Esophageal neuroendocrine tumor with initial presentation as painless forehead and neck masses

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.

Esophagogastric junctional neuroendocrine tumor with adenocarcinoma: A case report

“…They can occur in many parts of the body but are most often found in the digestive system, followed by the lungs. E-NETs are very rare[ 1 ], accounting for only 1.4% of all gastrointestinal pancreatic tumors[ 2 ] and 0.15%-2.80% of all esophageal tumors[ 3 ]. This is due to the poor development of the neuroendocrine system in this area of the body[ 2 ].…”

Relation between WHO Classification and Location- and Functionality-Based Classifications of Neuroendocrine Neoplasms of the Digestive Tract

“…Esophageal-NENs (E-NENs, eNENs) are relatively rare, predominantly develop sporadically, and have an estimated annual incidence of 0.09-0.3 cases per 100,000 persons (Table 3). They mainly comprise esophageal-NECs (E-NECs) and esophageal-MiNENs (E-MiNENs), with the latter showing a similar mutational landscape as small cell lung cancer [16,[26][27][28][29][30][31]. E-MiNENs either…”

68Ga-DOTATATE PET/CT Findings of Cervical Esophageal Neuroendocrine Tumor

“…Endoscopic treatment has been performed in NETs less than 1 cm in size with the absence of regional lymph node metastasis. However, chemotherapy is the treatment of choice in the presence of metastatic disease 6–8 . Therefore, accurate staging of the disease is essential.…”