Giant type III well-differentiated neuroendocrine tumor of the stomach: A case report

• A rare case of a large type III neuroendocrine tumor of the stomach is presented.
• Currently there is no published data regarding large neuroendocrine tumors of the stomach.
• Classification systems for neuroendocrine tumors are not universally accepted, making it difficult to compare data.
• Discrepancy between fine needle aspiration and final pathology is encountered in large neuroendocrine tumors.
• Type I and II gastric neuroendocrine tumors can be managed endoscopically. Type III and IV should be managed surgically.

IntroductionWe describe a case of a large type III neuroendocrine tumor of the stomach. Management and current literature are reviewed.Presentation of caseA 37 year old female presented with upper gastrointestinal bleed and epigastric pain. Further workup demonstrated a large ulcerated gastric mass near the GE junction. Computer tomography scan and endoscopic ultrasound showed a 10 cm mass with no evidence of distant disease. Fine needle aspiration pathology was consistent with a well differentiated neuroendocrine tumor (Ki67 index <2%), with elevated levels of chromogranin A and serotonin levels but normal gastrin. The patient underwent an uneventful total gastrectomy. Final pathology analysis reported a higher KI67 index (7.54%) and a final pathology of grade 2 type III, T3 N3, neuroendocrine tumor of the stomach. The chromogranin levels normalized and no recurrent disease has been detected in one year follow up.DiscussionGastric neuroendocrine tumors are extremely rare, accounting for 4% of all neuroendocrine tumors of the body and 1% of all neoplasms of the stomach. Based on histomorphologic characteristics and pathogenesis, gastric neuroendocrine tumors are classified into four types with differing prognosis and behavior. Current literature describes type 3 gastric neuroendocrine tumors as larger than 2 cm. However, there is no precedent in the literature for a tumor of this size.ConclusionThe incidence of gastric neuroendocrine tumors has been increasing during the last decade, underscoring the need to improve our understanding of their biology and behavior. When identified histologically, patient outcomes depend on appropriate determination of tumor biology and subsequent choice of treatment.