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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.

Yes, this metastatic teratoma is reportable.

This is a malignant teratoma by virtue of the lymph node metastases. Code the histology as 9080/3 [Teratoma, malignant, NOS]. Primary site is testis [C62_].

For tumors diagnosed prior to 2007:

Code as two primaries unless there is a physician's statement that the liver lesion is metastatic from the later small bowel carcinoid. Without such a statement regarding lesions 10 years apart, do not make an assumption that one is metastatic from the other.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.

When an aromatase inhibitor is part of the planned first course of therapy, code it under hormone treatment.

When a change of drug is PLANNED, it is part of the same course even if subcategories change. This is the usual situation with Tamoxifen and aromatase inhibitor (for example: Femara). The switch to Femara is planned, so it is not a new course. When a drug change happens that is not planned, it is still the same course if both drugs are in the same category and subcategory. An unplanned drug change to a different subcategory would be a new course.

For cases diagnosed 1998-2003:

Code the largest dimension mentioned, since that is the standard rule for coding tumor size. Keep in mind that tumor size is not used in analysis for certain sites such as stomach, colon & rectum, ovary, prostate, and urinary bladder. Tumor size is important for analysis for certain sites such as lung, bone, breast, and kidney.

Based on the information provided, code the tumor size as 035.  We asked an expert pathologist to review this question and she said to use the larger measurement.  She also said "the pathologist usually cuts the anus and rectum open like a tube; the “circumference” would be measured flat."

Do not code the ICD-O-3 grade for Ewing sarcoma unless documented in the record.

In the TNM system, grade is required to place Ewing sarcoma into a stage group. For TNM staging purposes, Ewing sarcoma is classified as G4. Do not apply TNM rules to ICD-O coding.

Code treatment with Iressa as chemotherapy.

Iressa is an epidermal growth factor inhibitor. While it doesn't kill cells directly, it damages the cell reproduction process. We classify it as a chemotherapy agent.

For tumors diagnosed prior to 2007:

Code the Histology and Grade, Differentiation fields to 8440/34 [cystadenocarcinoma, anaplastic] because a combination code for the specified histologic type does not exist.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.

Code Cixutumumab as Other Therapy.  Cixutumumab is still in clinical trials and not approved by FDA yet. Though it is classified as an immunotherapy agent, it is not approved.