CompArative Analysis Between, Thymic, pulmonaRy and Pancreatic Well Differentiated High Grade Neuroendocrine Tumors

The study involves the enrollment of 34 patients diagnosed with advanced thymic, pulmonary and duodeno-pancreatic well-differentiated high grade neuroendocrine tumors (Ki-67 \> 20%). The objective of this retrospective single-centre translational study will be to explore whether patients differ clinically in terms of diagnosis and treatment management. Currently, well differentiated high grade pulmonary NETs are managed using extrapolated algorithms from duodeno-pancreatic NETs, underlining a significant unmet clinical need. This is likely due to the rarity, uncertain pathological and molecular classification, and heterogeneous clinical course of well differentiated high grade pulmonary NETs. In this study a retrospective data-base of pulmonary, thymic and duodeno-pancreatic NETs with Ki-67 \> 20% will be created in order to analyze diagnostic and therapeutic pathways, clinical outcomes, imaging, disease evolution and molecular profiling. This study will adopt a hypothesis-generating approach to explore whether patients in these distinct groups differ clinically in terms of diagnosis and treatment management.

Sex

All

Inclusion Criteria:

* Histologically confirmed diagnosis of well-differentiated high grade neuroendocrine tumor (Ki-67 \> 20% according to WHO 2022) performed or reviewed by a NEN-dedicated pathologist.
* Primary tumor site:thyme, lung and duodenum-pancreas NETs.
* Advanced stage of tumor disease and Any number of lines of therapy
* Sufficient available clinical data on diagnosis, treatments, outcomes.

Exclusion Criteria:

* Poorly differentiated neuroendocrine carcinomas (NECs), GEP NET G1/G2, pulmonary carcinoid with Ki-67 \< 20%.
* Diagnosis of mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs)
* Inadequate or unavailable tumor tissue for molecular analysis.
* Incomplete clinical records or follow-up.
* Other primary sites, except lung or pancreas.