Table 1. Comparator evaluation of bronchopulmonary and thymic neuroendocrine neoplasia.
| Topic | Feature | Bronchopulmonary | Thymic |
|---|---|---|---|
| Epidemiology | Incidence (per/100,000) | 1.35 | 0.02 |
| Prevalence | 20% of lung neoplasms | 2–3% of thymic neoplasms | |
| Clinical | Presentation | 40% incidental | Symptomatic (~50%) |
| Age | 5–6th decade | 5th decade | |
| Gender | M = F | M > F | |
| Metastatic | 25% TC, ≥50% AC | Frequent (>70%) | |
| Histology | Histology | NET (>90%) (grade I, II) | NEC (>70%) |
| Imaging | SRI | Positive >80% | Positive >80% |
| 18FDG-PET | Positive (33% TC, 94% AC) | Positive* | |
| Biomarkers | Monoanalytes (CgA, NSE, ACTH) | Inadequate (<40% for CgA, <25% NSE) | Specific monoanalytes (ACTH ~40%) |
| Prognosis | Survival (5-year) | 50–95% | 30–70% |
| Genetic topography | Mutational burden | Low | Low |
| Nicotine mutation signature | No | Unknown** | |
| Germline MEN-1 | 5–13% | ~25% | |
| Sporadic MEN-1 | Frequent (~50%) | No*** | |
| Chromatin/epigenetic remodeling | Frequent (~50%) | Unknown | |
| Chromosomal abnormalities | Low <35% | Frequent: 31–88% | |
| Potential drug targets (copy number gains) | EGFR (gefitinib), MET (crizotinib), PDGFRβ (imatinib), HER4 (lapatinib), AKT1/FRAP1 (everolimus) | None | |
| Potential Informative cell biology/pathway expression | VHL, P21WAF1CIP1, stathmin, ER# | PAK3, β-catenin, NOTCH2 | |
| Potential prognostic biomarkers | CD44$, OTP$$, RET$$, E-cadherin/β-catenin&, DSG3&& | None |
*, no studies have been undertaken in TNETs (21); **, no exome sequencing available; ***, MEN-1 retains heterozygosity; #, TC =55%; AC =86%; $, increased in poor prognosis; $$, decreased in poor prognosis; &, decreased in TC; &&, DSG3 (desmoglein 3) decreased in AC. NEC, neuroendocrine carcinoma; NET, neuroendocrine tumor; SRI, somatostatin receptor imaging.