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.
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.
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.
Histologically confirmed diagnosis of thymic, pulmonaRy and pancreatic well differentiated high grade neuroendocrine tumors(Ki-67 \> 20% according to WHO 2022)
Cohort
Retrospective
Not listed
Therapeutic algorithm
The primary endpoint of this study will be to compare therapeutic algorithm applied to the two grups: well-differentiated duodeno-pancreatic, versus thymic and lung NETs with high proliferative indices.
overall survival
To compare overall survival (OS) in the two groups.
First line progression-free survival
To compare first line progression-free survival (PFS) in the histological groups.
treatment response across lines of therapy
To assess treatment response across lines of therapy, in both cohorts.
genetic alterations
To correlate specific genetic alterations with clinical outcomes (OS, PFS). To explore potential actionable molecular targets and their distribution across the two tumor origins.
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