Report | Question ID | Question | Discussion | Answer | Year |
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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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20210053 | Reportability/Heme & Lymphoid Neoplasms: Is ALK positive (ALK+) histiocytosis involving the bone marrow and kidney reportable? See Discussion. |
2021 Bone marrow biopsy showed erythroid hyperplasia, increased histiocytes with hemophagocytosis and Factor XIIIa positive histocytic cells. Moderate cytoplasmic staining for ALK 1, consistent with bone marrow involvement of ALK-positive histiocytosis. A subsequent kidney lesion biopsy was also found to have ALK-positive histiocytosis. The patient was then treated with clofarabine. Patient is 3 years old. 07/2020-Chart indicates patient presented in June with fevers and refusing to walk with pancytopenia, bone marrow biopsy showed no leukemia buthistiocytes. Impression: ALK positive histiocytosis involving BM and kidney. 10/2020 Bone marrow final diagnosis states right and left bone marrow aspirates and biopsies: No morphologic or immunohistochemical evidence of involvement by the patient's previously diagnosed ALK+ histiocytosis (see Comments) - Multiple histiocytic collections with prominent hemosiderin; favor reactive - background normocellular bone marrow with maturing trilineage hematopoiesis. The patient's prior bone marrow samples are reviewed (9/2020 and 7/2020). Similar to the September bone marrow sample, the current marrow shows numerous histiocyte collections with abundant associated hemosiderin deposition. These histiocytes have a stellate/dendritic appearance and lack the atypical features noted in the patient's marrow at diagnosis, favoring a reactive process. This impression is further supported by the lack of immunoreactivity for either Factor XIIIa or ALK1 among these cells. There is no convincing morphologic or immunohistochemical evidence of marrow involvement by the patient's previously diagnosed ALK+ histiocytosis within the sampled material. Of note, the marrow otherwise appears normocellular for the patient's age, indicative of ongoing marrow recovery post therapy. It is not clear whether this would be equivalent to Langerhans cell histiocytosis, disseminated (9751/3) as there is not a statement of Langerhans cell or whether this is just histiocytosis, NOS and not reportable. |
Do not report this case of histiocytosis. Based on the information provided, this case is not reportable. |
2021 |
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20210040 | Solid Tumor Rules (2018, 2021)/Histology--Breast: How is histology coded for a diagnosis ofmixed mucinous carcinoma? See Discussion. |
Patient was diagnosed with invasive ductal carcinoma with mucinous features in February 2021, left breast biopsies at 2:00 (3 cm from nipple) and 3:00 (8 cm from nipple) positions. Intraductal component was absent in those specimens. Subsequent left breast total mastectomy in March 2021, provided a final diagnosis of multifocal mixed mucinous carcinoma, grade 1, 27 and 8 mm and ductal carcinoma in situ (DCIS), low to intermediate grade, with focal mucinous features. The Staging Summary lists Histologic Type as mixed mucinous carcinomafor both foci of invasive carcinoma. DCIS is also noted as present negative for extensive intraductal component. There does not appear to be a clear instruction or code for this histology. As a central registry, we are unable to follow-up with the pathologist regarding this diagnosis. Just to be clear, there are 2 foci of invasive mixed mucinous carcinoma in this case measuring 27 mm and 8 mm. The DCIS component measured 56 mm. |
If the DCIS is separate from the mucinous, apply the breast M rules and abstract TWO primaries per M14. Since all we know of the “mixed mucinous” is there is mucinous present, code 8480/3. |
2021 |
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20210034 | Reportability/Histology--Endometrium: Is endometrial hyperplasia with atypia equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable? |
Endometrial hyperplasia with atypia is equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable for cases diagnosed 2021 and later. Our expert pathologist consultant confirmed this for us. |
2021 | |
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20210041 | Reportability/Behavior--Paraganglia: Is a 2021+ diagnosis of paraganglioma reportable if the grading of adrenal pheochromocytoma and paraganglioma (GAPP) score falls outside the stated requirements for malignancy? See Discussion. |
Patient was diagnosed with a retroperitoneal paraganglioma on April 2021 mass resection. Final diagnosis included the comment: Based on the modified grading of adrenal pheochromocytoma and paraganglioma (GAPP), the GAPP score is 1. Scores greater than or equal to 3 are malignant. We are aware that paraganglioma is classified as malignant for cases diagnosed in 2021+, however it is unclear how the pathologist's interpretation of the GAPP score may affect the behavior of this case. |
Report retroperitoneal paraganglioma based on ICD-O-3.2 histology/behavior that lists paraganglioma, NOS as 8680/3 for cases diagnosed 2021 and forward. While GAPP is a predictor of metastatic potential, it does not factor into behavior, thus reportability. |
2021 |
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20210033 | Reportability--Liver: Is a diagnosis of Liver Imaging Reporting and Data System (LI-RADS)-Treatment Response (LR-TR) viable nodule seen on imaging and treated with Y-90 radiotherapy reportable? See Discussion. |
Patient was initially diagnosed in 2017 with LR-5 lesions in segments 3 and 7 of liver and treated with radiofrequency ablation (RFA). Routine scans in 2019 show no evidence of residual or recurrent disease. Surveillance imaging in 2020 identifies LR-TR viable segment 3 treatment zone with slowly growing arterially-enhancing nodule as well as increasing arterial enhancement in the neighboring parenchyma. No new LR-4 or LR-5 observations. Patient is not a surgical candidate but is treated with Y-90 radiotherapy. Per Rule M10, tumors diagnosed more than 1 year apart are multiple primaries. However, there is no clear clinical statement of malignancy in this case. |
Do not report LR-TR viable as a new primary. LR-TR viable is a component of the Li-RADS Treatment Response algorithm designed to assess response for path-proven or presumed (e.g., LR-4, LR-5, LR-M) malignancy after locoregional treatment for hepatocellular cancer. LR-TR viable indicates it met the criteria as a viable tumor. |
2021 |
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20210051 | Primary site/Biliary tract--Ampulla of Vater: What is the correct primary site code for intra-ampullary and periampullary adenocarcinoma, C241 (8144/3) or C249? See Discussion. |
Ampulla, biopsy: High grade dysplasia with focal intramucosal carcinoma in a background of ulceration with acute and chronic inflammation. Surgery pathology: Head of pancreas, duodenum, and distal stomach, pancreaticoduodenectomy-Ampulla, Adenocarcinoma, intestinal type, intra-ampullary and peri-ampullary (mixed type). Grade moderately differentiated, 1.5cm. Tumor invades into duodenal submucosa. Lymphovascular Invasion: Foci suspicious for lymphovascular invasion identified. Perineural Invasion: Present. Synoptic report: Tumor Site Intra-ampullary and peri-ampullary (mixed type). Histologic Type Adenocarcinoma, intestinal type. There is not enough information regarding site in radiology reports or operative report. CT-A/P/C:The patient's known ampullary mass is not well visualized on this exam. No significant intrahepatic or extrahepatic biliary ductal dilation is identified. The pancreatic duct is normal caliber. |
Assign C241. Ampulla (C241) includes both periampullary and intra-ampullary. |
2021 |
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20210072 | Hormone Therapy--Breast: How are hormone therapy (HT) and other related data items coded when a patient had a previous breast primary and is still on HT when diagnosed with a new breast primary? See Discussion. |
In this scenario, we record that HT began for the second primary on the date of diagnosis, and the Systemic/Surgery Sequence ends up usually being coded 4 because the HT continues even if the specific agent may be changed. This does not seem to meet the definition of neoadjuvant therapy for the second primary so we approach the staging and grade coding as just clinical/pathological? For example, if the tumor size at surgery is a little larger than estimated on imaging, we would use the pathologic size for our staging. The tumor size and grade of the second primary are not being changed by the ongoing HT. Do we have the right approach? |
For this example: 1. Code HT as treatment on the date of diagnosis for the second primary. 2. Code Systemic/Surgery Sequence as 4. 3. Do not code neoadjuvant data items as neoadjuvant started/completed. The HT given would not qualify for neoadjuvant therapy since the intent of the HT was not neoadjuvant. The HT would affect the second primary, but it is still not neoadjuvant. 4. Code clinical and pathological tumor size accordingly, based on the imaging and the pathological findings. 5. Code Extent of Disease data items based on the pathological findings since pathological findings take priority over clinical and this is not neoadjuvant therapy. |
2021 |
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20210005 | Reportability/Histology--Ovary: Is a 2020 ovary case reportable with the positive malignant findings in adnexal cystic fluid and peritoneal washing? See Discussion. |
11/24/20 Adnexal mass, cyst fluid: Positive for malignant cells. Clusters of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells. Groups of inhibin-negative, CK7-positive epithelial cells consistent with serous borderline tumor cells. Peritoneal washing: Positive for malignant cells. Small groups of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells. A. Left ovarian mass: Adult granulosa cell tumor (AGCT) of ovary (see note). pTNM Stage: pT1c3 pNX - Serous borderline tumor (SBT) of ovary (see note). pTNM Stage: pT1a pNX. Fallopian tube; unremarkable. B. Right ovary: - Serous cystadenofibroma of ovary. Fallopian tube; unremarkable. C. Left pelvic wall nodule: Fibro-calcified nodule, consistent with necrotic appendix epiploica. D. Uterus (hysterectomy): Uterine leiomyomas. Endosalpingiosis of uterine serosa and paracervical tissue. Atrophic endometrium. Note: The left ovarian mass is involved by a combined adult granulosa cell tumor and a serous borderline tumor. The AGCT mainly involves the thick-walled cystic area while the SBT the thin-walled cyst/s. The 2 neoplastic elements do, however, demonstrate areas of intimate and close intermingling. From the current literature, it appears that, based on FOXL2 mutation, the AGCT component of combined AGCT and ovarian epithelial tumors is either a true neoplastic processes or an AGCT- like proliferation morphologically indistinguishable from AGCT. To further evaluate the nature of the AGCT component, a FOXL2 analysis is in progress and an addendum will follow. |
For cases diagnosed prior to 2021, report adult granulosa cell tumor of ovary only when stated to be malignant or when metastases are indicated, as by the positive peritoneal washings for this 2020 case. Beginning in 2021, report all cases of adult granulosa cell tumor of ovary based on ICD-O-3.2. |
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 |