Report | Question ID | Question | Discussion | Answer | Year |
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20210011 | Primary site: Is C720 the correct primary site for a diagnosis of a paraspinal neuroblastoma on autopsy in a nine month old with Noonan syndrome? See Discussion. |
Autopsy/Pathology Report (2020) excerpts External Examination Nervous System: There is an 8.5 cm mass located in the right thoracic paraspinal area. Final Anatomic Diagnosis Clinical History: Paraspinal mass suspicious for neuroblastic tumor (detected by imaging studies) Nervous System: Right thoracic paraspinal neuroblastoma, poorly differentiated |
Assign primary site code C473 for this case based on the information provided (peripheral nerves and autonomic nervous system of thorax). From our expert pathologist consultant: The origin of neuroblastomas is generally in the adrenal medulla or one of the sympathetic ganglia on either side of the vertebral column (although they have been reported in many other locations given the migration of the neural crest cells embryologically). |
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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20210061 | First course treatment/Update to current manual: Should the instruction regarding expectant management in the 2021 (and 2022) SEER Manual include how to code for the patient’s decision to proceed with expectant management? See Discussion. |
Currently, First Course Therapy instruction for expectant management (also referred to as active surveillance, watchful waiting, etc.) instructs one to code 0 or 00 (not done) for all data items when the physician opts for expectant management. We find that the treatment decisions can be driven by the patient, physician, or combination of both patient and physician depending on the options presented. |
Instructions for First Course of Therapy include using the documented first course of therapy (treatment plan) from the medical record. While a patient may weigh in on the treatment decision, the physician is responsible for developing and managing the treatment plan including closely watching a patient’s condition but not giving treatment unless symptoms appear or change. We can add language to a future manual to clarify. |
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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20210057 | Reportability/Histology--Kidney: Is an oncocytic renal neoplasm of low malignant potential (ORNLMP) reportable? See Discussion. |
Kidney, right interpolar neoplasm, partial nephrectomy: Oncocytic renal neoplasm of low malignant potential (ORNLMP). Within part B, right interpolar kidney neoplasm, the neoplasm shows oncocytic features, with abundant granular eosinophilic cytoplasm and enlarged vesicular nuclei with prominent central nucleoli. The cells are arranged in small nests and tubules with hypocellular fibrous stroma identified within the background. Scattered binucleated cells are present, and rare cells with irregular nuclear membranes are present. No perinuclear halos or prominent cell membranes are present. Given the histologic features, the neoplasm is best classified as an oncocytic renal neoplasm of low malignant potential (ORNLMP). |
Oncocytic renal neoplasm of low malignant potential is not reportable. |
2021 |
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20210069 | EOD 2018/Summary Stage 2018--Intrahepatic Bile Duct: How should Extent of Disease (EOD) Primary Tumor (PT) be coded for invasion of or into (but not through) the visceral peritoneum for an intrahepatic bile duct primary? See Discussion. |
Invasion of the visceral peritoneum is Regional (code 2) in Summary Stage. EOD PT code 500 is for invasion BEYOND the visceral peritoneum into adjacent connective tissues, and maps to T3 and Regional Summary Stage, but that code seems too extensive. All lower EOD codes map to Localized Summary Stage. |
Assign code 500 for EOD Primary Tumor for now. We have confirmed with AJCC that "invasion of" but not "through" the visceral peritoneum maps to a T2 and not T3. Involvement of the visceral peritoneum for Summary Stage is Regional and does not make a distinction between "invasion of" or "invasion through." Any involvement of the visceral peritoneum is regional. To correct this situation would require a new code, which would derive a T2/RE. That code will be added to the updates for 2023. Code 500 will derive the appropriate Summary Stage of 2 (Regional). We are aware that this will derive the incorrect T; however, there is no work around at this time that will derive the correct T and Summary Stage, so we are defaulting to deriving the correct Summary Stage. |
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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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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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 |
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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 |