6533b853fe1ef96bd12accfe
RESEARCH PRODUCT
A rare rarity: neuroendocrine tumor of the esophagus
Elisa GiannettaValentina GuarnottaFrancesca RotaFederica De CiccoFederica GrilloAnnamaria ColaoAntongiulio FaggianoM. AlbertelliA. BianchiL. CircelliA. DicitoreC. Di DatoS. Di MolfettaG. FanciulliF. FerrauM. GalloE. GrossrubatscherE. GuadagnoF. Lo CalzoE. KaraP. MalandrinoE. MessinaR. ModicaG. MuscogiuriG. PizzaP. RazzoreM. RubinoR. M. RuggeriC. SciammarellaG. VitaleM. C. Zatellisubject
0301 basic medicinemedicine.medical_specialtyCarcinoid tumorsesophageal neoplasmsNeuroendocrine tumorsSmall-cell carcinomaGastroenterology03 medical and health sciences0302 clinical medicinegastroenteropancreatic NETInternal medicineEpidemiologymedicinerisk factorsesophageal NEC; gastroenteropancreatic NET; large cell esophageal NEN; MANEC; small cell carcinoma; delayed diagnosis; esophageal neoplasms; humans; neuroendocrine tumors; prognosis; rare diseases; risk factorsEsophagusStage (cooking)esophageal NECdelayed diagnosishumanssmall cell carcinomabusiness.industryLarge cellMANECrare diseasesHematologymedicine.diseaseDysphagia030104 developmental biologymedicine.anatomical_structureOncology030220 oncology & carcinogenesislarge cell esophageal NENprognosismedicine.symptomneuroendocrine tumorsbusinessdescription
Abstract Esophageal Neuroendocrine tumors (NETs) are rare, aggressive and lacking specific symptoms. This causes a diagnostic delay, worsening the prognosis. Numerous cases are reported in literature, without a consensus on the management. Our aim was to clarify epidemiology, clinical presentation, diagnostic, therapeutic management of esophageal NETs. Extensive literature search identified a total of 226 articles. One hundred twenty-five articles (n = 1676) met the inclusion criteria, showing that: the incidence of esophageal NET varies geographically; men (60–70 years) are more affected; smoking and alcohol abuse are the major risk factors; dysphagia, weight loss, appetite loss are the most common clinical features. The histotypes include high-grade small and large cell esophageal carcinomas and low-grade carcinoid tumors. Mixed neuroendocrine/non-neuroendocrine neoplasms are the most common. Often the diagnosis occurs randomly on endoscopic examination. Circulating markers, functional combined with conventional imaging contributes to the diagnosis and management. Treatment depends on type, grade and stage of the tumor.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2019-01-01 |