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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20210071 | Solid Tumor Rules (2018/2021)/Histology--Breast: How is histology coded for a diagnosis of invasive mammary neuroendocrine tumor (NET), grade 2/3? See Discussion. |
Table 3 (Breast Equivalent Terms and Definitions) lists “Neuroendocrine tumor, well-differentiated” of the breast as histology 8246/3. There is no entry for a grade 2 neuroendocrine tumor of the breast in Table 3. The pathologist did not indicate the neuroendocrine tumor was poorly differentiated (or it would otherwise be a small cell carcinoma). The pathologist noted “By current WHO criteria, this tumor is characteristic of a mammary neuroendocrine tumor, grade 2. These invasive tumors have similar prognostic and predictive features of invasive ductal carcinoma of the same grade and stage.” |
Assign code 8249/3, neuroendocrine tumor, grade 2 based on the pathologist statement of mammary neuroendocrine tumor grade 2. According to WHO Classification of Tumors of the Breast, 5th edition, neuroendocrine tumor (NET) is an invasive tumor characterized by low/intermediate grade. If the histology term is not listed in the Solid Tumor rules, the instructions state to also check ICD-O and updates. Per ICD-O, NET, grade 2 is coded 8249/3. Breast Table 3 will be updated for 2023. |
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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20210027 | Reportability--Heme and Lympoid Neoplasms--Polycythemia vera: Is secondary polycythemia vera reportable? See Discussion. |
A physician stated the patient likely had secondary polycythemia vera due to cardiac and pulmonary conditions but that a polycythemia vera could not be ruled out. A JAK2 was ordered that was positive for JAK2 V617F mutation. The patient was treated with hydrea. According to SEER SINQ 20120049, secondary polycythemia vera is not reportable. However, in this case, the patient was positive for JAK2 V617F mutation. Therefore, is this reportable? We looked for guidance in the Hematopoietic and Lymphoid Neoplasms Database and found it confusing that secondary polycythemia vera was not mentioned or discussed under polycythemia vera in the database. The only thing we could find was secondary polycythemia NOS that was discussed under polycythemia. |
Abstract as a new primary for polycythemia vera, 9950/3. JAK2 is commonly used to assess suspected polycythemia vera and in this case, the mutation is positive for V617F. Based on the JAK2 results, this looks like a true polycythemia vera and not a secondary polycythemia. |
2021 |
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20210007 | First Course Treatment/Reason for No Surgery of Primary Site: How should we be coding Reason For No Surgery of Primary Site for cases where surgery was planned but ultimately cancelled due to progression? See Discussion. |
There is a discrepancy in the SEER and STORE manual definition of code 2 for Reason for No Surgery of Primary Site. STORE includes progression of tumor prior to planned surgery as part of the definition for code 2, but the SEER Manual does not. The progression statement is included in the SEER Manual (2018 and 2021) for Reason for No Radiation, but not for Reason for No Surgery. |
Assign code 2 for cases where surgery was planned but ultimately cancelled due to progression in the data item Reason For No Surgery of Primary Site. Code 2 description contains examples and is not exhaustive of reasons for no surgery. We will add the example for consistency in the next version of the SEER manual. |
2021 |
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20210058 | Multiple Primaries/Histology--Lymphoma: What is the histology code and how many primaries are there based on a gastrohepatic lymph node biopsy that shows: Nodular lymphocyte-predominant Hodgkin lymphoma with T-cell/histiocyte rich diffuse large B-cell lymphoma (DLBCL)-like transformation. If two primaries, what is the diagnosis date for each primary? See Discussion. |
4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored. 4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH. 5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia. 6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma. |
We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3). The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern. In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis. We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns. |
2021 |
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20210020 | Behavior--Breast: Should the behavior change to /3, invasive, to get a case to clear edits? The histology of this breast case is ductal carcinoma in situ (DCIS), 8500/2. Lymph nodes are positive for micro-mets (0.2 mm-2 mm). SEER Summary Stage: 3, regional lymph nodes positive. This creates an edit for SEER Summary Stage due to the behavior code of /2, in situ. |
Code the behavior to /3, not just to pass edits, but because this is an invasive case based on the positive lymph nodes. For most cases, behavior is based on the primary tumor, but in situations like this where an invasive component cannot be found and there are positive lymph nodes, the /3 behavior is assigned based on the positive lymph nodes. |
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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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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20210010 | Reportability--Head & Neck: Is chondrosarcoma, grade 1 reportable for cases diagnosed 01/01/2021 and later? See Discussion. |
Neither the ICD-O-3.2 Implementation Guidelines nor the ICD-O-3.2 Coding Guidelines (including Tables 1-7) address reportability changes for chondrosarcoma grade 1. In the Solid Tumor Rules Manual, Head and Neck Equivalent Terms and Definitions, Table 7 (Tumors of Odontogenic and Maxillofacial Bone (Mandible, Maxilla)), Chrondrosarcoma grade 2/3 (9220/3) is included as a subtype/variant for sarcomas in these sites, but it does not address chrondrosarcoma, grade 1. The ICD-O-3.2 Coding Table lists Chondrosarcoma, grade 1 as morphology code 9222/1. If Chondrosarcoma, grade 1 is no longer a reportable tumor for cases diagnosed 01/01/2021 and later, why wasn't this reportability change included in the ICD-O-3.2 Implementation Guidelines? If the standard setters chose not to include this reportability change, shouldn't Table 7 also indicate that all chondrosarcomas (NOS, grade 1, grade 2 or grade 3) are reportable for cases diagnosed 2018 and later? How are registrars to make reportability and histology coding decisions for chondrosarcomas when neither source provides clear instructions regarding these tumors? |
Chrondrosarcoma, grade 1 (9222/1) is not reportable according to the Reportability section in the 2021 SEER Manual. The histology (9222/1) is listed in ICD-O-3.2 as a synonym for atypical cartilaginous tumor (preferred term). In general, the tables do not include non-reportable terms and codes. Registrars should refer to their standard setter (to whom they submit data) for reportable neoplasms. Currently, /0 and /1 neoplasms are reportable for central nervous system sites only. ICD-O-3.2 includes all neoplasms but that does not mean they are reportable. If a facility collects non-malignant neoplasms, use the corresponding ICD-O code in 3.2. |
2021 |