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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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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20210073 | Solid Tumor Rules (2018/2021)/Multiple Primaries--Corpus Uteri: How many primaries should be reported when a hysterectomy identifies primary endometrial carcinosarcoma (8980/3) and the endometrium has a background of endometrioid intraepithelial neoplasia (EIN) (8380/2)? A tumor size is provided for the carcinosarcoma, but not the background EIN. |
Patient was diagnosed with carcinosarcoma of Mullerian origin on omental/pelvic biopsies in March 2021. First course treatment was neoadjuvant chemotherapy followed by July 2021 resection showing residual primary endometrial carcinosarcoma with cervical stromal invasion and involvement of bilateral tubes/ovaries, omentum, and mesenteric nodule. Additional findings included endometrium with background endometroid intraepithelial neoplasia (EIN). |
Abstract this case as a single primary and code histology as carcinosarcoma (8980/3). The carcinosarcoma is intermixed with the EIN making this a single primary coded to the invasive histology. EIN is a precursor of endometrial carcinoma in the WHO Classification of Female Genital Tumors, 5th edition. Carcinosarcoma of the uterus is described in the literature as an aggressive variant of endometrial carcinoma characterized by unusual histologic features including discrete malignant epithelial and mesenchymal components (carcinoma and sarcoma). |
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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20210006 | Behavior/Summary Stage 2018--Colon: What is the correct behavior and Summary Stage for a case of intramucosal adenocarcinoma arising in tubular adenoma? AJCC states this is Tis, though SEER Summary Stagie states this is Localized (code 1). The histology is 8140/2 (adenocarcinoma in situ), but the SEER Summary Stage is Locallized. |
Intramucosal carcinoma of the colon is assigned behavior code of /3. Intramucosal is not the same as in situ in terms of behavior. Behavior and staging are separate concepts, although there is some overlap. Use the instructions for coding behavior to code this field. Do not use stage to determine behavior in this case. For purposes of Summary Stage, intramucosal carcinoma is a localized lesion; however, for purposes of AJCC staging, assign Tis for the stage. |
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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20200049 | Summary Stage 2018/EOD 2018--Lymphoma Orbital Adnexa: What is the correct Summary Stage 2018 (SS2018) for the site/histology Orbit, NOS (C696), 9699/3? In SEER*RSA, Extent of Disease (EOD) Primary Tumor references code 7 (Distant), whereas SS2018 assigns code 2 (Regional)? See Discussion. |
We received an edit error in SEER*DMS on the following site/histology (Orbit, NOS (C696)/9699/3) that involved an incorrect staging code being assigned to SS2018. The staging language is identical in AJCC, EOD and SS2018. SEER*RSA notes that SS2018 should be coded distant, but in the SS2018 manual, this language is noted Regional. Staging language is: Orbital adnexal lymphoma AND extraorbital lymphoma extending beyond the orbit to adjacent structures--Bone, Brain, Maxillofacial sinuses |
To clear this edit of the derived Summary Stage (based on EOD) and the manually assigned Summary Stage (based on Summary Stage 2018), assign the manually assigned Summary Stage to 7. For this particular case, EOD Primary Tumor 700 (which is correct based on the information received) derives Distant; however, for Summary Stage 2018, this description is under Code 2 for Regional by direct extension. This is an error. For 2022, Summary Stage for Lymphoma Ocular Adnexa description under Code 2 (Regional by direct extension) will be moved to Distant. No changes will be done to EOD. |
2020 |
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20200022 | Solid Tumor Rules (2018)/Multiple primaries--Breast: How many primaries should be reported for a December 2013 diagnosis of lobular carcinoma in situ (8520/2) in the left breast, treated with a lumpectomy, followed by a July 2018 diagnosis of invasive ductal carcinoma (8500/3) also in the left breast? See Discussion. |
In the April and July 2019 updates to the Solid Tumor Rules, the term simultaneous and Note 1 indicating histologies must be the same behavior were removed from rule M10 (ductal and lobular are a single primary). We would like to confirm that rule M10 is the correct rule to apply to this case. This case is an invasive diagnosis approximately 4.5 years after an in situ diagnosis, so it seems like M17 should apply (invasive tumor following an in situ tumor more than 60 days later are multiple primaries). An invasive tumor following an in situ tumor more than 60 days later of the same histology is a new primary. Similarly, it seems like an invasive tumor following an in situ tumor more than 60 days later of different histologies should be a new primary. |
Abstract a single primary using 2018 Breast Solid Tumor Rule M10. Unless the tumors were diagnosed more than 5 years apart, they are a single primary. The 2021 breast update will include examples and notes plus updating table 2. |
2020 |
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20200060 | First Course Treatment/Reportability: Are there situations for which a case with a class-of-case code in the 30's should be reported to the central registry? We know these are not reportable to the CoC, but should they be reported to the central registry? See Discussion. |
Example: 3/22/2017-26 year old white female seen in the emergency room with abdominal pain. Patient was diagnosed about a month ago with breast cancer. Impression: menstrual pain. In this example the patient is newly diagnosed with breast cancer, but the second hospital does not treat or diagnose the patient; pain management for a separate condition is received only. Is this patient reported due to the history of active disease? |
Work with your central registry to determine which cases they require you to report. In general, any case still undergoing first course of treatment, even if not given at your facility, should be reported to the central registry. Many central registries will appreciate knowing that the patient was seen at your facility to update date last seen and other data items. |
2020 |
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20200047 | Stage-related Data Item/Lymphovascular Invasion--Ovary: The 2018 SEER Program Coding and Staging Manual states that LVI is coded 8 (Not applicable) for Ovary (Schema 00551). What is the reason for having lymphovascular invasion (LVI) coded "8" for Ovary? See Discussion. |
This direction is also in SEER*RSA for Ovary. Researching a possible explanation for this, we found that LVI is an independent predictor of progression and survival in patients with primary epithelial ovarian cancer at early stage but not at advanced stage. However, studies also recommend that routine evaluation of LVI in ovarian cancer is highly recommended in daily practice. |
The coding instructions were developed and implemented in concert with the AJCC Cancer Staging Manual, 7th edition, and updated with the 8th edition as per the 2018 STORE Manual and were based on sites where distinguishing between lymphatic/small vessel invasion and venous/large vessel invasion was not medically appropriate. SEER required LVI for penis and testis cases only beginning in 2016; sites other than penis or testis are coded 8 unless required by state or central registries. The list for use of code 8 has been changed for 2021 and will no longer include Ovary. |
2020 |