2021: Right thigh excision: sebaceous carcinoma Proposed primaries using MP/H Other Sites Rules #1: 4/15/2018: C445-1 #2: 1/21/2019: C445-2, separate from #1 per M8, same as 1/21/19 C445-9 per M18 #3: 8/7/2019: C445-1, separate from #1 per M10, separate from #2 per M8 #4: 8/7/2019: C447-2, separate from #1 & #3 per M8, separate from #2 per M12 #5: 8/7/2019: C632, separate from #1 per M10, separate from #2-#4 per M11 #6: 9/30/2020: C445-2, separate from #1 & #3 per M8, separate from #2, #4 & #5 per M10 #7: 9/30/2020: C445-1, separate from #2, #4 & #6 per M8, separate from #1, #3 & #5 per M10; I do not think the back, NOS (C445-9) is a new primary per M18. #8: 10/18/2021: C447-1, separate from #2, #4 & #6 per M8, separate from #1, #3, #5 & #7 per M10 |
Assign the number of primaries following the Other Sites Solid Tumor Rules. Based on sites, laterality and or timing there are 8 primaries. This is similar to SINQ 20061112 that advised to follow the Multiple Primaries/Histology rules for sebaceous carcinoma. According to the WHO Classification of Skin Tumors, 5th edition, there is a 30-40% risk of local tumor recurrence, and 20-25% risk of distant metastasis. In only one instance did a physician refer this as a recurrence in the available notes. |
2021 |
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20210019 | Reportability/Histology--Cervix: Is a stratified mucin-producing intraepithelial lesion (SMILE) lesion reportable? Is it reportable if it is invasive SMILE? What is the correct histology? See Discussion. |
Cervix, loop electrosurgical excision procedure: Cervix at transformation zone with stratified mucin-producing intraepithelial lesion (SMILE). SMILE is present at the ectocervical margin. An immunohistochemical stain* for p16 demonstrates strong, diffuse positivity in the lesional epithelium. A mucicarmine stain is also positive in the lesional epithelium, supporting the diagnosis of SMILE. |
Stratified mucin-producing intraepithelial lesion (SMILE) of the cervix is not reportable. SMILE is a variant of adenocarcinoma in situ and is coded 8140/2. In situ neoplasms of the cervix are not reportable. According to the WHO Classification of tumors, p16 is positive and there is a high Ki-67 proliferation index. If SMILE is stated to be invasive, it is reportable, as any other invasive cervical malignancy would be reportable. |
2021 |
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20210014 | Solid Tumor Rules (2018, 2021)/Multiple Primaries--Lung: How many primaries should be reported for a 4/2019 diagnosis of left upper lobe (LUL) adenosquamous carcinoma (left lingula mass biopsy: adenosquamous carcinoma; LUL lung biopsy: pulmonary adenocarcinoma, stated to be a collision tumor and single primary per the Tumor Board), treated with radiation followed by an enlarging LUL mass in 7/2020 found to be squamous cell carcinoma? See Discussion. |
The physician stated the prior LUL adenosquamous carcinoma was PD-L1 negative and the LUL squamous cell carcinoma is PD-L1 positive and is calling it a new primary. 5/22-7/3/19 6000x30 IMRT Photons LUL lung Chemo refused Not a Surg candidate 10/01/2019 CT Chest: IMP: In comparison to CT chest 03/06/2019 and PET/CT 03/21/2019, left lingular mass has mildly decreased in size. Left apical anterior and posterior lung lesions more anterior lesion appears slightly increased in size, the other slight decreased in size, with adjacent areas of atelectasis and scarring. 06/23/2020 CT Chest: MP: In comparison to CT chest 10/1/2019, left lingular mass has increased in size concerning for increasing tumor with adjacent thicker focal pleural thickening involving the chest wall, concerning for possible chest wall invasion. Left apical anterior and posterior lung lesions appears more solid in appearance, representing known adeno CA, given that the appearance has changed, is concerning for residual tumor. 07/06/2020 PET: Hypermetabolic lingular mass and peripheral nodularity has increased in size and FDG avidity on the prior PET/CT. Left apical nodular opacity is difficult to separate from fairly uniform mild left apical pleural hypermetabolism which may be treatment related and/or neoplastic. |
Abstract two primaries: 8560 and 8140 using rule M6. One of the original tumors with adenosquamous now shows only residual SCC following XRT. PD-L-1 is not used to determine multiple primaries. Assuming three tumors (the post-XRT SCC is not a new tumor but residual from one of the adenosquamous tumors) there are two primaries: 8560 and 8140 per M6. For collision tumors, each histology identified in the tumor is used to determine multiple primaries. |
2021 |
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20210068 | Mets at Diagnosis Fields/Primary Site--Lymph Nodes: How are the Mets at Diagnosis fields coded when the metastatic adenocarcinoma involves only one lymph node area and the primary site is unknown? See Discussion. |
In 2018, patient has lymph node metastasis confined to left retroperitoneal area; core biopsy was done which showed metastatic adenocarcinoma, unknown primary site. There are no other sites of disease found. Should I code Mets at Diagnosis--Distant Lymph Node(s) as 1, and the others such as bone and lung as 0? |
In a situation like this with one area of metastatic involvement and an unknown primary, if there is no further information, we advise that the metastasis are "regional" until/unless proven otherwise. With this in mind, code the Mets at Diagnosis fields as 0, including the Mets at Diagnosis--Distant Lymph Node(s). This case should continue to be worked up to identify the primary site. If a primary site is identified later, update the abstract accordingly. In the meantime, use text fields to describe the situation. |
2021 |
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20210016 | Solid Tumor Rules (2018, 2021)/Histology--Kidney: What is the correct histology code for a kidney primary described as clear cell papillary renal cell carcinoma"? Should we use H2 and code 8312/3 or H3 and code 8323/3? |
Assign 8323/3, clear cell papillary renal cell carcinoma using the 2018 Kidney Solid Tumor Rules, Rule H1, as this is a single histology, a variant of renal cell carcinoma NOS. |
2021 | |
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20210021 | EOD 2018/Lymph Nodes-EOD--Breast: Should Extent of Disease (EOD) Regional Nodes be coded as 150 (Clinical assessment only; Positive needle core biopsy/fine needle aspirate [FNA]) when the patient has a biopsy-proven, clinically apparent, movable ipsilateral axillary lymph node, but no evidence of involvement at surgery after neoadjuvant therapy? See Discussion. |
The Breast EOD Regional Nodes notes contain new clarification regarding the clinical assessment vs. pathological assessment codes, but the new Note 2 does not specifically indicate an exception for neoadjuvant therapy. However, if the pre-treatment lymph node core biopsy proved cN1 disease, and the post-treatment resection proved ypN0 disease, should the clinical assessment code (code 150) have priority over any pathological assessment code (including 200) since the involved lymph node was only clinically positive and not pathologically positive? Should an exception be added to Note 2 to address cases where neoadjuvant therapy is given, but the clinical assessment is greater than the pathological assessment? |
The clinical assessment code takes priority over the pathological assessment code in this case because the clinical assessment was worse than the pathologic assessment. Although there was a pathological assessment, the clinical assessment is greater. According to the general coding guidelines for neoadjuvant therapy, code the worst information, which in this case is the clinical assessment. The 2018 EOD General Instructions for EOD Regionals Nodes, instruction #4, addresses neoadjuvant therapy as follows. Neoadjuvant (preoperative) therapy: If the patient receives neoadjuvant (preoperative) systemic therapy (chemotherapy, immunotherapy) or radiation therapy, code the clinical information if that is the most extensive lymph node involvement documented. A new note is being included for the 2022 updates. Exception: If patient has neoadjuvant therapy, and the clinical assessment is greater than the pathological assessment, the clinical assessment code takes priority. |
2021 |
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20210012 | Solid Tumor Rules (2018, 2021/Multiple Primaries/)--Lung: How many primaries should be reported and what M rule applies when a diagnosis of presumed adenocarcinoma in situ (AIS) of the left lung follows a known diagnosis of progressive multifocal malignant adenocarcinoma in the right lung? See Discussion. |
Patient was initially diagnosed with a right lower lobe (RLL) lung adenocarcinoma in 2014 followed by subsequent right upper lobe (RUL) lung adenocarcinoma in 2016 (single primary). Both were treated with radiation and the nodules were seen as stable on surveillance. There was subsequent growth in the RUL nodule in 2019 and RLL nodule in 2020 as well as a new right middle lobe (RML) nodule in 2020. All left sided nodules were noted to be stable and/or ground glass opacities. There was no documented diagnosis of malignancy in the left lung until June 2020 when the physician noted that if there was a response in the left lung to systemic treatment, then this was probably multifocal AIS. However, only one tumor in the left lung responded to treatment. While it seems somewhat unlikely that only a single AIS in the contralateral lung should be metastasis from the right lung malignancy, it is difficult to apply the multiple tumors rules to this case. |
Abstract a single primary using 2018 Lung Solid Tumor Rule M9. The 2014 and 2016 R lung tumors were pathologically confirmed; it is not stated if they were resected. Follow up after XRT noted stable disease but no indication of NED. Subsequent right lung tumor is also the same primary. The issue is the assumed left lung adenocarcinoma in situ. It is not clear how long the left lung nodules were present, but they appeared to be stable as well and only diagnosed as a malignancy based on treatment response. At this time M9 applies and the left lung AIS is not a separate primary. We have discussed at length with lung pathology experts the issue of determining multiple primaries. Identifying and diagnosing lung tumors has become easier with new technology and the result is patients are being diagnosed with multiple lung tumors. Some lung experts feel we are under-reporting lung primaries but all noted the many issues with creating rules for consistency. |
2021 |
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20210003 | Solid Tumor Rules (2018)/Primary Site--Head & Neck: The instructions for Table 9 of the Head and Neck Solid Tumor Rules instruct registrars to code the primary site to C479 (Autonomic nervous system) for paragangliomas that arise in the head and neck region, but the ICD-O-3.2 provides a site-associated code for most of these tumors (C754, Carotid body and C755, Paraganglion). Which primary site is correct? See Discussion. |
While we recognize that paragangliomas originate in the parasympathetic or sympathetic nervous system, these are endocrine tumors and endocrine glands/structures are not included in ICD-O site code C479 (Autonomic nervous system). Endocrine tumors of the paraganglia have their own site codes (C75_) per the ICD-O. Additionally, the ICD-O-3.2 provides specific sites for most of the paragangliomas included in Table 9. Per the ICD-O-3.2, carotid body paraganglioma is C754, and middle ear paraganglioma, glomus jugulare tumor, jugulotympanic paraganglioma, and paraganglioma (NOS) are C755. Why are paragangliomas not coded to the paraganglia sites (C75_) provided in the ICD-O? Should these sites be added to the Head & Neck schema for the specific paragangliomas arising in the head and neck? Obtaining consistency in coding primary site for these tumors will be difficult if registrars use the ICD-O provided site codes instead of the primary site statement preceding Table 9. Additionally, as most registrars may use the ICD-O provided site code, the Head and Neck schema in the Solid Tumor Rules would not apply, the Other Sites schema would apply to sites C754 and C755. |
Always code primary site to the site of origin. Look for information about where the neoplasm originated. Primary site should always be coded to reflect the site of origin according to the medical opinion on the case. Always code the primary site based on where the tumor arose / site of origin. Site of origin may be indicated by terms such as "tumor arose from," "tumor originated in," or similar statements. Refer to ICD-O-3.2 and ICD-O-3 for topographty codes that are associated with specific histologies whenthe medical documentation does not specify the primary site. |
2021 |