Epithelial Neuroendocrine Neoplasms (NEN)

Primary focus on epithelial neuroendocrine neoplasm <<127575007 |Malignant epithelial neuroendocrine neoplasm (morphologic abnormality)| and related disorders.

Neural neuroendocrine and Pituitary neuroendocrine neoplasms  addressed in a separate tasks.

Existing concepts for <<55937004 |Neuroendocrine tumor (morphologic abnormality)| are to be inactivated as ambiguous due to conflation of well-differentiated neuroendocrine tumor morphologies and neuroendocrine carcinoma morphologies.

New concepts created for Well-differentiated neuroendocrine tumor (NET) and Neuroendocrine carcinoma (NEC)

All NET and NEC morphologies are considered malignant with only limited exceptions (e.g., 32071008|Strumal carcinoid (morphologic abnormality)|).  Therefore, all “benign” NET and NEC morphologies are to be inactivated as outdated.  They will be replaced by their malignant counterparts.

Per lead Blue Book authors, carcinoid morphologies are equivalent to well-differentiated neuroendocrine tumors (NET).  Therefore, all carcinoid morphologies are to be inactivated and replaced by NET morphologies.  When prevailing clinical practice retains the use of “carcinoid” in descriptions of morphologies, a synonym containing carcinoid is maintained.  This is prevalent in lung pathology, for example.

Multiple disorder concepts are  inactivated as erroneous based on modeling and/or considered outdated.  Replacement concepts were created where appropriate.

Several FSNs were incomplete and/or incorrect for the disorder concepts.  These were corrected and/or inactivated with replacement concepts created.

For all concepts with Orphanet mappings, the Orphanet definition was reviewed along with the defining morphology concept.  Where the SNOMED CT concept was not accurately modeled for the Orphanet definition, the concept was inactivated and replaced by a new concept.  Mapping information is provided to the mapping team.