Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis.

Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) are a peculiar entity that can occur throughout the whole gastrointestinal trait, and pancreatic localization is rare. Their main characteristic is the presence of at least a neuroendocrine and an epithelial component, each accounting for at least 30% of the tumour mass. The presence of epithelial ductal component defines adeno-MiNEN. We report a case of a 59-year-old woman affected by pancreatic adeno-MiNEN with challenging diagnosis and successfully treated. A systematic literature review and pooled analysis was also performed, aiming to define the management and outcomes of pancreatic adeno-MiNEN. Out of 190 identified records, 15 studies including 28 patients affected by pancreatic-adeno-MiNEN were included in the analysis. Pancreatic adeno-MiNEN occurred mainly in males (82.8%) and at a mean age of 61.7 (range: 24-82) years. Pre-operative diagnosis was possible only in 14.2% of cases. At presentation, the majority had already advanced disease (TNM stage III (53.8%) and stage IV 19.3%). Adjuvant therapy was performed in 55% of patients, and the tumour recurrence rate was in 30% of cases. Median disease-free survival (DFS) was 12 months (range: 0-216 months) with a 5-year DFS of 16.6%, while the median overall survival (OS) was 12 months (range: 0-288 months) with a 5-year OS of 23.5%. Pancreatic adeno-MiNENs are rare; as they have very heterogenous behaviour, they are rarely diagnosed preoperatively and have poor prognosis. Treatment of localised MiNEN still relies on radical surgical resection, which seems essential to achieve a good oncological prognosis. International registry on MiNEN is necessary to improve the knowledge on this rare tumour and to improve its outcomes.

Ileal Neuroendocrine Tumor in a Patient with Sclerosing Mesenteritis: Which Came First?

BACKGROUND Jejunoileal neuroendocrine tumors (JI-NETs) are rare tumors that can be associated with mesenteric fibrosis. This case report is of an incidental finding of a JI-NET in a patient who was previously misdiagnosed with sclerosing mesenteritis. CASE REPORT A 42-year-old man was admitted to our institution with diffuse abdominal pain and clinical and radiographic signs of bowel obstruction. He had a previous diagnosis of sclerosing mesenteritis, which had been histologically diagnosed after an exploratory laparoscopy performed in 2009 for recurrent acute abdominal pain. He was also annually monitored through computed tomography scans for an incidentally discovered, gradually enlarging mesenteric mass for which a "wait and watch" management approach was adopted. After a period of fasting and observation, the patient underwent an urgent exploratory laparotomy because of his worsening condition. Intraoperatively, an ileocecal resection was performed, along with excision of the known mesenteric mass. The pathology report revealed an ileal NET with nodal metastases within the mesentery and mesenteric tumor deposits (pT3N1). CONCLUSIONS JI-NETs are rare entities, which are usually encountered as incidental findings or in patients with unspecific abdominal pain. Our case represents a probable delayed diagnosis of JI-NET in the context of sclerosing mesenteritis; therefore, a possible association between these 2 conditions should be investigated.

Idiopathic gastric fundus necrosis: Case report about a rare and fatal clinical condition.

INTRODUCTION: Gastric fundus ischemia is a rare event, which does not account for many significant clinical studies. This disorder could have different etiologies, but authors agree that a prompt diagnosis and a proper treatment could avoid dangerous complications and ultimately the death of the patient. PRESENTATION OF CASE: We herein report an interesting idiopathic case of acute gastric dilatation and fundus ischemia of an 83-year-old Caucasic woman who was admitted to the Emergency Department complaining of abdominal discomfort, vomiting and constipation. DISCUSSION: In literature, only a few case reports about this condition are reported. Possible risk factors, etiologies, and the different therapeutic options available for this condition are examined, in order to try to favor clinicians to formulate a timely diagnosis and provide patients with rapid healthcare services. CONCLUSION: Further investigations are still needed to analyze the pathophysiological pathways responsible of gastric fundus ischemia and to provide a definitive treatment to this dangerous disorder.

Completely Isolated Enteric Duplication Cyst and Incidental Neuroendocrine Tumor of the Appendix: A Case Report.

BACKGROUND Completely isolated enteric duplication cysts (CIDCs) are rare malformations that can occur at any site in the gastrointestinal system. This report describes a woman with a CIDC and an incidental appendiceal neuroendocrine tumor (ANET). CASE REPORT A 26-year-old woman who presented with dysmenorrhea was assessed by ultrasound (US), which revealed a pelvic mass. Other imaging modalities, including magnetic resonance imaging (MRI), failed to clarify the origin of the mass. Intraoperative findings during diagnostic laparoscopy revealed an isolated, ovaloid mass with autonomous peristalsis and a short pedicle towards the root of the ileal mesentery. In addition, the appendix appeared enlarged with a hardened consistency. The mass was resected and an appendectomy performed laparoscopically. The pelvic mass was diagnosed as a CIDC and the appendix was incidentally found to contain a pT3Nx carcinoid tumor. Based on histological examination and guidelines of the European Neuroendocrine Tumor Network (ENET), the patient later underwent a laparoscopic right hemicolectomy. CONCLUSIONS CIDC in adulthood is very rare, especially when combined with an incidentally discovered pT3Nx appendiceal carcinoid tumor. Neither US nor MRI was able to provide a precise preoperative diagnosis. Diagnostic laparoscopy clarified the nature of the mass and revealed a lesion missed during the preoperative workup. Because of the diagnosis of ANET, the patient subsequently underwent a laparoscopic right hemicolectomy.