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Reportability of neuroendocrine neoplasms depends on primary site, terminology, and differentiation. "Neuroendocrine neoplasm" is an umbrella term for a variety of neuroendocrine tumors and carcinomas.

Neuroendocrine neoplasm, not otherwise specified (NEN, NOS) is not reportable as in your example unless it is being used as a synonym for neuroendocrine tumor (NET), as with digestive system tumors.  According to WHO Classification of Digestive System Tumors, 5th ed., NENs of the appendix and liver are epithelial neoplasms with neuroendocrine differentiation, including well-differentiated tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs).

The guidance in SINQ 20180097, 20150001, and 20140051 is still valid.

Assign code 8163/3. Based on the microinvasion, the correct term for this neoplasm is pancreatobiliary-type carcinoma. Unfortunately, WHO did not provide all synonyms or related terms for some of the new ICD-O codes. Pathologists may continue using non-preferred terminology as well.

The 2021 AIN III is not a new primary. According to our GI pathology expert, findings of AIN I and/or AIN II following a diagnosis of AIN III indicates the patient was never NED and indicates persistent disease.

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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.

This is a correction to the SEER manual. Lymphomas originating in lymph nodes (C77) could have distant metastases to any site except lymph nodes. The following corrections to the manual apply now and will appear in the next version of the manual.

Remove C770-C779 from the instruction for assigning code 8 on the following pages.

Page 135 Mets at Dx--Bone

Page 137 Mets at Dx--Brain

Page 139 Mets at Dx--Liver

Page 141 Mets at Dx--Lung

Page 145 Mets at Dx--Other

Example

Biopsy of axillary lymph node: Diffuse Large B-Cell lymphoma. Lymph nodes involved above and below the diaphragm, multiple nodules seen in lung, lesions in liver. Bone marrow biopsy positive for DLBLC. Per Hematopoietic manual, primary site would be C778 for multiple lymph node regions involved.

Mets at Dx--Bone-0

Mets at Dx--Brain-0

Mets at Dx--Liver-1

Mets at Dx--Lung-1

Mets at Dx--Distant Lymph Nodes-8

Mets at Dx--Other-1

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.

Assign the neoadjuvant therapy data items as if the patient had neoadjuvant therapy.  Neoadjuvant Therapy data item would be coded either code 1 or 2 depending on whether the chemotherapy was completed or not. In this case, they are a surgical candidate by having the chemotherapy with the plan from the beginning to evaluate the chemotherapy after X cycles to see if surgery can be performed. After the patient is evaluated, update the abstract as needed.

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.

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.

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.