Extrapulmonary neuroendocrine carcinoma: lines of therapy
5 standard-of-care settings across 5 lines and 2 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.
| Line | All comers | HR-positive |
|---|---|---|
| Screening, prevention and diagnosis | 1 | · |
| Early / localised | 1 | · |
| Advanced, first line | 1 | · |
| Second line | 1 | · |
| Other settings | · | 1 |
Screening, prevention and diagnosis
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Diagnosis and staging | Biopsy with Ki-67, morphology and p53 or Rb immunohistochemistry to separate carcinoma from grade 3 tumour; FDG PET and CT; somatostatin receptor PET only if radioligand therapy is contemplated. | NCCN Guidelines: Neuroendocrine and Adrenal Tumors | 89 |
Early / localised
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Localised disease | Resection or definitive chemoradiotherapy with perioperative platinum-etoposide, following the limited-stage small-cell lung cancer model. | NCCN Guidelines: Neuroendocrine and Adrenal Tumors | 82 |
Advanced, first line
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Metastatic, first line | Platinum with etoposide (cisplatin or carboplatin), with a PD-1 or PD-L1 antibody added by extrapolation from small-cell lung cancer or within a trial. | NCCN Guidelines: Neuroendocrine and Adrenal Tumors | 90 |
Second line
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Second line | FOLFIRI, FOLFOX, capecitabine-temozolomide or topotecan; nivolumab with ipilimumab in selected patients (DART); DLL3-directed T-cell engagers in trials. | FOLFIRI (5-FU, leucovorin, irinotecan)FOLFOX (5-FU, leucovorin, oxaliplatin)Capecitabine + temozolomide (CAPTEM)TopotecanNivolumabIpilimumabDART (SWOG S1609): nivolumab plus ipilimumab in rare tumoursDAREON®-NEC-1: A Study in People With Advanced Extrapulmonary Neuroendocrine Cancer (epNEC) to Compare Obrixtamig Plus Carboplatin and Etoposide Treatment With Standard ChemotherapyDAREON™-5: A Study to Test Whether Different Doses of BI 764532 Help People With Small Cell Lung Cancer or Other Neuroendocrine CancersDLL3 | NCCN Guidelines: Neuroendocrine and Adrenal Tumors | 86 |
Other settings
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| HR-positive | Ki-67 near 20 to 55 percent with well-differentiated features | Reclassify as grade 3 neuroendocrine tumour and treat accordingly. | NCCN Guidelines: Neuroendocrine and Adrenal Tumors | 81 |
Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.