title: Clinicopathological Profile of Pure Neuroendocrine Neoplasms of the Esophagus: A South Indian Center Experience.
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.
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Govind
authors.initials: G
authors.lastname: Babu Kanakasetty
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Loknatha
authors.initials: L
authors.lastname: Dasappa
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Kuntegowdanahalli Chinnagiriyappa
authors.initials: KC
authors.lastname: Lakshmaiah
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Mangesh
authors.initials: M
authors.lastname: Kamath
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Linu Abraham
authors.initials: LA
authors.lastname: Jacob
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Suresh Babu
authors.initials: SB
authors.lastname: Mallekavu
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Lakkavalli Krishnappa
authors.initials: LK
authors.lastname: Rajeev
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Rudresha Antapura
authors.initials: RA
authors.lastname: Haleshappa
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Lokesh
authors.initials: L
authors.lastname: Kadabur Nagendrappa
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Smitha Carol
authors.initials: SC
authors.lastname: Saldanha
authors.affiliation: Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, No. 30, Bangalore 560029, India.
authors.forename: Rekha V
authors.initials: RV
authors.lastname: Kumar