Tumor neuroendocrino pancreático (pNET), bem diferenciado G1/G2
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| ID | DIS-PNET |
|---|---|
| Tipo | Doença |
| Alegais | PNET funcional (insulinoma, gastrinoma, glucagonoma, VIPoma)Non-functional pNETTumor de células de islote pancreáticasTumor neuroendocrino pancreáticoPNETНейроендокринна пухлина підшлункової залози (пНЕП), добре-диференційована G1/G2 |
| Estado | reviewed 2026-05-03 | pending_clinical_signoff |
| Doenças | Nenhuma declaração |
| Fontes | SRC-NCCN-NET-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8240/3 |
|---|---|
| icd_o_3_topography | ["C25"] |
| icd_10 | C25.4 |
| who_classification | WHO Classification of Digestive System Tumours 5th ed (2019): Pancreatic neuroendocrine tumour G1 (Ki67 <3%), G2 (3–20%), G3 (>20% but well-differentiated) |
Notas
Well-differentiated pNET G1/G2 is the dominant subtype in clinical practice (non-functional, discovered incidentally or via abdominal mass/liver mets). Grading per WHO 2019: G1 Ki67 <3%, G2 Ki67 3–20%. G3 well-differentiated pNET (Ki67 >20%) occupies a grey zone — still well-differentiated morphologically but higher proliferation; NCCN recommends everolimus/sunitinib/SSA ± chemo; not modelled separately here (flagged via RF-NET-HIGH-GRADE-G3 for MDT review). Distinguishing from poorly differentiated NEC (ICD-O 8246/3) is essential: NEC requires platinum-etoposide chemotherapy (not SSA/TKI/mTOR). Key trials: RADIANT-3 (everolimus, Yao NEJM 2011), Raymond 2011 (sunitinib), CLARINET pNET subgroup (lanreotide). Surgery remains the only curative option for localized disease.
Usado por
Relevância terapêutica
BMA-CHROMOGRANIN-A-NET-MEN1-SURVEILLANCE- A cromogranina A (CgA) é uma proteína secretaria da família da granina, expressa em...
Algoritmos
ALGO-MEN1-CARRIER-SURVEILLANCE- ALGO-MEN1-CARRIER-SURVEILLANCEALGO-PNET-METASTATIC-1L- ALGO-PNET-METASTATIC-1LALGO-VHL-CARRIER-SURVEILLANCE-VHL-ALLIANCE- ALGO-VHL-CARRIER-SURVEILLANCE-VHL-ALLIANCE
Doença
DIS-GI-NET- Tumor neuroendocrino gastroenteropancreático — origem GI (carcinoide), bem diferenciado G1/G2DIS-PARATHYROID-CARCINOMA- Carcinoma paratiroideoDIS-PITUITARY-ADENOMA- Adenoma de pituitária
Indicação
IND-MEN1-CARRIER-INTENSIFIED- IND-MEN1-CARRIER-INTENSIFIEDIND-MEN1-CARRIER-SURVEILLANCE- IND-MEN1-CARRIER-SURVEILLANCEIND-MEN1-SUSPICION-PREVENTION-ENHANCED-SURVEILLANCE- IND-MEN1-SUSPICION-PREVENTION-ENHANCED-SURVEILLANCEIND-MEN1-SUSPICION-PREVENTION-GENETIC-COUNSELING- IND-MEN1-SUSPICION-PREVENTION-GENETIC-COUNSELINGIND-MEN4-CARRIER-INTENSIFIED- IND-MEN4-CARRIER-INTENSIFIEDIND-MEN4-CARRIER-SURVEILLANCE- IND-MEN4-CARRIER-SURVEILLANCEIND-PNET-METASTATIC-1L-EVEROLIMUS- IND-PNET-METASTATIC-1L-EVEROLIMUS
Procedimentos
PROC-NEPHRON-SPARING-PARTIAL-NEPHRECTOMY-VHL- Nefrectomia parcial poupadora de nefronos para VHL lesão renal (≥3 cm Limiar)
Questionários
QUEST-PNET-1L-STUB- Tumor neuroendocrino pancreático — primeira linha
Sinal de alerta
RF-MEN1-CONFIRMED-CARRIER- Confirmada variante patogênica da linha germinal / patogênica provável no gene MEN1 (menina).RF-MEN1-FAMILY-HISTORY-SUSPICION- Pedigree familiar sugestivo de Neoplasia Endócrina Múltipla tipo 1 (MEN1, Síndrome de Wermer)...RF-MEN4-CONFIRMED-CARRIER- Variante patogênica confirmada da linha germinal / patogênica provável em CDKN1B (p27/Kip1) — Múltiplas...RF-NET-CARCINOID-SYNDROME- Síndrome carcinoide ativa em um paciente com GI-NET metastático ou irressecável (ou outra...RF-NET-HIGH-GRADE-G3- Well-differentiated or poorly-differentiated neuroendocrine tumor with Ki67 >20% (WHO 201...
Fontes
SRC-CLARINET-CAPLIN-2014- Lanreotide em tumores neuroendocrinos metastáticos (CLARINET; fase III)SRC-NCCN-NET-2025- Tumores neuroendocrinos e adrenais NCCNSRC-RADIANT3-YAO-2011- Everolimus para tumores neuroendocrinos pancreáticos avançados (RADIANT-3; fase III)