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1
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Li W, Shao M, Hu S, Xie S, He B. The diagnostic value of endoscopic ultrasound for esophageal subepithelial lesions: A review. Medicine (Baltimore) 2024; 103:e40419. [PMID: 39560558 PMCID: PMC11576025 DOI: 10.1097/md.0000000000040419] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/06/2024] [Accepted: 10/18/2024] [Indexed: 11/20/2024] Open
Abstract
Esophageal subepithelial lesions (ESELs) encompass a variety of diseases, including leiomyoma, granular cell tumors, hemangioma, lipoma, stromal tumors, leiomyosarcoma, schwannoma, neuroendocrine tumors and more. These lesions often present asymptomatically, leading to a generally low clinical diagnosis rate. Common imaging techniques for diagnosing ESELs include conventional endoscopy, spiral computed tomography, and endoscopic ultrasound (EUS). Among these, EUS is currently regarded as one of the most accurate methods for diagnosing ESELs. In recent years, EUS has increasingly been combined with advanced technologies such as artificial intelligence, submucosal saline injection, high-frequency impedance measurement, and enhanced imaging to improve diagnostic accuracy and reduce missed diagnoses. This article reviews the application and recent advancements of EUS in diagnosing esophageal submucosal lesions.
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Affiliation(s)
- Wanwen Li
- Department of Thoracic Surgery, Sichuan Provincial People’s Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Mengqi Shao
- Department of Thoracic Surgery, The Second Xiangya Hospital of Central South University, Changsha, China
| | - Shichen Hu
- Department of Thoracic Surgery, Sichuan Provincial People’s Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Shenglong Xie
- Department of Thoracic Surgery, Sichuan Provincial People’s Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Bin He
- Department of Thoracic Surgery, Sichuan Provincial People’s Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
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2
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Esposito I, Häberle L, Yavas A. Neuroendokrine Neoplasien. DIE GASTROENTEROLOGIE 2024; 19:202-213. [DOI: 10.1007/s11377-024-00784-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 03/12/2024] [Indexed: 01/06/2025]
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Taori K, Kirnake V, Junare PR, Padwale V. An Uncommon Encounter: A Comprehensive Case Report of an Advanced Esophageal Neuroendocrine Carcinoma. Cureus 2024; 16:e53027. [PMID: 38410310 PMCID: PMC10895312 DOI: 10.7759/cureus.53027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2023] [Accepted: 01/26/2024] [Indexed: 02/28/2024] Open
Abstract
Esophageal neuroendocrine carcinomas (E-NECs) are rare malignant tumors with unknown etiology and pathogenesis. The aggressive nature of E-NECs coupled with a tendency to metastasize and no available treatment guidelines lead to poor prognosis. Here, we report a case of a 65-year-old, previously healthy female who presented with difficulty in swallowing solids, burning sensation over the epigastric region, weight loss (>10%), and altered bowel habits for the last three months. Contrast-enhanced CT (CECT) thorax revealed asymmetric mid-esophageal wall thickening with lymphadenopathy and metastasis in both hepatic lobes. Esophageal endoscopy revealed a large circumferential ulcero-proliferative mass and umbilicated lesions. A histopathological examination revealed small cells with scant cytoplasm and pleomorphic, hyperchromatic nuclei with prominent nuclear molding, and extensive necrosis. Immunohistochemistry revealed positivity for synaptophysin, chromogranin A, and CD56. Ki-67 index was 53%. These findings suggested poorly differentiated, small cell type, high-grade E-NEC. Chemotherapy with cisplatin (30mg/m2) + irinotecan (60mg/m2) was initiated. However, following two chemotherapy cycles, the patient succumbed.
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Affiliation(s)
- Kamlesh Taori
- Department of Gastroenterology and Hepatology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, IND
| | - Vijendra Kirnake
- Department of Gastroenterology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, IND
| | - Parmeshwar R Junare
- Department of Gastroenterology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, IND
| | - Vishal Padwale
- Department of Gastroenterology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, IND
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Lin Z, Chen YY, Liu CH, Panzuto F, Ramirez RA, Lang M, Kim H, Ding ZY. Comparison of clinicopathological features and survival analysis between esophageal neuroendocrine carcinoma and esophageal squamous cell carcinoma based on the SEER database, alongside nomogram analysis for esophageal neuroendocrine carcinoma. J Gastrointest Oncol 2023; 14:2309-2323. [PMID: 38196527 PMCID: PMC10772701 DOI: 10.21037/jgo-23-905] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/12/2023] [Accepted: 12/08/2023] [Indexed: 01/11/2024] Open
Abstract
Background Esophageal neuroendocrine carcinoma (ENEC) is a rare subtype of esophageal cancer (EC). It presents distinctive clinical and pathological features in comparison to esophageal squamous cell carcinoma (ESCC). To better elucidate the disparities between the two and establish a prognostic prediction model for ENEC, we conducted this study. Methods Data of ENEC and ESCC patients (1975 to 2016) were extracted from the Surveillance, Epidemiology and End Results (SEER) database. Patients with a confirmed pathological diagnosis of ENEC and ESCC were enrolled in the study. The Chi-square test was employed to compare categorical variables, and the median survival time was analyzed using the Kaplan-Meier curve. Training and validation groups were randomly assigned at a ratio of 7:3. Factors with a significance level of <0.05 in the multifactor regression model as well as age were integrated into the nomogram model. Concordance index (C-index), calibration curves, and decision curve analyses (DCA) were generated for model validation. Results This study encompassed a total of 737 ENEC patients and 29,420 ESCC. Compared to ESCC, ENEC patients had higher probability of liver metastasis (13.8% vs. 1.9%, P<0.001), poor differentiation (68.0% vs. 37.1%, P<0.001), and late SEER stage (52.8% vs. 26.9%, P<0.001). Patients who received either surgery, radiotherapy (RT), or chemotherapy had a significantly longer disease-specific survival (DSS) and overall survival (OS) (all P<0.001). After propensity score matching (PSM), ENEC patients were associated with shorter DSS (7.0 months vs. not reached, P<0.0001) and OS (7.0 vs. 12.0 months, P<0.0001) compared to ESCC. Race, SEER stage, surgery, RT, and chemotherapy were identified as predictors of DSS and were incorporated into the nomogram model together with age. The validation of the model using C-index (0.751 and 0.706, respectively) and calibration curves reflected the better discrimination power of the model. In addition, DCA supported the favorable potential clinical effect of the predictive model. Lastly, a risk classification based on the nomogram also verified the reliability of the model. Conclusions ENEC and ESCC exhibit distinct clinicopathological features. Patients with ENEC experience significantly poorer survival outcomes compared to those with ESCC. Surgical intervention, radiation therapy, and chemotherapy significantly improve OS and DSS for ENEC patients. The nomogram prediction model, constructed based on age, race, stage, and treatment regimen, demonstrates accurate and effective predictive capabilities for prognostic factors in ENEC patients.
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Affiliation(s)
- Zhen Lin
- Department of Biotherapy, Cancer Center, West China Hospital, Sichuan University, Chengdu, China
| | - Yue-Yun Chen
- Department of Biotherapy, Cancer Center, West China Hospital, Sichuan University, Chengdu, China
| | - Chun-Hua Liu
- Department of Biotherapy, Cancer Center, West China Hospital, Sichuan University, Chengdu, China
| | - Francesco Panzuto
- Department of Medical-Surgical Sciences and Translational Medicine, Sapienza University of Rome, Digestive Disease Unit, Sant’ Andrea University Hospital, ENETS Center of Excellence, Rome, Italy
| | - Robert A. Ramirez
- Division of Hematology and Oncology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA
| | - Matthias Lang
- Department of General, Visceral and Transplantation Surgery, University Hospital, Heidelberg, Germany
| | - Hyunchul Kim
- Department of Pathology, CHA Ilsan Medical Center, Goyang, Republic of Korea
| | - Zhen-Yu Ding
- Department of Biotherapy, Cancer Center, West China Hospital, Sichuan University, Chengdu, China
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Ortigão R, Isidoro R, Dinis-Ribeiro M, Libânio D. A rare case of an esophageal neuroendocrine carcinoma arising on an endoscopic resection scar. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2023; 115:721-722. [PMID: 36809910 DOI: 10.17235/reed.2023.9523/2023] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/17/2023]
Abstract
Here we report a case of a 61-year-old woman who underwent en-bloc endoscopic submucosal dissection (ESD) of a 10mm depressed lesion (Paris 0-IIc, Figure A) in the mid-esophagus. Histopathology showed a lesion with high-grade squamous dysplasia (R0). On follow-up endoscopy at 6 and 12 months the scar was regular, without signs of recurrence. Seven months after the last endoscopy, the patient presented with chest pain and dysphagia. Endoscopy showed an ulcero-vegetating tumor with 3cm at the same location of previous ESD (Figure B), and biopsies showed a poorly differentiated small cell neuroendocrine carcinoma (NEC). Subsequent computed tomography identified peri-tumor and hilar lymph nodes, and an extensive periceliac nodal conglomerate adherent to the liver (stage IV). This is, to our knowledge, the first case described of esophageal NEC arising on the endoscopic resection scar.
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Affiliation(s)
- Raquel Ortigão
- Gastrenterology , Instituto Português de Oncologia do Porto, Portugal
| | | | | | - Diogo Libânio
- Gastroenterology , Instituto Português de Oncologia do Porto
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Helderman NC, Suerink M, Kilinç G, van den Berg JG, Nielsen M, Tesselaar ME. Relation between WHO Classification and Location- and Functionality-Based Classifications of Neuroendocrine Neoplasms of the Digestive Tract. Neuroendocrinology 2023; 114:120-133. [PMID: 37690447 PMCID: PMC10836754 DOI: 10.1159/000534035] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/27/2023] [Accepted: 08/21/2023] [Indexed: 09/12/2023]
Abstract
Practice of neuroendocrine neoplasms (NENs) of the digestive tract, which comprise of a highly diverse group of tumors with a rising incidence, faces multiple biological, diagnostic, and therapeutic issues. Part of these issues is due to misuse and misinterpretation of the classification and terminology of NENs of the digestive tract, which make it increasingly challenging to evaluate and compare the literature. For instance, grade 3 neuroendocrine tumors (NETs) are frequently referred to as neuroendocrine carcinomas (NECs) and vice versa, while NECs are, by definition, high grade and therefore constitute a separate entity from NETs. Moreover, the term NET is regularly misused to describe NENs in general, and NETs are frequently referred to as benign, while they should always be considered malignancies as they do have metastatic potential. To prevent misconceptions in future NEN-related research, we reviewed the most recent terminology used to classify NENs of the digestive tract and created an overview that combines the classification of these NENs according to the World Health Organization (WHO) with location- and functionality-based classifications. This overview may help clinicians and researchers in understanding the current literature and could serve as a guide in the clinic as well as for writing future studies on NENs of the digestive tract. In this way, we aim for the universal use of terminology, thereby providing an efficient foundation for future NEN-related research.
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Affiliation(s)
- Noah C. Helderman
- Department of Clinical Genetics, Leiden University Medical Centre, Leiden, The Netherlands
| | - Manon Suerink
- Department of Clinical Genetics, Leiden University Medical Centre, Leiden, The Netherlands
| | - Gül Kilinç
- Department of Infectious Diseases, Leiden University Medical Centre, Leiden, The Netherlands
| | - José G. van den Berg
- Department of Pathology, Netherlands Cancer Institute, Amsterdam, The Netherlands
| | - Maartje Nielsen
- Department of Clinical Genetics, Leiden University Medical Centre, Leiden, The Netherlands
| | - Margot E.T. Tesselaar
- Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands
- Department of Gastrointestinal Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands
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7
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Swied MY, Turk YA, Jegadeesan R. A Poorly Differentiated Esophageal Neuroendocrine Carcinoma With Brain Metastasis. ACG Case Rep J 2023; 10:e01156. [PMID: 37753106 PMCID: PMC10519462 DOI: 10.14309/crj.0000000000001156] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/05/2023] [Accepted: 08/22/2023] [Indexed: 09/28/2023] Open
Abstract
Esophageal neuroendocrine carcinomas (NECs) are a rare type of esophageal neoplasm that can initially present with vague signs and symptoms. Gastrointestinal manifestations, such as dysphagia and abdominal discomfort, are the most common symptoms of neuroendocrine neoplasms. Although there is a potential for distant metastases because of esophageal NEC, few cases of brain metastasis have been reported. We report a rare case of an esophageal NEC metastasizing to the brain region.
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Affiliation(s)
| | - Yahia Al Turk
- Southern Illinois University School of Medicine, Springfield, IL
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Gopakumar H, Jahagirdar V, Koyi J, Dahiya DS, Goyal H, Sharma NR, Perisetti A. Role of Advanced Gastrointestinal Endoscopy in the Comprehensive Management of Neuroendocrine Neoplasms. Cancers (Basel) 2023; 15:4175. [PMID: 37627203 PMCID: PMC10453187 DOI: 10.3390/cancers15164175] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2023] [Revised: 08/15/2023] [Accepted: 08/15/2023] [Indexed: 08/27/2023] Open
Abstract
Neuroendocrine neoplasms (NENs), also called neuroendocrine tumors (NETs), are relatively uncommon, heterogenous tumors primarily originating in the gastrointestinal tract. With the improvement in technology and increasing use of cross-sectional imaging and endoscopy, they are being discovered with increasing frequency. Although traditionally considered indolent tumors with good prognoses, some NENs exhibit aggressive behavior. Timely diagnosis, risk stratification, and management can often be a challenge. In general, small NENs without local invasion or lymphovascular involvement can often be managed using minimally invasive advanced endoscopic techniques, while larger lesions and those with evidence of lymphovascular invasion require surgery, systemic therapy, or a combination thereof. Ideal management requires a comprehensive and accurate understanding of the stage and grade of the tumor. With the recent advancements, a therapeutic advanced endoscopist can play a pivotal role in diagnosing, staging, and managing this rare condition. High-definition white light imaging and digital image enhancing technologies like narrow band imaging (NBI) in the newer endoscopes have improved the diagnostic accuracy of traditional endoscopy. The refinement of endoscopic ultrasound (EUS) over the past decade has revolutionized the role of endoscopy in diagnosing and managing various pathologies, including NENs. In addition to EUS-directed diagnostic biopsies, it also offers the ability to precisely assess the depth of invasion and lymphovascular involvement and thus stage NENs accurately. EUS-directed locoregional ablative therapies are increasingly recognized as highly effective, minimally invasive treatment modalities for NENs, particularly pancreatic NENs. Advanced endoscopic resection techniques like endoscopic submucosal dissection (ESD), endoscopic submucosal resection (EMR), and endoscopic full-thickness resection (EFTR) have been increasingly used over the past decade with excellent results in achieving curative resection of various early-stage gastrointestinal luminal lesions including NENs. In this article, we aim to delineate NENs of the different segments of the gastrointestinal (GI) tract (esophagus, gastric, pancreatic, and small and large intestine) and their management with emphasis on the endoscopic management of these tumors.
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Affiliation(s)
- Harishankar Gopakumar
- Department of Gastroenterology and Hepatology, University of Illinois College of Medicine at Peoria, Peoria, IL 61605, USA;
| | - Vinay Jahagirdar
- Department of Internal Medicine, University of Missouri-Kansas City, Kansas City, MO 64110, USA; (V.J.); (J.K.)
| | - Jagadish Koyi
- Department of Internal Medicine, University of Missouri-Kansas City, Kansas City, MO 64110, USA; (V.J.); (J.K.)
| | - Dushyant Singh Dahiya
- Division of Gastroenterology, Hepatology & Motility, The University of Kansas School of Medicine, Kansas City, KS 66160, USA;
| | - Hemant Goyal
- Department of Surgery, Center for Interventional Gastroenterology at UT (iGUT), The University of Texas Health Science Center, Houston, TX 77054, USA;
| | - Neil R. Sharma
- Advanced Interventional Endoscopy & Endoscopic Oncology (IOSE) Division, GI Oncology Tumor Site Team, Parkview Cancer Institute, 11104 Parkview Circle, Suite 310, Fort Wayne, IN 46845, USA;
| | - Abhilash Perisetti
- Division of Gastroenterology and Hepatology, Kansas City Veteran Affairs, Kansas City, MO 64128, USA
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Patel P, Zaher EA, Sqour H. High-Grade Pure Esophageal Neuroendocrine Carcinoma Arising From Barrett's Mucosa: A Rare Phenomenon. Cureus 2023; 15:e40644. [PMID: 37476144 PMCID: PMC10355687 DOI: 10.7759/cureus.40644] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/18/2023] [Indexed: 07/22/2023] Open
Abstract
Esophageal neuroendocrine carcinoma (E-NEC) is a very rare neuroendocrine tumor. There are only a few case reports where pure esophageal NEC is found to be arising from Barett's mucosa. Here we present a case of high-grade pure E-NEC arising from Barrett's esophagus, which was metastasized to the liver at the time of diagnosis.
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Affiliation(s)
- Parth Patel
- Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
| | - Eli A Zaher
- Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
| | - Hasan Sqour
- Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
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10
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Awada H, Hajj Ali A, Bakhshwin A, Daw H. High-grade large cell neuroendocrine carcinoma of the esophagus: a case report and review of the literature. J Med Case Rep 2023; 17:144. [PMID: 37024958 PMCID: PMC10080843 DOI: 10.1186/s13256-023-03879-0] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/13/2022] [Accepted: 03/07/2023] [Indexed: 04/08/2023] Open
Abstract
BACKGROUND Neuroendocrine carcinomas are extremely rare in the esophagus as they represent less than 0.04% of all neuroendocrine tumors. To date, only 14 cases of poorly differentiated, high-grade esophageal NEC have been described in the literature. The majority of these patients presented with typical dysphagia symptoms. Due to its rarity, no standardized guidelines have been proposed to treat esophageal neuroendocrine carcinoma, although general recommendations suggest surgery with adjuvant chemoradiotherapy as the treatment of choice. CASE PRESENTATION A 67-year-old previously healthy White male presented with a year-long intermittent nonspecific retrosternal discomfort, with the absence of any other symptoms. Esophagogastroduodenoscopy revealed an ulcerative mass in his lower esophagus, with concern of malignancy. Endoscopic ultrasound-guided biopsy revealed poorly differentiated neuroendocrine carcinoma of the esophagus with metastasis to a diaphragmatic lymph node. He was treated with neoadjuvant chemoradiation followed by surgery, and he has been in remission for over 5 years. CONCLUSION Here, we review the literature and report a unique case of a patient with a vague presentation of esophageal neuroendocrine carcinoma as he enters his sixth year of survival following neoadjuvant chemoradiotherapy.
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Affiliation(s)
- Hussein Awada
- Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.
| | - Adel Hajj Ali
- Heart, Vascular & Thoracic Institute, Cleveland Clinic, Cleveland, OH, 44195, USA
| | - Ahmed Bakhshwin
- Pathology Department, Robert J. Tomsich Pathology & Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, 44195, USA
| | - Hamed Daw
- Hematology and Oncology, Cleveland Clinic Fairview Hospital, Cleveland, OH, 44111, USA
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11
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Hou G, Zhao N, Li F, Jing H, Zheng R. Prognostic value of pretreatment 18F-FDG PET/CT metabolic parameters in esophageal high-grade neuroendocrine carcinoma: A bicenter retrospective study. Front Oncol 2023; 13:1145557. [PMID: 36994200 PMCID: PMC10042323 DOI: 10.3389/fonc.2023.1145557] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/16/2023] [Accepted: 02/27/2023] [Indexed: 03/14/2023] Open
Abstract
ObjectiveThe aim of this bicentric retrospective study was to assess the prognostic value of 18F-FDG PET/CT in patients with esophageal high-grade neuroendocrine carcinoma (NECs).MethodsFrom the database of two centers, 28 patients affected by esophageal high-grade NECs who underwent 18F-FDG PET/CT before treatment were retrospectively reviewed. Metabolic parameters (SUVmax, SUVmean, tumor-to-blood-pool SUV ratio (TBR), tumor-to-liver SUV ratio (TLR), metabolic tumor volume (MTV), total lesion glycolysis (TLG)) of the primary tumor were measured. Univariate and multivariate analyses were performed for progression-free survival (PFS) and overall survival (OS).ResultsAfter a median follow-up period of 22 months, disease progression occurred in 11 (39.3%) patients, and death occurred in 8 (28.6%) patients. The median PFS was 34 months, and the median OS was not reached. Univariate analyses revealed that among metabolic parameters, only MTV and TLG were significant prognostic factors, while among clinical variables, only distant metastasis was a significant factor for both PFS and OS (P< 0.05). On multivariate analyses, MTV and TLG were independent prognostic factors for both PFS and OS (P< 0.05).ConclusionsIn patients with esophageal high-grade NEC, MTV and TLG measured on pretreatment 18F-FDG PET/CT are independently prognostic factors for predicting PFS and OS, and might be used as quantitative prognostic imaging biomarkers.
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Affiliation(s)
- Guozhu Hou
- Nuclear Medicine Department (PET-CT Center), National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Nuclear Medicine Department (PET-CT Center), National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Langfang, China
- Department of Nuclear Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Ningning Zhao
- Department of Nuclear Medicine, Weifang People’s Hospital, Weifang, Shandong, China
| | - Fang Li
- Department of Nuclear Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Hongli Jing
- Department of Nuclear Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- *Correspondence: Rong Zheng, ; Hongli Jing,
| | - Rong Zheng
- Nuclear Medicine Department (PET-CT Center), National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Nuclear Medicine Department (PET-CT Center), National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Langfang, China
- *Correspondence: Rong Zheng, ; Hongli Jing,
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12
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Poston LM, Gupta S, Alvarado CE, Sinopoli J, Vargas LT, Linden PA, Towe CW. Contemporary outcomes of esophageal and gastroesophageal junction neuroendocrine tumors. Dis Esophagus 2023:6995427. [PMID: 36688874 DOI: 10.1093/dote/doad001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/05/2022] [Revised: 11/28/2022] [Accepted: 12/31/2022] [Indexed: 01/24/2023]
Abstract
Adenocarcinoma and squamous cell esophageal cancers have been extensively studied in the literature. Esophageal neuroendocrine (NET)/carcinoid tumors are less commonly studied and have only been described in small series. The purpose of this study was to describe the demographics and natural history of esophageal NETs, as well as optimal treatments. We hypothesized that surgical resection would be the best treatment of esophageal NETs. The National Cancer Database was used to identify adult patients with esophageal or gastroesophageal junction (GEJ) cancer from 2004 to 2018. Patients were characterized as carcinoid/NET, adenocarcinoma, or squamous cell cancer. Clinical and demographic characteristics were compared between the histology groups. The primary outcome was overall survival, which was assessed by multivariable Cox analysis. Multivariable Cox analysis was also used to analyze factors associated with survival among NET patients who underwent surgery. Among 206,321 patients with esophageal cancer, 1,563 were NETs (<0.01%). Relative to the other two histologies, NETs were associated with younger age, female sex, and advanced clinical stage at diagnosis. Multivariate analysis suggested that NETs were less likely to be treated with surgical resection (OR 0.51, P < 0.001). Nonetheless, surgical resection was associated with improved survival (HR 0.64, P = 0.003). Among patients with NETs who received surgery, neoadjuvant therapy was associated with improved overall survival (HR 0.38, P = 0.013). NET of the esophagus presents with more advanced disease than other common histologies. Among patients with nonmetastatic cancer, surgical resection appears to be the best treatment. Neoadjuvant systemic therapy may offer survival benefit, but future studies are necessary.
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Affiliation(s)
- Lauren M Poston
- Case Western Reserve University School of Medicine, Cleveland, OH, USA
| | - Shreya Gupta
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Christine E Alvarado
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Jillian Sinopoli
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Leonidas T Vargas
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Philip A Linden
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Christopher W Towe
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
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13
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Nikolic AL, Gullifer J, Johnson MA, Hii MW. Oesophageal neuroendocrine tumours-case series of a rare malignancy. J Surg Case Rep 2022; 2022:rjac582. [PMID: 36570553 PMCID: PMC9769948 DOI: 10.1093/jscr/rjac582] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2022] [Accepted: 11/26/2022] [Indexed: 12/24/2022] Open
Abstract
Oesophageal neuroendocrine neoplasms (NENs) are rare tumours. Neuroendocrine carcinomas (NECs) are the highest grade of NENs, with aggressive biological behaviour and poor outcomes. No standardized treatment pathways exist for these tumours, with management being individualized based on patient and tumour factors. We present five cases, four men and one women between 63 and 68 years old, who were diagnosed with symptomatic primary oesophageal NECs. Three were diagnosed with localized disease, and two were diagnosed with metastatic disease. Endoscopy, biopsy and staging scan results are outlined. Two patients received neoadjuvant chemotherapy. Three patients with localized disease underwent oesophagectomy. Two of these patients received neoadjuvant chemotherapy. Four patients have succumbed to their disease, with a median survival following a diagnosis of 18 months (5-34 months). This case series highlights the variability of presentation and stage at diagnosis of oesophageal NECs. Multimodal treatment is commonly utilized; however, outcomes are universally poor. Further research is required to determine the optimal treatment regimen for oesophageal NENS.
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Affiliation(s)
- Amanda L Nikolic
- Correspondence address. St Vincent’s Public Hospital Melbourne, 41 Victoria Parade, Fitzroy 3065, Australia. E-mail:
| | - James Gullifer
- Department of Anatomical Pathology, St Vincent’s Hospital Melbourne, Fitzroy 3065, Australia
| | - Mary Ann Johnson
- Department of Hepatobiliary and Upper Gastrointestinal Surgery, St Vincent’s Hospital Melbourne, Fitzroy 3065, Australia
| | - Michael W Hii
- Department of Hepatobiliary and Upper Gastrointestinal Surgery, St Vincent’s Hospital Melbourne, Fitzroy 3065, Australia,The University of Melbourne, Department of Surgery, St Vincent’s Hospital Melbourne, Fitzroy 3065, Australia
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14
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Pavlidis ET, Pavlidis TE. Molecular factors, diagnosis and management of gastrointestinal tract neuroendocrine tumors: An update. World J Clin Cases 2022; 10:9573-9587. [PMID: 36186187 PMCID: PMC9516923 DOI: 10.12998/wjcc.v10.i27.9573] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/28/2022] [Revised: 07/16/2022] [Accepted: 08/17/2022] [Indexed: 02/05/2023] Open
Abstract
The prevalence of gastrointestinal neuroendocrine tumors (GI-NETs) is increasing, and despite recent advances in their therapy, it remains inadequate in patients with advanced well-differentiated neuroendocrine tumors. These tumors present many challenges concerning the molecular basis and genomic profile, pathophysiology, clinicopathological features, histopathologic classification, diagnosis and treatment. There has been an ongoing debate on diagnostic criteria and clinical behavior, and various changes have been made over the last few years. Neuroendocrine carcinoma of the gastrointestinal system is a rare but highly malignant neoplasm that is genetically distinct from gastrointestinal system neuroendocrine tumors (NETs). The diagnosis and management have changed over the past decade. Emerging novel biomarkers and metabolic players in cancer cells are useful and promising new diagnostic tools. Progress in positron emission tomography-computerized tomography and scintigraphy with new radioactive agents (64Cu-DOTATATE or 68Ga-DOTATATE) replacing enough octreoscan, has improved further the current diagnostic imaging. Promising results provide targeted therapies with biological agents, new drugs, chemotherapy and immunotherapy. However, the role of surgery is important, since it is the cornerstone of management. Simultaneous resection of small bowel NETs with synchronous liver metastases is a surgical challenge. Endoscopy offers novel options not only for diagnosis but also for interventional management. The therapeutic option should be individualized based on current multidisciplinary information.
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Affiliation(s)
- Efstathios Theodoros Pavlidis
- Department of 2nd Surgical Propedeutic, Hippocration Hospital, Aristotle University of Thessaloniki, School of Medicine, Thessaloniki 54642, Greece
| | - Theodoros Efstathios Pavlidis
- Department of 2nd Surgical Propedeutic, Aristotle University of Thessaloniki, School of Medicine, Thessaloniki 54642, Greece
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15
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Vest M, Shah D, Nassar M, Niknam N. Neuroendocrine Carcinoma of the Esophagus With Liver Metastasis: A Case Report. Cureus 2022; 14:e28842. [PMID: 36225482 PMCID: PMC9536476 DOI: 10.7759/cureus.28842] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/06/2022] [Indexed: 11/05/2022] Open
Abstract
Neuroendocrine carcinoma (NEC) of the esophagus is a rare and aggressive malignancy. It is challenging to manage NEC due to its rarity. NEC may be asymptomatic or present with various symptoms such as dysphagia, abdominal discomfort, weight loss, melena, hot flushes, or diarrhea. We present the case of a 55-year-old male with a large cell neuroendocrine carcinoma of the esophagus. His aggressive and rapid progression of neuroendocrine carcinoma of the esophagus resulted in a poor clinical outcome.
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16
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Kong ZZ, Zhang L. Esophagogastric junctional neuroendocrine tumor with adenocarcinoma: A case report. World J Clin Cases 2022; 10:6241-6246. [PMID: 35949855 PMCID: PMC9254174 DOI: 10.12998/wjcc.v10.i18.6241] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/30/2021] [Revised: 03/16/2022] [Accepted: 04/26/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND At present, cases of esophageal neuroendocrine tumors combined with cardia adenocarcinoma are extremely rare worldwide, and there are no clinical reports. Herein, we describe such a case for clinical reference.
CASE SUMMARY The presence of cardia cancer and esophageal neuroendocrine tumors in a single patient has not yet been reported. The patient in this case underwent prompt endoscopic treatment and additional surgical resection. Pathology revealed the following: The distance between the cardia cancer and the esophageal neuroendocrine tumors was small, approximately 3 mm. Vascular invasion was observed. The esophageal neuroendocrine tumor was determined to be grade G3. According to the treatment guidelines, after the patient received an explanation of their condition, additional surgical procedures were provided in a timely manner. Early detection and early treatment can successfully prolong survival and improve the quality of life of patients.
CONCLUSION Early detection and early treatment can successfully prolong survival and improve the quality of life of such patients.
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Affiliation(s)
- Zhen-Zhen Kong
- Department of Gastroenterology, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310000, Zhejiang Province, China
| | - Lu Zhang
- Department of Gastroenterology, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310000, Zhejiang Province, China
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17
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Chen C, Hu H, Zheng Z, Yang Y, Chen W, Qiao X, Li P, Zhang S. Clinical characteristics, prognostic factors, and survival trends in esophageal neuroendocrine carcinomas: A population-based study. Cancer Med 2022; 11:4935-4945. [PMID: 35596661 PMCID: PMC9761068 DOI: 10.1002/cam4.4829] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2022] [Revised: 04/14/2022] [Accepted: 05/01/2022] [Indexed: 02/03/2023] Open
Abstract
BACKGROUND Esophageal neuroendocrine carcinoma (ENEC) is an extremely rare type of malignancy. Clinical data of ENEC are limited to case reports and case series. More information is needed on its clinical feature, management, and prognosis. METHODS This study collected information of ENEC patients diagnosed pathologically from 2010 to 2018. Data including demographic information, clinical features, and survival trends were obtained from the Surveillance, Epidemiology, and End Results (SEER) database. Statistical analyses were performed with STATA/SE 15.1, SPSS 25.0, and GraphPad Prism 8. RESULTS A total of 283 ENEC patients were included in this study. The small-cell and large-cell subtypes of ENEC possess similar clinical features. The lower third of the esophagus (58%) was the most common location of ENEC. At the time of diagnosis, most ENEC patients were AJCC 7th stage IV (48.1%). Metastasis occurred in more than half of the ENEC patients (53.4%), and the most common metastatic site was the liver (37.1%). Compared with poorly differentiated esophageal squamous cell carcinoma (ESCC), another aggressive malignancy of the esophagus sometimes confused with ENEC because of similar histological features, our study showed differences in tumor location and metastatic rate, but similar poor survival rates. Multivariate survival analysis showed that ENEC located at the middle third of esophagus (p = 0.013), "Brain metastasis" (p = 0.019), and "Liver metastasis" (p < 0.001) were independent predictors of worse outcomes. "Surgery" (p = 0.003), and "Chemotherapy" (p < 0.001) were associated with better survival. CONCLUSION A significant proportion of patients with newly diagnosed ENEC presented with metastatic disease. Predictors of poor survival included tumor location, brain metastasis, and liver metastasis. ENEC and poorly differentiated ESCC share certain histological features, but differ in tumor location and metastatic rate. Yet, no standard treatment strategy has been established, but surgery and chemotherapy were related to better outcomes.
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Affiliation(s)
- Chuyan Chen
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Haiyi Hu
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Zhibo Zheng
- Department of International Medical ServicesPeking Union Medical College Hospital, Chinese Academy of Medical SciencesBeijingChina,Department of Thoracic SurgeryPeking Union Medical College Hospital, Chinese Academy of Medical SciencesBeijingChina
| | - Yi Yang
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Wei Chen
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Xinwei Qiao
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Peng Li
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Shutian Zhang
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
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18
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Borbath I, Pape UF, Deprez PH, Bartsch DK, Caplin M, Falconi M, Garcia-Carbonero R, Grozinsky-Glasberg S, Jensen RT, Arnold R, Ruszniewski P, Toumpanakis C, Valle JW, O Toole D. ENETS standardized (synoptic) reporting for endoscopy in neuroendocrine tumors. J Neuroendocrinol 2022; 34:e13105. [PMID: 35233848 DOI: 10.1111/jne.13105] [Citation(s) in RCA: 17] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2021] [Revised: 01/27/2022] [Accepted: 02/05/2022] [Indexed: 02/06/2023]
Abstract
Despite efforts from various endoscopy societies, reporting in the field of endoscopy remains extremely heterogeneous. Harmonisation of clinical practice in endoscopy has been highlighted by application of many clinical practice guidelines and standards pertaining to the endoscopic procedures and reporting are underlined. The aim of the proposed "standardised reporting" is to (1) facilitate recognition of gastrointestinal neuroendocrine neoplasms (NEN) on initial endoscopy, (2) to enable interdisciplinary decision making for treatment by a multidisciplinary team, (3) to provide a basis for a standardised endoscopic follow-up which allows detection of recurrence or progression reliably, (4) to make endoscopic reports on NEN comparable between different units, and (5) to allow research collaboration between NEN centres in terms of consistency of their endoscopic data. The ultimate goal is to improve disease management, patient outcome and reduce the diagnostic burden on the side of the patient by ensuring the highest possible diagnostic accuracy and validity of endoscopic exams and possibly interventions.
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Affiliation(s)
- Ivan Borbath
- Department of Hepato-Gastroenterology, Cliniques universitaires Saint-Luc, Bruxelles, Belgium
| | - Ulrich-Frank Pape
- Department of Internal Medicine and Gastroenterology, Asklepios Klinik St. Georg, Asklepios Tumorzentrum Hamburg, Hamburg, Germany
- Department of Hepatology and Gastroenterology, Charité Campus Mitte, Charité University Medicine Berlin, Berlin, Germany
| | - Pierre H Deprez
- Department of Hepato-Gastroenterology, Cliniques universitaires Saint-Luc, Bruxelles, Belgium
| | - Detlef Klaus Bartsch
- Department of Visceral-, Thoracic- and Vascular Surgery at the Philipps-University Marburg, Marburg, Germany
| | - Martyn Caplin
- Neuroendocrine Tumour Unit, ENETS Centre of Excellence, Royal Free Hospital and University College London, London, UK
| | - Massimo Falconi
- Department of Surgery, San Raffaele Hospital IRCCS, Università Vita-e-Salute, Milan, Italy
| | | | - Simona Grozinsky-Glasberg
- Neuroendocrine Tumor Unit, Division of Medicine, Endocrinology & Metabolism Department, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
| | - Robert T Jensen
- Gastrointestinal Cell Biology Section, Digestive Disease Branch, National Institute of Health, Bethesda, Maryland, USA
| | - Rudolf Arnold
- Zentrum für Innere Medizin, Universitätsklinikum Marburg, Marburg, Germany
| | - Philippe Ruszniewski
- Department of Gastroenterology-Pancreatology, Beaujon Hospital and Université de Paris, Clichy, France
| | - C Toumpanakis
- Neuroendocrine Tumour Unit, ENETS Centre of Excellence, Royal Free Hospital and University College London, London, UK
| | - Juan W Valle
- Department of Medical Oncology, University of Manchester/The Christie NHS Foundation Trust, Manchester, UK
| | - Dermot O Toole
- National Centre for Neuroendocrine Tumours, St Vincent's University Hospital and St James's Hospital and Trinity College Dublin, Dublin, Ireland
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19
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Galanis I, Simou M, Floros G. Large-Cell Esophageal Neuroendocrine Carcinoma: Report of a Rare Case. Cureus 2022; 14:e22041. [PMID: 35340509 PMCID: PMC8913815 DOI: 10.7759/cureus.22041] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/08/2022] [Indexed: 12/02/2022] Open
Abstract
Neuroendocrine tumors (NETs) are neoplasms with neuroendocrine characteristics such as secretion of neuropeptides, large secretory vesicles, and a lack of neural structures. Neuroendocrine carcinoma (NEC) of the esophagus is a very rare malignancy. We present the case of a 58-year-old male with a pure large cell NEC of the esophagus.
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20
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Basu S, Sitani K. High-grade esophageal neuroendocrine neoplasm with waxing and waning disease course and differential response to chemotherapy: Dual tracer positron emission tomography-computed tomography (18F-flurodeoxyglucose and 68Ga-DOTATATE) features and disease monitoring with functional molecular imaging. JOURNAL OF RADIATION AND CANCER RESEARCH 2022. [DOI: 10.4103/jrcr.jrcr_51_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
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21
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Rapidly Extensive Recurrence of Esophageal Neuroendocrine Carcinoma After Complete Pathologic Response to Definitive Chemoradiation. ACG Case Rep J 2022; 9:e00730. [PMID: 35083361 PMCID: PMC8785926 DOI: 10.14309/crj.0000000000000730] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/14/2021] [Accepted: 09/14/2021] [Indexed: 01/22/2023] Open
Abstract
Primary esophageal neuroendocrine carcinoma is a rare, aggressive malignancy lacking evidence-based treatment guidelines. The timing and nature of relapse after successful treatment of locoregional disease are not well characterized. We report a patient lacking risk factors for esophageal cancer who rapidly developed extensive disease recurrence 4 months after achieving complete pathologic response to nonsurgical treatment. Although optimal survival for early stage nonmetastatic disease is achieved by esophagectomy with adjuvant therapy, definitive chemotherapy is also appropriate for late stage nonmetastatic patients. There are presently no protocols for maintenance therapy. We highlight complex treatment considerations for this rare malignancy.
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22
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Branstetter H, Agarwal A, Paulson S, Nguyen AD, Konda V. Early esophageal neuroendocrine tumor. Proc AMIA Symp 2022; 35:80-81. [PMID: 34970043 DOI: 10.1080/08998280.2021.1955562] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022] Open
Abstract
Esophageal neuroendocrine tumors are rare and often found incidentally on endoscopy. We present a unique case of an esophageal neuroendocrine tumor found in the setting of dysplasia associated with Barrett's esophagus. The tumor was removed endoscopically. This case highlights the incidence, prognosis, and management of esophageal neuroendocrine tumors.
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Affiliation(s)
| | - Atin Agarwal
- Department of Clinical Pathology, Baylor University Medical Center, Dallas, Texas
| | | | - Anh D Nguyen
- Baylor Scott and White Center for Esophageal Diseases, Dallas, Texas
| | - Vani Konda
- Baylor Scott and White Center for Esophageal Diseases, Dallas, Texas
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23
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Liu S, Ge X, Gao Z, Zhou Q, Shi Y, Jiang W, Yang M, Sun X. Clinicopathological analysis of 67 cases of esophageal neuroendocrine carcinoma and the effect of postoperative adjuvant therapy on prognosis. Medicine (Baltimore) 2021; 100:e27302. [PMID: 34713821 PMCID: PMC8556034 DOI: 10.1097/md.0000000000027302] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/23/2020] [Accepted: 09/02/2021] [Indexed: 01/05/2023] Open
Abstract
The clinicopathological properties of esophageal neuroendocrine carcinoma (ENEC) and its optimal therapy have not been widely studied, as the disease is not common. Consequently, we conducted a retrospective study to analyze the clinical features as well as the prognosis of patients with surgically resected ENEC.The clinicopathological data of patients with ENEC who underwent esophagostomy with regional lymphadenectomy at Jiangsu Province People's Hospital and Jiangsu Provincial Tumor Hospital starting January 2008 until December 2014 were collected.Ninety-two cases of ENEC were part of this study. However, only 67 patients were analyzed and followed up. A univariate model for the Cox proportional hazards revealed that prognosis was associated with postoperative adjuvant therapy, age, and lymph node metastasis (P < .05); a multivariate Cox proportional hazards model showed that postoperative adjuvant therapy was a significant independent prognostic factor. Postoperative adjuvant therapy directly affected overall survival, with a significant disparity noted between the groups (P = .022). In this study, patients who received adjuvant therapy had an average time of survival of 39 months (interquartile range: 27.068-50.932 months), while those who did not receive adjuvant therapy had an average survival time of 13 months (interquartile range: 10.129-15.871 months). The survival time was longer in the treated group than in the untreated group (hazard ratio = 0.47; 95% confidence interval: 0.23-0.94; P = .034).ENEC is a heterogeneous tumor with a very poor prognosis. Combining surgery with adjuvant and/or chemotherapy significantly prolongs the survival of patients, and the optimal treatment for ENEC should be determined with future prospective studies.
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Affiliation(s)
- Shenxiang Liu
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
- Department of Oncology, The Affiliated Hospital of Yangzhou University, Yangzhou University, Jiangsu, China
| | - Xiaolin Ge
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
| | - Zhenzhen Gao
- Department of Oncology, The Second Affiliated Hospital of Jiaxing University, Zhe Jiang, China
| | - Qing Zhou
- Department of Oncology, Jiangsu Provincial Tumor Hospital, Jiangsu, China
| | - Yu Shi
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
| | - Wangrong Jiang
- Department of Radiotherapy, People's Liberation Army (PLA) 81 Hospital, Jiangsu, China
| | - Min Yang
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
- Jiangsu Institute of Nuclear Medicine, Jiangsu, China
| | - Xinchen Sun
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
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24
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Belvis Jiménez M, Guerra Veloz MF, Rodríguez-Téllez M. An esophageal neuroendocrine tumor in a Barrett's esophagus successfully treated with endoscopic resection. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2020; 112:885-886. [PMID: 33054288 DOI: 10.17235/reed.2020.6907/2020] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Neuroendocrine tumors rarely occur in the esophagus because the neuroendocrine system is not well developed in the esophagus. The case of a neuroendocrine esophageal tumor developed in a patient with Barret's esophagus is presented. It was successfully trated by endoscopy.
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25
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Li Z, Hu J, Chen P, Zeng Z. Incidence, treatment, and survival analysis in esophageal neuroendocrine carcinoma population. Transl Cancer Res 2020; 9:4317-4329. [PMID: 35117798 PMCID: PMC8797452 DOI: 10.21037/tcr-19-2650] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2019] [Accepted: 05/29/2020] [Indexed: 12/26/2022]
Abstract
BACKGROUND As a subtype of esophageal neuroendocrine neoplasms (ENENs), esophageal neuroendocrine carcinoma (ENEC) is rare. ENEC can be poorly differentiated and highly aggressive. This study aims to illustrate the incidence, treatment, and prognosis of ENEC by using a population-based database. METHODS We collected clinicopathological data [1975-2016] of ENEC, esophageal adenocarcinoma (EACA), and esophageal squamous cell carcinoma (ESqCC) from the Surveillance, Epidemiology and End Results (SEER) database, and analyzed their incidence, treatment, and prognosis. RESULTS A total of 60,238 cases were in our study cohort, including ENEC (n=686), EACA (n=26,575), and ESqCC (n=32,977). The incidence of ENEC in 2016 was 0.044 per 100,000 persons. The tumor grade of ENEC was significantly higher than that of EACA and ESqCC (P<0.001). Both cancer-specific survival (CSS) and overall survival (OS) of ENEC were significantly worse than those of EACA and ESqCC (P<0.001). Kaplan-Meier analysis showed significant improvement of CSS and OS by surgery, radiotherapy, and chemotherapy (all P<0.001). Besides, compared with monotherapies, combinational therapies brought more benefits to both CSS and OS of patients with ENEC. CONCLUSIONS Our population-based evidence revealed that ENEC, as a rare cancer, had a worse prognosis, compared with EACA and ESqCC. Surgery, chemotherapy, and radiotherapy can all improve the prognosis of ENEC patients. Combinational therapy can yield a better prognosis than monotherapy.
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Affiliation(s)
- Zhenhua Li
- Department of Cardiothoracic Surgery, Xianning Central Hospital, The First Affiliated Hospital of Hubei University of Science and Technology, Xianning, China
| | - Jiali Hu
- Department of Pathology, Jiujiang University Clinic College/Hospital, Jiujiang, China
| | - Pifeng Chen
- Department of Pediatric Surgery, Jiujiang Maternal and Child Health Hospital, Jiujiang, China
| | - Zhi Zeng
- Department of Pathology, Xianning Central Hospital, The First Affiliated Hospital of Hubei University of Science and Technology, Xianning, China
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26
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Wu IC, Chu YY, Wang YK, Tsai CL, Lin JC, Kuo CH, Shih HY, Chung CS, Hu ML, Sun WC, Wang JP, Wang HP. Clinicopathological features and outcome of esophageal neuroendocrine tumor: A retrospective multicenter survey by the digestive endoscopy society of Taiwan. J Formos Med Assoc 2020; 120:508-514. [PMID: 32600867 DOI: 10.1016/j.jfma.2020.06.024] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2020] [Revised: 05/15/2020] [Accepted: 06/21/2020] [Indexed: 12/22/2022] Open
Abstract
BACKGROUND & AIMS Esophageal neuroendocrine tumors (NET) are very rare and mostly carcinomic, carrying poor prognosis. There is still no guideline or consensus on the treatment for esophageal NET. METHODS Patients with histologically-proven esophageal neuroendocrine tumor were recruited from 9 hospitals in Taiwan between 2002 and 2017. Clinical, laboratory, radiological, endoscopic, pathological data, treatment strategies, follow-up periods, and survivals were collected retrospectively. RESULTS In total, 39 esophageal NET were analyzed and 38 were neuroendocrine carcinoma (NEC). Sixteen (41%) patients had mixed components with either adenocarcinoma (N = 9, 23%) or squamous-cell carcinoma (SCC) (N = 7, 18%). 64.1% of the patients experienced dysphagia and ulcerative mass was the most comment endoscopic finding. There was a higher proportion of drinkers (54.1%), betel chewers (21.6%) and smokers (64.9%) among the patients than in the general population in Taiwan. Five patients (12.8%) had been diagnosed with other cancers. Definite chemoradiotherapy (N = 14, 35.9%) and surgery (N = 7, 17.9%) were the major treatment. Patients with Ki-67% above the median level (50%) in the tumors tended to have worse survival (P = 0.06). However, presence of mixed component was not a significant survival predictor in our study (P = 0.56). CONCLUSION Mixed component of an esophageal NET is commonly observed. Staged workup and the principle of treatment can follow that for the common cancer type of esophagus. The risk factors and behaviors of esophageal NEC in Taiwan seem to be similar to that of esophageal SCC.
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Affiliation(s)
- I-Chen Wu
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; Department of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Yin-Yi Chu
- Department of Gastroenterology and Hepatology, Linkou Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan; Department of Gastroenterology and Hepatology, New Taipei Municipal TuCheng Hospital and Chang Gung University, New Taipei City, Taiwan
| | - Yao-Kuang Wang
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; Department of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Cho-Lun Tsai
- Department of Internal Medicine, Ditmanson Medical Foundation Chia-Yi Christian Hospital, Chia-Yi, Taiwan
| | - Jung-Chun Lin
- Division of Gastroenterology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
| | - Chao-Hung Kuo
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; Department of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Department of Internal Medicine, Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung, Taiwan
| | - Hsiang-Yao Shih
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
| | - Chen-Shuan Chung
- Department of Internal Medicine, Far Eastern Memorial Hospital, New Taipei City, Taiwan; Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University, College of Medicine, Taipei, Taiwan
| | - Ming-Luen Hu
- Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan
| | - Wei-Chih Sun
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Kaohsiung Veteran General Hospital, Kaohsiung, Taiwan
| | - Jack P Wang
- Division of Gastroenterology, Department of Internal Medicine, Taipei City Hospital, Renai Branch, Taipei, Taiwan
| | - Hsiu-Po Wang
- Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University, College of Medicine, Taipei, Taiwan.
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27
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Wang H, Chen Y, Pi G, Zhu Y, Yang S, Mei H, Lin Z, Zhang T. Validation and proposed modification of the 8th edition American Joint Committee on Cancer staging system for patients with esophageal neuroendocrine neoplasms: Evaluation of a revised lymph node classification. Oncol Lett 2020; 19:4122-4132. [PMID: 32382351 DOI: 10.3892/ol.2020.11480] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2019] [Accepted: 01/23/2020] [Indexed: 12/22/2022] Open
Abstract
There is currently no universally accepted staging system for esophageal neuroendocrine neoplasms (ENENs). In the present study, patients with ENENs, identified from the Surveillance, Epidemiology, and End Results registry (SEER) (n=191 patients) and the multicentric series (n=51 patients), were stratified to assess the validity of the 8th American Joint Committee on Cancer (AJCC) staging systems, particularly for esophageal squamous cell carcinoma and esophageal adenocarcinoma. The Kaplan-Meier method was used to assess disease-specific survival (DSS), according to the Tumor-Node-Metastasis (TNM) status, and the Cox model was applied to evaluate differences in prognosis after adjustment for potential confounders. For the 8th AJCC staging classifications, only the pathological stage groups (pTNM) conferred increased hazard ratios from stage I to stage IV, with overlaps between adjacent stages. According to the current findings, the regional lymph nodes involvement status other than the current N classification was a significant predictor of DSS. Consequently, a revised N(Nr) classification was proposed and therefore a new TNrM staging system was adopted, for which progressively poorer DSS associated with increasing stage was observed. Moreover, the concordance index with the modified staging system was slightly higher in patients with ENENs from the SEER registry compared with that of the 8th pTNM system. In conclusion, lymph node status, rather than the number of positive lymph nodes, was a marker of poorer DSS and the modified staging system provided an easier and more accurate staging tool. The present results indicate that revisions to the current staging classifications may be improve the assessment of patient prognosis.
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Affiliation(s)
- Haihong Wang
- Cancer Center, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430022, P.R. China
| | - Yaobing Chen
- Institute of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, P.R. China
| | - Guoliang Pi
- Department of Radiation Oncology, Hubei Cancer Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430079, P.R. China
| | - Ying Zhu
- Cancer Center, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430022, P.R. China
| | - Shengli Yang
- Cancer Center, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430022, P.R. China
| | - Hong Mei
- Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430022, P.R. China
| | - Zhenyu Lin
- Cancer Center, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430022, P.R. China
| | - Tao Zhang
- Cancer Center, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430022, P.R. China
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Zhang G, Wu B, Wang X, Li J. A competing-risks nomogram and recursive partitioning analysis for cause-specific mortality in patients with esophageal neuroendocrine carcinoma. Dis Esophagus 2019; 32:5304731. [PMID: 30715226 DOI: 10.1093/dote/doy129] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/16/2018] [Indexed: 12/11/2022]
Abstract
The objective of this study is to estimate the probability of cause-specific mortality using a competing-risks nomogram and recursive partitioning analysis in a large population-based cohort of patients with esophageal neuroendocrine carcinoma. The surveillance, epidemiology and end results database was used to identify 162 patients diagnosed with esophageal neuroendocrine carcinoma from 1998 to 2014. We estimated a cumulative incidence function for cause-specific mortality. A nomogram was constructed by using a proportional subdistribution hazard model, validated using bootstrap cross-validation, and evaluated with a decision curve analysis to assess its clinical utility. Finally, we performed risk stratification using a recursive partitioning analysis to divide patients with esophageal neuroendocrine carcinoma into clinically useful prognostic groups. Tumor location, distant metastasis, surgery, radiotherapy, and chemotherapy were significantly associated with cause-specific mortality. The calibration plots demonstrated good concordance between the predicted and actual outcomes. The discrimination performance of a Fine-Gray model was evaluated by using the c-index, which was 0.723 for cause-specific mortality. The decision curve analysis ranged from 0.268 to 0.968 for the threshold probability at which the risk model provided net clinical benefits relative to hypothetical all-screening and no-screening scenarios. The risk groups stratified by a recursive partitioning analysis allowed significant distinction between cumulative incidence curves. We determined the probability of cause-specific mortality in patients with esophageal neuroendocrine carcinoma and developed a nomogram and recursive partitioning analysis stratification system based on a competing-risks model. The nomogram and recursive partitioning analysis appear to be suitable for risk stratification of cause-specific mortality in patients with esophageal neuroendocrine carcinoma and will help clinicians to identify patients at increased risk of cause-specific mortality to guide treatment and surveillance decisions.
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Affiliation(s)
- G Zhang
- Department of Thoracic Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan Province, China
| | - B Wu
- Department of Thoracic Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan Province, China
| | - X Wang
- Department of Thoracic Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan Province, China
| | - J Li
- Department of Thoracic Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan Province, China
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29
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Ye L, Lu H, Wu L, Zhang L, Shi H, Wu HM, Tu P, Li DM, Wang FY. The clinicopathologic features and prognosis of esophageal neuroendocrine carcinomas: a single-center study of 53 resection cases. BMC Cancer 2019; 19:1234. [PMID: 31852479 PMCID: PMC6921601 DOI: 10.1186/s12885-019-6420-8] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2019] [Accepted: 12/01/2019] [Indexed: 01/08/2023] Open
Abstract
Background Esophageal neuroendocrine carcinomas (NECs) are exceedingly rare and poorly understood. The aims of the retrospective study were to delineate the clinicopathologic features and prognosis of patients with the disease. Methods We performed a retrospective study containing 53 patients of esophageal NECs in our center from 2002 through 2018. Patients were assigned to the pure esophageal NECs group and the esophageal NECs mixed with squamous carcinoma and/or esophageal adenocarcinoma (MiNECs) group. Demographic, clinical, pathologic and prognostic factors were recorded and analyzed. Results Of the 53 patients, elderly male patients were predominant. Dysphagia was the most common symptom (45/53, 84.9%). Most tumors were centered in the middle esophagus (36/53,67.9%).Ulcerated appearance was frequently seen in the pure NECs (56.8%), and the tumors in the MiNECs group mostly represented elevated types (57.9%). Synaptophysin (38/45, 84.4%), chromogranin A (21/38, 55.3%) and CD56(23/27, 85.2%) have been proven to be positive markers for NECs. Most patients (46/53, 86.8%) received surgery combined with chemotherapy. Though the pathologic stages were alike (P = 0.129), the median survival time was 3.53 years for the pure NECs group and 7 years for the MiNECs group. In multivariate analysis, pathologic stage (RR = 1.938, P = 0.045) and age (RR = 2.410, P = 0.028) were independent prognostic factors for patients with MiNECs. The prognosis of patients with pure NECs was independent from any factors. Conclusions Careful endoscopic examination could help distinguish pure NECs from MiNECs. NECs were aggressive, but a relative better prognosis for patients with MiNECs. Surgery should be performed if applicable, and chemotherapy might be helpful.
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Affiliation(s)
- Lei Ye
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Heng Lu
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Lin Wu
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Lei Zhang
- Department of cardiothoracic surgery, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Hui Shi
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Hui Min Wu
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Pin Tu
- Department of Pathology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - De Min Li
- Department of cardiothoracic surgery, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China.
| | - Fang Yu Wang
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China.
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Sanghi V, Amin H, Sanaka MR, Thota PN. Resection of early esophageal neoplasms: The pendulum swings from surgical to endoscopic management. World J Gastrointest Endosc 2019; 11:491-503. [PMID: 31798770 PMCID: PMC6885444 DOI: 10.4253/wjge.v11.i10.491] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/11/2019] [Revised: 08/09/2019] [Accepted: 09/11/2019] [Indexed: 02/06/2023] Open
Abstract
Esophageal cancer is a highly lethal disease and is the sixth leading cause of cancer related mortality in the world. The standard treatment is esophagectomy which is associated with significant morbidity and mortality. This led to development of minimally invasive, organ sparing endoscopic therapies which have comparable outcomes to esophagectomy in early cancer. These include endoscopic mucosal resection and endoscopic submucosal dissection. In early squamous cell cancer, endoscopic submucosal dissection is preferred as it is associated with cause specific 5-year survival rates of 100% for M1 and M2 tumors and 85% for M3 and SM1 tumors and low recurrence rates. In early adenocarcinoma, endoscopic resection of visible abnormalities is followed by ablation of the remaining flat Barrett’s mucosa to prevent recurrences. Radiofrequency ablation is the most widely used ablation modality with others being cryotherapy and argon plasma coagulation. Focal endoscopic mucosal resection followed by radiofrequency ablation leads to eradication of neoplasia in 93.4% of patients and eradication of intestinal metaplasia in 73.1% of patients. Innovative techniques such as submucosal tunneling with endoscopic resection are developed for management of submucosal tumors of the esophagus. This review includes a discussion of various endoscopic techniques and their clinical outcomes in early squamous cell cancer, adenocarcinoma and submucosal tumors. An overview of comparison between esophagectomy and endoscopic therapy are also presented.
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Affiliation(s)
- Vedha Sanghi
- Department of Internal Medicine, Cleveland Clinic, Cleveland, OH 44195, United States
| | - Hina Amin
- Department of Gastroenterology and Hepatology, Cleveland Clinic, Cleveland, OH 44195, United States
| | - Madhusudhan R Sanaka
- Department of Gastroenterology and Hepatology, Cleveland Clinic, Cleveland, OH 44195, United States
| | - Prashanthi N Thota
- Department of Gastroenterology and Hepatology, Cleveland Clinic, Cleveland, OH 44195, United States
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Erdem S, Troxler E, Warschkow R, Tsai C, Yerokun B, Schmied B, Stettler C, Blazer DG, Hartwig M, Worni M, Gloor B. Is There a Role for Surgery in Patients with Neuroendocrine Tumors of the Esophagus? A Contemporary View from the NCDB. Ann Surg Oncol 2019; 27:671-680. [PMID: 31605338 DOI: 10.1245/s10434-019-07847-1] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2019] [Indexed: 12/11/2022]
Abstract
BACKGROUND Esophageal neuroendocrine tumors (eNETs) are exceedingly rare, aggressive and have a poor prognosis. Treatment guidelines are ill-defined and mainly based on evidence from case reports and analogous experiences drawn from similar disease sites. METHODS The NCDB was reviewed for histologically confirmed stage I-III, primary eNETs from 2006 to 2014. Patients were grouped into whether or not they underwent primary tumor resection. Univariate, multivariable, and full bipartite propensity score (PS) adjusted Cox regression analyses were used to assess overall and relative survival differences. RESULTS A total of 250 patients were identified. Mean age was 65.0 (standard deviation [SD] 11.9) years, and 174 (69.6%) patients were male. Most patients had stage III disease (n = 136, 54.4%), and the most common type of NET was small cell eNET (n = 111, 44.4%). Chemotherapy was used in 186 (74.4%), radiation therapy in 178 (71.2%), and oncological resection was performed in 69 (27.6%) patients. Crude 2-year survival rates were higher in the operated (57.3%) compared with the nonoperated group (35.2%; p < 0.001). The survival benefit held true after multivariable adjustment (hazard ratio [HR] 0.47, 95% confidence interval [CI] 0.32-0.69, p < 0.001). After full bipartite PS adjustment analysis, survival was longer for patients who received a surgical resection compared with those who did not (HR 0.48, 95% CI 0.31-0.75, p = 0.003) with a corresponding 2-year overall survival rate of 63.3% (95% CI 52.0-77.2) versus 38.8% (95% CI 30.9-48.8), respectively. CONCLUSIONS Multimodal treatment that includes surgery is associated with better overall survival for eNETs. Additional research is needed to more definitively identify patients who benefit from esophagectomy and to establish an appropriate treatment algorithm.
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Affiliation(s)
- Suna Erdem
- Department of Visceral Surgery and Medicine, Inselspital, University of Bern, Bern, Switzerland
| | - Esther Troxler
- Department of Visceral Surgery and Medicine, Inselspital, University of Bern, Bern, Switzerland
| | - René Warschkow
- Department of Surgery, Kantonsspital St. Gallen, St. Gallen, Switzerland
| | - Catherine Tsai
- Department of Visceral Surgery and Medicine, Inselspital, University of Bern, Bern, Switzerland
| | | | - Bruno Schmied
- Department of Surgery, Kantonsspital St. Gallen, St. Gallen, Switzerland
| | - Christoph Stettler
- Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism, Inselspital, University of Bern, Bern, Switzerland
| | | | | | - Mathias Worni
- Duke University Medical Center, Durham, NC, USA. .,Clarunis, Department of Visceral Surgery, University Centre for Gastrointestinal and Liver Diseases, St. Clara Hospital and University Hospital Basel, Basel, Switzerland.
| | - Beat Gloor
- Department of Visceral Surgery and Medicine, Inselspital, University of Bern, Bern, Switzerland
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Findeis SK, Agarwal A. Incidental neuroendocrine tumor of the esophagus: A case report and review of the literature. HUMAN PATHOLOGY: CASE REPORTS 2019. [DOI: 10.1016/j.ehpc.2019.02.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
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Poorly Differentiated Esophageal Neuroendocrine Carcinoma Treated With the CDK4/6 Inhibitor, Palbociclib: A Case Report and Literature Review. Am J Ther 2019; 25:e595-e598. [PMID: 29232286 DOI: 10.1097/mjt.0000000000000610] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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Giannetta E, Guarnotta V, Rota F, de Cicco F, Grillo F, Colao A, Faggiano A. A rare rarity: Neuroendocrine tumor of the esophagus. Crit Rev Oncol Hematol 2019; 137:92-107. [PMID: 31014519 DOI: 10.1016/j.critrevonc.2019.02.012] [Citation(s) in RCA: 35] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/30/2018] [Revised: 08/08/2018] [Accepted: 02/26/2019] [Indexed: 01/09/2023] Open
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.
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Affiliation(s)
- Elisa Giannetta
- Dept. of Experimental Medicine, "Sapienza" University of Rome, Italy.
| | - Valentina Guarnotta
- Biomedical Department of Internal and Specialist Medicine (DIBIMIS), Section of Endocrine-Metabolic Diseases, University of Palermo, Italy
| | - Francesca Rota
- Unit of Endocrinology, San Camillo-Forlanini Hospital, Rome, Italy
| | - Federica de Cicco
- Department of Clinical Medicine and Surgery, University "Federico II", Naples, Italy
| | - Federica Grillo
- Pathology Unit, Department of Surgical Sciences and Integrated Diagnostics (DISC), University of Genova and Ospedale Policlinico San Martino, Genova, Italy
| | - Annamaria Colao
- Department of Clinical Medicine and Surgery, University "Federico II", Naples, Italy
| | - Antongiulio Faggiano
- Thyroid and Parathyroid Surgery Unit, Istituto Nazionale per lo Studio e la Cura dei Tumori "Fondazione G. Pascale", IRCCS, Naples, Italy
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Hu Q, Jin P, Zhao X, Wu W, Huang B, Shao S, Que R, Liang T. Esophageal neuroendocrine carcinoma complicated with unexpected hyperprocalcitonin: Case report and literature review. Medicine (Baltimore) 2018; 97:e12219. [PMID: 30290592 PMCID: PMC6200475 DOI: 10.1097/md.0000000000012219] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/08/2023] Open
Abstract
RATIONALE Neuroendocrine tumors (NETs) with hyperprocalcitonin are relatively rare with a low incidence rate. PATIENT CONCERNS An afebrile 63-year-old male with persistent low back pain unexpectedly presented with an extreme hyperprocalcitonin. Radiological assessment revealed thickening of the esophageal wall with vertebral bone destruction and liver lesions. Endoscopy showed an irregular-shaped esophageal lesion which turned out to be poorly-differentiated NETs. DIAGNOSIS Esophageal NETs with multiple metastases. INTERVENTIONS The patient was treated with chemotherapies, and was evaluated by procalcitonin level and radiology within follow-up. OUTCOME The procalcitonin levels were altered in line with the therapeutic response and disease progression during the treatment course. LESSONS Increased procalcitonin occurs in several malignancies with neuroendocrine components, such as NETs of the digestive system.
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Affiliation(s)
- Qida Hu
- Department of Hepatobiliary and Pancreatic Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou
| | - Piaopiao Jin
- Department of Gastroenterology, First Affiliated Hospital, Wenzhou Medical University, Wenzhou, China
| | - Xinyu Zhao
- Department of Hepatobiliary and Pancreatic Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou
| | - Wangteng Wu
- Department of Hepatobiliary and Pancreatic Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou
| | - Bingfeng Huang
- Department of Hepatobiliary and Pancreatic Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou
| | - Shiyi Shao
- Department of Hepatobiliary and Pancreatic Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou
| | - Risheng Que
- Department of Hepatobiliary and Pancreatic Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou
| | - Tingbo Liang
- Department of Hepatobiliary and Pancreatic Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou
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Fujihara S, Kobayashi M, Nishi M, Yachida T, Yoshitake A, Deguchi A, Muraoka A, Kobara H, Masaki T. Composite neuroendocrine carcinoma and squamous cell carcinoma with regional lymph node metastasis: a case report. J Med Case Rep 2018; 12:227. [PMID: 30139375 PMCID: PMC6108124 DOI: 10.1186/s13256-018-1775-z] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2018] [Accepted: 07/18/2018] [Indexed: 12/11/2022] Open
Abstract
Background Neuroendocrine cell carcinoma is a rare variant of esophageal carcinoma. The characteristic clinical features and diagnosis of superficial neuroendocrine cell carcinoma remain to be established. We report a rare case of superficial coexistence of neuroendocrine cell carcinoma with squamous cell carcinoma treated by endoscopic submucosal dissection, and regional lymph node metastasis was detected after additional surgical treatment. Case presentation A 77-year-old Japanese man with esophageal squamous cell carcinoma received endoscopic submucosal dissection in en-bloc resection. Histopathological findings showed that lymphovascular invasion by the neuroendocrine cell carcinoma component occurred in the deep part of the muscularis mucosa. Regional lymph node metastasis was identified after additional surgical treatment. After surgical treatment, our patient received chemotherapy consisting of etoposide and carboplatin for 3 months. He is alive and shows no sign of disease recurrence 12 months after surgery. Conclusions This case report highlights the fact that even if neuroendocrine cell carcinoma is small and limited to superficial, the tumor has the potential for metastasis if lymphovascular invasion by the neuroendocrine cell carcinoma component occurs. In addition, this case indicates the necessity of close follow-up of small neuroendocrine cell carcinoma after treatment.
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Affiliation(s)
- Shintaro Fujihara
- Department of Gastroenterology, Kagawa Rosai Hospital, 3-3-1, Joutou, Marugame, Kagawa, Japan.
| | | | - Masako Nishi
- Department of Gastroenterology, Kagawa Rosai Hospital, 3-3-1, Joutou, Marugame, Kagawa, Japan
| | - Tatsuo Yachida
- Department of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Miki, Kagawa, Japan
| | - Akira Yoshitake
- Department of Gastroenterology, Kagawa Rosai Hospital, 3-3-1, Joutou, Marugame, Kagawa, Japan
| | - Akihiro Deguchi
- Department of Gastroenterology, Kagawa Rosai Hospital, 3-3-1, Joutou, Marugame, Kagawa, Japan
| | - Atsushi Muraoka
- Department of Surgery, Kagawa Rosai Hospital, Marugame, Kagawa, Japan
| | - Hideki Kobara
- Department of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Miki, Kagawa, Japan
| | - Tsutomu Masaki
- Department of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Miki, Kagawa, Japan
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Watson GA, Ahmed Y, Picardo S, Chew S, Cobbe S, Mahony C, Crotty J, Wallis F, Shelly MJ, Kiely P, Ipadeola OB, Healy V, Osman N, Gupta RK. Unusual Sites of High-Grade Neuroendocrine Carcinomas: A Case Series and Review of the Literature. AMERICAN JOURNAL OF CASE REPORTS 2018; 19:710-723. [PMID: 29915166 PMCID: PMC6044230 DOI: 10.12659/ajcr.908953] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Case series Patient: Female, 29 • Female, 69 • Female, 52 • Female, 71 • Male, 62 • Female, 67 Final Diagnosis: Neuroendocrine carcinoma Symptoms: Abdominal pain Medication: — Clinical Procedure: — Specialty: Oncology
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Affiliation(s)
- Geoffrey A Watson
- Department of Medical Oncology, University Hospital Limerick, Limerick, Ireland
| | - Yasar Ahmed
- Department of Medical Oncology, University Hospital Limerick, Limerick, Ireland
| | - Sarah Picardo
- Department of Medical Oncology, University Hospital Limerick, Limerick, Ireland
| | - Sonya Chew
- Department of Medical Oncology, University Hospital Limerick, Limerick, Ireland
| | - Shona Cobbe
- Graduate Entry Medical School, University Hospital Limerick, Limerick, Ireland
| | - Cillian Mahony
- Graduate Entry Medical School, University Hospital Limerick, Limerick, Ireland
| | - James Crotty
- Department of Radiology, University Hospital Limerick, Limerick, Ireland
| | - Fintan Wallis
- Department of Radiology, University Hospital Limerick, Limerick, Ireland
| | - Martin J Shelly
- Department of Radiology, University Hospital Limerick, Limerick, Ireland
| | - Patrick Kiely
- Department of Radiology, University Hospital Limerick, Limerick, Ireland
| | - Olu Bunmi Ipadeola
- Department of Histopathology, University Hospital Limerick, Limerick, Ireland
| | - Vourneen Healy
- Department of Histopathology, University Hospital Limerick, Limerick, Ireland
| | - Nemer Osman
- Department of Medical Oncology, University Hospital Limerick, Limerick, Ireland
| | - Rajnish K Gupta
- Department of Medical Oncology, University Hospital Limerick, Limerick, Ireland.,Graduate Entry Medical School, University Hospital Limerick, Limerick, Ireland
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Schizas D, Mastoraki A, Kirkilesis GI, Sioulas AD, Papanikolaou IS, Misiakos EP, Arkadopoulos N, Liakakos T. Neuroendocrine Tumors of the Esophagus: State of the Art in Diagnostic and Therapeutic Management. J Gastrointest Cancer 2018; 48:299-304. [PMID: 28656561 DOI: 10.1007/s12029-017-9978-x] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
INTRODUCTION Neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms composed of cells containing dense-core neuroendocrine secretory granules in their cytoplasm. NETs of the esophagus are exceedingly uncommon, with a parallel absence of data published on clinical features, prognosis, and proposed treatment strategies. METHODS As relevant classification is not well-established, knowledge acquired in NETs of lung and gastrointestinal sites usually guides esophageal NET management. Associated subtypes are divided based upon shared neuroendocrine features into small and large cell NET, typical and atypical carcinoid. RESULTS Common presenting symptoms include dysphagia, abdominal discomfort, weight loss, melena, and on occasion, signs of carcinoid syndrome. Endoscopic findings describe a polypoid, nodular elevated lesion with an overlying surface depicted as mostly smooth and glistening. Disease metastasis is assessed using anatomical imaging, including computed tomography (CT), endoscopic ultrasonography (EUS), and positron emission tomography (PET)-CT. Prognosis is influenced by the extent of lymph node metastasis and potential lymphovascular invasion. Furthermore, proliferative activity, estimated using mitotic count or Ki-67 immunostaining, has been suggested as a significant prognostic parameter. CONCLUSION Therapeutic approach depends on clinical staging. Nevertheless, currently, a specific treatment algorithm for esophageal NETs has not been elucidated. Endoscopic resection has been proposed in NETs less than 1 cm in size with absence of regional lymph node metastasis, while surgical excision combined with adjuvant chemotherapy remains the treatment of choice.
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Affiliation(s)
- Dimitrios Schizas
- 1st Department of Surgery, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece
| | - Aikaterini Mastoraki
- 4th Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Chaidari, Athens, Greece.
| | - George I Kirkilesis
- 1st Department of Surgery, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece
| | - Athanasios D Sioulas
- 3rd Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Chaidari, Athens, Greece
| | - Ioannis S Papanikolaou
- 4th Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Chaidari, Athens, Greece
| | - Evangelos P Misiakos
- 3rd Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Chaidari, Athens, Greece
| | - Nikolaos Arkadopoulos
- 4th Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Chaidari, Athens, Greece
| | - Theodore Liakakos
- 1st Department of Surgery, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece
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Savva C, Kaye P, Soomro I, Parsons SL, James E, Madhusudan S. Primary Esophagogastric Neuroendocrine Carcinoma: a Retrospective Study from the Nottingham Upper Gastrointestinal Cancer Center. J Gastrointest Cancer 2017; 49:85-92. [PMID: 29230683 DOI: 10.1007/s12029-017-0039-2] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Affiliation(s)
- Constantinos Savva
- Department of Oncology, Nottingham University Hospitals, Nottingham, NG5 1PB, UK
| | - Philip Kaye
- Department of Pathology, Nottingham University Hospitals, Nottingham, NG5 1PB, UK
| | - Irshad Soomro
- Department of Pathology, Nottingham University Hospitals, Nottingham, NG5 1PB, UK
| | - Simon L Parsons
- Department of Surgery, Nottingham University Hospitals, Nottingham, NG5 1PB, UK
| | - Eleanor James
- Department of Oncology, Nottingham University Hospitals, Nottingham, NG5 1PB, UK
| | - Srinivasan Madhusudan
- Department of Oncology, Nottingham University Hospitals, Nottingham, NG5 1PB, UK. .,Translational Oncology, Division of Cancer and Stem Cells, School of Medicine, University of Nottingham, Nottingham, NG5 1PB, UK.
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40
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Chin YP, Lai WF, Chiang MT, Chang SC. Esophageal neuroendocrine tumor with initial presentation as painless forehead and neck masses: A case report. Medicine (Baltimore) 2017; 96:e9282. [PMID: 29390385 PMCID: PMC5815797 DOI: 10.1097/md.0000000000009282] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
Abstract
RATIONALE Esophageal neuroendocrine tumors (NETs) are a rare type of esophageal tumor which are usually positive for chromogranin A, synaptophysin, and CD56 in tumor immunohistochemical staining. The most common symptoms of esophageal NETs are gastrointestinal symptoms such as dysphagia and/or abdominal discomfort. While esophageal NETs have the potential for distant metastasis, there have only been a few reports of brain metastasis originating from esophageal NET. PATIENT CONCERNS We report the case of a 60-year-old Taiwanese female who initially presented with a 1 month history of painless forehead and bilateral neck masses. She did not complain of any other symptoms, which complicated the diagnosis. DIAGNOSES Chest and abdominal computed tomography were arranged for a thorough evaluation, and a paraesophageal lesion as well as multiple metastases in the liver, bilateral adrenal glands, and bone were found. Panendoscopy and pathology confirmed the diagnosis of an esophageal NET. INTERVENTIONS Best supportive care. OUTCOMES The clinical course of this case deteriorated drastically, and she died of tumor progression 10 days after the diagnosis had been made. LESSONS To the best of our knowledge, this is the first article in the literature to report a case of esophageal NET whose initial presentation was painless forehead and bilateral neck masses. Clinicians should be aware of the early signs and symptoms of esophageal NET to allow for a prompt diagnosis.
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Affiliation(s)
- Yen-Po Chin
- Faculty of Medicine, National Yang-Ming University, Taipei
| | - Wei-Fu Lai
- Faculty of Medicine, National Yang-Ming University, Taipei
| | | | - Shih-Chieh Chang
- Faculty of Medicine, National Yang-Ming University, Taipei
- Division of Chest Medicine, Department of Internal Medicine, National Yang-Ming University Hospital, Yilan, Taiwan
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Chin JL, O'Toole D. Diagnosis and Management of Upper Gastrointestinal Neuroendocrine Tumors. Clin Endosc 2017; 50:520-529. [PMID: 29207862 PMCID: PMC5719910 DOI: 10.5946/ce.2017.181] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2017] [Accepted: 11/19/2017] [Indexed: 12/14/2022] Open
Abstract
Upper gastrointestinal neuroendocrine tumors (NETs) are rare tumors which are increasingly recognised by practising endoscopists. After confirmation by endoscopic biopsies of these focal lesions, many questions may arise. As NETs are less frequently encountered compared to other malignancies or gastrointestinal pathology, many endoscopists may not fully understand the natural history, diagnosis and management of these tumors. In this review, we aim to update the practising endoscopist on the key clinical features and management of patients with upper gastrointestinal NET.
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Affiliation(s)
- Jun Liong Chin
- Department of Gastroenterology, St. Vincent's University Hospital, University College Dublin, Dublin, Ireland
| | - Dermot O'Toole
- Department of Gastroenterology, St. Vincent's University Hospital, University College Dublin, Dublin, Ireland.,Department of Clinical Medicine, Trinity College Dublin, The University of Dublin, Dublin, Ireland
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42
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Ma Z, Cai H, Cui Y. Progress in the treatment of esophageal neuroendocrine carcinoma. Tumour Biol 2017; 39:1010428317711313. [PMID: 28653897 DOI: 10.1177/1010428317711313] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/14/2023] Open
Abstract
Esophageal neuroendocrine neoplasms are rare. With the improvement and popularization of diagnostic methods, the morbidity statistics have increased annually in recent years. There are currently no treatment guidelines for esophageal neuroendocrine neoplasms, and surgery is the only cure. This usually involves radical surgery when the tumor is limited to the primary site or when only regional lymph node metastasis occurs. Surgical treatment is key to treating esophageal neuroendocrine neoplasms, but combined treatment with chemotherapy and radiotherapy can significantly improve patient survival. The effect of radiotherapy alone on this disease is poor. However, targeted endocrine therapy can improve endocrine hormone symptoms. The prognosis of patients with esophageal neuroendocrine neoplasms is mainly determined by the pathological stage. With the development of molecular biology techniques, the combination of targeted drugs and traditional chemotherapy is expected to provide novel ideas and directions for the treatment of esophageal neuroendocrine neoplasms in the coming years. In this article, the status of esophageal neuroendocrine tumor treatments was reviewed in detail.
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Affiliation(s)
- Zheng Ma
- Department of Thoracic Surgery, The First Hospital of Jilin University, Changchun, China
| | - Hongfei Cai
- Department of Thoracic Surgery, The First Hospital of Jilin University, Changchun, China
| | - Youbin Cui
- Department of Thoracic Surgery, The First Hospital of Jilin University, Changchun, China
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Tustumi F, Takeda FR, Uema RH, Pereira GLS, Sallum RAA, Cecconello I. Primary neuroendocrine neoplasm of the esophagus - Report of 14 cases from a single institute and review of the literature. ARQUIVOS DE GASTROENTEROLOGIA 2017; 54:4-10. [PMID: 28079231 DOI: 10.1590/s0004-2803.2017v54n1-01] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/09/2016] [Accepted: 08/22/2016] [Indexed: 02/08/2023]
Abstract
BACKGROUND: Most prevalent esophageal neoplasm is squamous cell carcinoma and adenocarcinoma. Other tumors are uncommon and poorly studied. Primary neuroendocrine esophageal neoplasm is a rare carcinoma and most of its therapy management is based on lung neuroendocrine studies. Neuroendocrine tumors can be clustered in the following subtypes: high grade (small cell carcinoma or large cell carcinoma) and low grade (carcinoids). OBJECTIVE: The present study aims to assess clinical and pathological neuroendocrine esophageal tumors in a single oncologic center. METHODS: A retrospective analysis of patients and review of the literatures was performed. RESULTS: Fourteen patients were identified as neuroendocrine tumors, 11 male and 3 female patients. Mean age was 67.3 years old. Ten patients were classified as small cell, 3 as large cell and 1 as carcinoid. Four patients presented squamous cell carcinoma simultaneously and 1 also presented adenocarcinoma. Main sites of metastasis were liver, peritoneum, lung and bones. Most patients died before 2 years of follow-up. Patient with longer survival died at 35 months after diagnosis. CONCLUSION: Neuroendocrine esophageal tumors are rare; affect mainly men in their sixties or seventies. High grade tumors can be mixed to other subtypes neoplasms, such as adenocarcinoma and squamous cell carcinoma. Most of these patients have poor overall survival rates.
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Affiliation(s)
- Francisco Tustumi
- Cirurgia do Aparelho Digestivo, Faculdade de Medicina, Hospital das Clínicas, USP, SP, Brasil
| | | | - Rodrigo Hideki Uema
- Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, SP, Brasil
| | | | | | - Ivan Cecconello
- Cirurgia do Aparelho Digestivo, Faculdade de Medicina, Hospital das Clínicas, USP, SP, Brasil
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Deng HY, Ni PZ, Wang YC, Wang WP, Chen LQ. Neuroendocrine carcinoma of the esophagus: clinical characteristics and prognostic evaluation of 49 cases with surgical resection. J Thorac Dis 2016; 8:1250-6. [PMID: 27293844 DOI: 10.21037/jtd.2016.04.21] [Citation(s) in RCA: 46] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
BACKGROUND The clinicopathological features and optimum treatment of esophageal neuroendocrine carcinoma (NEC) are hardly known due to its rarity. Therefore, we conducted a retrospective study to analyze the clinical characteristics and prognosis of patients with surgically resected esophageal NEC. METHODS We collected clinicopathological data on consecutive limited disease stage esophageal NEC patients who underwent esophagectomy with regional lymphadenectomy in West China Hospital from January 2007 to December 2013. RESULTS A total of forty-nine patients were analyzed retrospectively. The mean age of the patients was 58.4±8.2 years with male predominance. Fifty-five percent of the esophageal NEC were located in the middle thoracic esophagus. Histologically, 28 (57.1%) patients were found to be small cell NECs. Fifty-one percent of the patients were found to have lymph node metastasis. According to the 2009 American Joint Committee on Cancer (AJCC) staging system for esophageal squamous cell carcinoma, 9 patients were at stage I, 21 patients stage II, and 19 patients stage III. Twenty-six patients (53.1%) received adjuvant therapy. After a median follow-up of 44.8 months [95% confidence interval (CI), 35.2-50.4 months], the median survival time of the patients was 22.4 months (95% CI, 14.0-30.8 months). The 1-year and 3-year survival rates for the whole cohort patients were 74.9% and 35.3%, respectively. In univariate analysis, TNM staging, lymph node metastasis and adjutant therapy significantly influenced survival time. In multivariate analysis, TNM staging was the only independent prognostic factor. CONCLUSIONS Esophageal NEC has a poor prognosis. The 2009 AJCC TNM staging system for esophageal squamous cell carcinoma may also fit for esophageal NEC. Surgery combined with adjuvant therapy may be a good option for treating limited disease stage esophageal NEC. Further prospective studies defining the optimum therapeutic regimen for esophageal NEC are needed.
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Affiliation(s)
- Han-Yu Deng
- Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China
| | - Peng-Zhi Ni
- Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China
| | - Yun-Cang Wang
- Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China
| | - Wen-Ping Wang
- Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China
| | - Long-Qi Chen
- Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China
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Clinicopathological Profile of Pure Neuroendocrine Neoplasms of the Esophagus: A South Indian Center Experience. JOURNAL OF ONCOLOGY 2016; 2016:2402417. [PMID: 27340404 PMCID: PMC4906204 DOI: 10.1155/2016/2402417] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 12/23/2015] [Accepted: 03/10/2016] [Indexed: 12/19/2022]
Abstract
Purpose. Neuroendocrine neoplasms (NENs) of the esophagus are very uncommon with only a few studies published worldwide. Studies on clinical profile, management, and outcomes are very uncommon. Methods. We report the largest single institution retrospective review of 43 patients of pure esophageal NENs out of our registry of gastrointestinal neuroendocrine tumors treated between 2005 and 2014. Data on the incidence, tumor location, clinical symptoms, stage at presentation, grading, treatment protocol, and treatment outcomes was collected and analyzed. Results. Among 1293 cases of esophageal cancers, pure esophageal NENs were diagnosed in 43 cases. The mean patient age was 55.8 years. The male : female ratio was 1.5 : 1. 81.4% of the tumors were located in the lower third of the esophagus and gastroesophageal junction. Neuroendocrine carcinomas (NEC; G3) accounted for the vast majority of NENs (83.7%). 53.5% patients were Stage IV and 32.5% were Stage III at presentation. The combined median survival of stages II and III patients was 18.25 months, with treatment. The median survival of treated patients with metastatic disease was 6.5 months. Conclusion. Esophageal NENs most commonly were neuroendocrine carcinomas, presented in metastatic stage and were associated with poor prognosis. Grade 2 (G2) tumors had better outcomes than NEC (G3). In nonmetastatic disease, presence of lymph node metastasis and unresectable disease had poorer outcomes.
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46
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Concurrent Lesions in Oesophagus: an Approach to Diagnosis with a Case Report. J Gastrointest Cancer 2016; 48:201-204. [PMID: 27105636 DOI: 10.1007/s12029-016-9822-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
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47
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Kim MH, Jeong HY, Seong JK, Moon HS, Kang SH, Kim DK. A Case of Endoscopically Complete Remission of Esophageal Neuroendocrine Tumors by Concurrent Chemoradiation Therapy. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2016; 68:265-269. [DOI: 10.4166/kjg.2016.68.5.265] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/11/2023]
Affiliation(s)
- Myung Hee Kim
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Hyun Yong Jeong
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Jae Kyu Seong
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Hee Seok Moon
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Sun Hyung Kang
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
| | - Duk Ki Kim
- Department of Internal Medicine, Chungnam National University School of Medicine, Daejeon, Korea
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Tao H, Li F, Wang J, Dong W, Gao J, Jiao S, Hu Y. Management of treatment-naïve limited-stage small cell esophagus carcinoma. Saudi Med J 2015; 36:297-303. [PMID: 25737171 PMCID: PMC4381013 DOI: 10.15537/smj.2015.3.11368] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
Objectives: To identify the problems and principles of treatment decisions in treatment-naïve limited-stage small cell esophagus carcinoma (LD-SCEC). Methods: Clinical data from 39 patients with LD-SCEC treated in the Chinese People’s Liberation Army General Hospital, Beijing, China between 2000 and 2013 were retrospectively collected with regard to pathologic characteristics, overall survival (OS), and relevant prognostic factors. Results: The median OS was 21.1 months (95% confidence interval [CI]: 12.4-29.7 months). The one-year OS was 76%, 3-year was 25%, and the 5-year OS was 8%. Depth of invasion, lymph metastasis status, and chemotherapy were independent prognostic factors. Of the 39 cases, only 38.4% (15 cases) were diagnosed as SCEC by the biopsy specimen. Eight of the 15 patients (group A) received chemotherapy and/or radiotherapy, while the remaining 7 patients (group B) and the other 24 patients (group C) received surgery as initial treatment. The one-year survival of group A was 87%, of group B was 69%, and of group C was 74% (p=0.037). The accuracy of the biopsy diagnosis influenced the treatment decisions and prognosis. Conclusion: Small cell esophagus carcinoma is a systemic disease, with depth of invasion, lymph metastasis status, and chemotherapy as independent prognostic factors. Systemic therapy based on chemotherapy is recommended. The top priority is to improve the accuracy of diagnosis before deciding on the initial treatment option.
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Affiliation(s)
- Haitao Tao
- Department of Oncology, Chinese People's Liberation Army General Hospital, Beijing, China. E-mail.
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Kuriry H, Swied AM. Large-Cell Neuroendocrine Carcinoma of the Esophagus: A Case from Saudi Arabia. Case Rep Gastroenterol 2015; 9:327-34. [PMID: 26600769 PMCID: PMC4649715 DOI: 10.1159/000441381] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/19/2022] Open
Abstract
Neuroendocrine carcinomas of the esophagus are very rare, and the majority are high grade (poorly differentiated). They occur most frequently in males in their sixth and seventh decades of life. There have been no concrete data published on clinical features or on prognosis. We report a case of large-cell neuroendocrine carcinoma of the esophagus in a 66-year-old Saudi female with progressive dysphagia and weight loss. Upper endoscopy revealed an esophageal ulcerated mass.
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Affiliation(s)
- Hadi Kuriry
- King Abdulaziz Medical City, Riyadh, Saudi Arabia
| | - Abdul Monem Swied
- King Saud bin Abdulaziz University for Health Science, Riyadh, Saudi Arabia
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50
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Surgical Resection of Rare Esophageal Cancers. Ann Thorac Surg 2015; 101:311-5. [PMID: 26277558 DOI: 10.1016/j.athoracsur.2015.06.015] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/22/2015] [Revised: 04/22/2015] [Accepted: 06/01/2015] [Indexed: 01/08/2023]
Abstract
BACKGROUND Although surgical resection of adenocarcinoma or squamous cell carcinoma of the esophagus is standard practice, the treatment strategy for other malignant rare esophageal cancers is still under debate. The aim of this study was to examine the treatment of rare malignant esophageal cancers and to evaluate the survival of these patients. METHODS A retrospective review of all esophagectomies performed at Mayo Clinic from 1980 to 2014 (approximately 4,000 cases) identified 24 patients with histologic features other than adenocarcinoma or squamous cell carcinoma. Their medical records were reviewed for demographics, presenting symptoms, evaluation, surgical management, pathologic features, and short-term and long-term outcome. RESULTS Pathologic identifications included small cell carcinoma, lymphoma, and undifferentiated carcinoma in 4 (16.7%) patients each and neuroendocrine, melanoma, leiomyosarcoma, sarcomatoid, sarcoma, and gastrointestinal stromal tumor in 2 (8.3%) patients each. The most common presenting symptoms included dysphasia in 91.7% patients (22/24), pain in 75.0% (18/24), and weight loss in 62.5% (15/24). Preoperative evaluation included barium swallow in 91.7% (22/24), computed tomography in 91.7% (22/24), positron emission tomography in 54.2% (13/24), esophagogastroduodenoscopy in 100% (24/24), and endoscopic ultrasonography in 29.2% (7/24) patients. The location of the tumor was at the gastroesophageal junction in 41.7% (10/24). There was no operative mortality, and 13 patients (54.16%) had at least one postoperative adverse event. The 1-year survival after esophagectomy was 69.7%, the 5-year survival was 42.7%, and the 10-year survival was 37.4%. CONCLUSIONS Esophageal cancer with pathologic features other than squamous cell carcinoma or adenocarcinoma is rare. Esophagectomy for rare types of malignant esophageal cancers should be considered part of the effective treatment paradigm.
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