Project description:BackgroundType I gastric neuroendocrine tumors (GNETs) originate from hyperplasia of enterochromaffin-like (ECL) cells and are commonly detected in patients with chronic atrophic gastritis, including autoimmune gastritis. Typical treatment for type I GNETs comprises simple surveillance and/or endoscopic resection. For alleviation of hypergastrinemia resulting in ECL cell hypertrophy, antrectomy is a treatment option. Type I GNETs mostly have excellent prognosis, and if a surgical approach is chosen, the procedure must be minimally invasive. One such technique for multiple type I GNETs, minimally invasive single-incision laparoscopic antrectomy (SILA), is reported here for the first time.Case presentationWe performed SILA on a 46-year-old woman who developed type I GNETs caused by hypergastrinemia due to autoimmune gastritis. A Lap-Protector was inserted in a 3 cm incision at the umbilicus, and set an EZ Access equipped with two 5 mm trocars and one 12 mm trocar. Antrectomy without lymph node dissection was performed using a 5 mm forward-oblique viewing endoscope, a vessel sealing device, and linear staplers, while reconstruction was by Billroth I reconstruction. Side-to-side anastomosis was performed using a 45 mm linear stapler. The stapler entry hole was sutured intracorporeally using barbed suture material. The operation time was 140 min and blood loss was 5 ml. The patient was discharged ten days after surgery without complications. Serum gastrin level decreased to within the normal range on the day after the operation. One year after surgery, esophagogastroduodenoscopy showed pathological disappearance of all lesions of the remnant stomach.ConclusionsSILA is a minimally-invasive and tolerable technique for treatment of multiple type I GNETs. In this reported case there was good cohesiveness and effectiveness in normalizing gastrin levels and in elimination of remnant gastric lesions.
Project description:Gastric adenocarcinoma of the fundic gland type (GA-FG) is a rare gastric neoplasm. We present a unique case of multiple GA-FG that coexisted with the well-differentiated neuroendocrine tumors in a patient with autoimmune gastritis. To our knowledge, this is the first documented instance of such a co-occurrence and the molecular mechanism of their origin has been reviewed systematically. A 47-year-old male presented to our hospital with abdominal distension for over 10 years. Gastroscopy revealed multiple gastric eminence lesions (0.2-1.5 cm). After endoscopic mucosal resection, the pathological morphology showed mixed tumor components infiltrating into the submucosa with puzzling similarity. One with uniform-sized tumor cells arranged in nests or tubes and the other a well-differentiated tubular adenocarcinoma with irregular branching and visible gland fusion. Immunohistochemistry findings revealed the first component expressed typical markers of neuroendocrine tumor, whereas the second component expressed pepsinogen and mucin-6, indicating the presence of oxyntic gland adenocarcinoma. Due to the tumors' proximity to the surgical margins, the patient underwent laparoscopic subtotal gastrectomy three months after the diagnosis without any tumor residue and showed no recurrence or metastasis occurred in the following regular checkups.
Project description:Neuroendocrine tumors (NET) originate from the diffuse neuroendocrine system. These can arise in almost every organ of the body, although they are most commonly found in the gastrointestinal tract and respiratory system. The skull base and sellar region are extremely rare sites for neuroendocrine carcinoma. Consequently, in this case, both diagnosis and definition of surgical goals, as well as further treatment strategies were challenging.A 65-year-old woman was admitted to our Neurosurgery Department with a rapidly progressive visus reduction, drowsiness, polyuria, and polydipsia. Neuroimaging showed a sellar/suprasellar mass (diameter of 2 cm) with a heterogeneous signal compressing the optic chiasm and extending laterally toward the cavernous sinus. Differential diagnosis based on imaging included pituitary macroadenoma or metastasis. The patient underwent endoscopic endonasal transsphenoidal surgery. A total resection of the mass was impossible because of the infiltration of the optic chiasm and the intraoperative histological diagnosis of malignant epithelial neoplasm. Further histological evaluation revealed that the lesion was a NET with no other primary or metastatic sites detectable. Subsequently, the patient was successfully treated with fractioned stereotactic radiotherapy and polychemotherapy. Four years after the surgery, follow-up magnetic resonance imaging showed stability of the residual disease. Neurologic examination revealed a complete visual recovery.Primary pituitary NET, though rare, should be included in the differential diagnosis of sellar lesions. A multimodality treatment approach is needed. Finally, the present case highlights, that in the case of a pituitary lesion infiltrating the optic chiasm, including NET, the endoscopic endonasal transsphenoidal subtotal resection followed by fractioned stereotactic radiotherapy and chemotherapy may represent an effective and safe choice of treatment.
Project description:Introduction and importancePeriampullary tumors are characterized as tumors that emerge nearby to the major papilla in the duodenum. They are rare lesions with an incidence rate of 0.4-0.48 per 100,000. Neuroendocrine tumors (NETs) constitute only 3% of all duodenal tumors. Their proximity to the major and minor papillae along with the gastric outlet raises a surgical challenge.Case presentationOur patient is a 40-year-old, male, medically free. He presented with history of a testicular mass. A CT scan of the abdomen and pelvis was done and showed a large retroperitoneal lymph node. A biopsy of the epididymal mass showed no evidence of malignancy. Excision of the left para-aortic mass revealed a metastatic lymph node of a well-differentiated neuroendocrine tumor. Further evaluation by gallium-68 PET-CT scan showed a periampullary neuroendocrine tumor. The decision to operate was concluded in a multidisciplinary team meeting, and intraoperatively the duodenum showed a well-defined mass between the first and second part of the duodenum which was excised via a trans-duodenal submucosal approach. A frozen section confirmed a negative margin. The final histopathology report showed a grade 2 metastatic well-differentiated neuroendocrine tumor. The latest follow-up was 3 years post-op via fluorine-18 fluorodeoxyglucose PET-CT and it showed no FDG avid disease at the duodenum or pancreases with no FDG avid lymphadenopathy or distant metastasis.ConclusionPeriampullary tumors that fall under certain parameters could be resected via transduodenal local resection. This procedure yields equivalent results to more invasive surgeries, such as a Whipple's procedure, with less morbidity.
Project description:Pulmonary hypoplasia is diagnosed during the perinatal period and is a cause of death in newborns. However, these developmental abnormalities are diagnosed in adulthood in some cases. A 70-year-old male smoker was diagnosed with stage IIIA pulmonary adenocarcinoma in the right upper lobe with right middle lobe hypoplasia. He subsequently underwent right upper and middle lobectomy with lymph node dissection by video-assisted thoracoscopic surgery. In addition to an invasive adenocarcinoma in the right upper lobe, pathological examination of the hypoplastic lobe revealed neuroendocrine hyperplasia, as well as tumorlets and a typical carcinoid. Eight cases of pulmonary neuroendocrine tumors that developed from pulmonary hypoplasia have been reported to date. Interestingly, all but one case occurred in the right middle lobe. Neuroendocrine cell hyperplasia has been reported to develop in hypoplastic lungs postnatally; therefore, we speculated that the lesion was the origin of these neuroendocrine tumors. Moreover, the pathological findings suggested that atelectasis was involved in the pathogenesis of this rare condition. In adults, when lobar hypoplasia is diagnosed, neuroendocrine tumors should be anticipated.
Project description:Background & Aims Primary hepatic neuroendocrine tumors (PHNETs) are rare malignant liver tumors that present diagnostic challenges owing to their rarity and absence of specific clinical features. This study aimed to investigate the characteristics of this rare liver tumor to enhance our understanding of the disease, improve diagnostic accuracy, and explore standardized diagnostic and treatment approaches. Case description During physical examination, two elderly women, aged 64 and 74 years, were found to have liver masses. 18F-FDG Positron Emission Tomography-Computed Tomography (18F-FDG PET-CT) and Ga68-DOTATATE PET-CT scans of both individuals revealed multiple liver masses that were initially suspected to be hepatic neuroendocrine tumors. Subsequent puncture pathology confirmed the diagnosis of neuroendocrine tumors. Furthermore, in Case 1, the tumor was also detected by 18F-FDG PET-CT in the lung, suggesting a metastatic tumor, in conjunction with liver immunohistochemistry and imaging findings. Laboratory tests revealed no significant abnormalities in liver function or autoimmune liver disease indicators, and there was no evidence of viral hepatitis infection. However, partial hepatectomy was not indicated for cases with distant metastasis or multiple space-occupying lesions. Individualized treatment approaches have been developed for such situations. A large portion of the tumor underwent Transarterial Embolization (TAE), and targeted combination chemotherapy or endocrine therapy was administered based on the pathological results. During regular follow-ups a 13 and 12 months, the tumor remained stable. The patients’ quality of life was good, and their psychological well-being was healthy. They led active lifestyles, demonstrated a thorough understanding of their disease and its progression, and actively cooperated during the follow-up process. Conclusion Our findings suggest that a combination of serological, radiological, and immunohistochemical examinations can aid in the diagnosis of PHNET. In addition, we determined that TAE combined with drug therapy could be an effective method for controlling PHNET progression. Regular postoperative follow-ups are important for monitoring the prognosis and tumor progression status of patients with PHNET.
Project description:BackgroundDunbar syndrome or median arcuate ligament syndrome is a rare disorder. In this disorder, a malposition of the arcuate ligament compresses the celiac trunk and causes nonspecific symptoms including postprandial pain, abdominal bruit and weight loss. Surgical management is the primary treatment.Pancreatic neuroendocrine tumors (PNETS) are also rare. It comprises about 1-3% of pancreatic neoplasm. The patient could be symptomatic or asymptomatic depends on the tumor being functional or nonfunctional. In addition, surgical therapy is the choice.Case presentationIn this paper, we report a case of 28 old female patient complaining from a long term of recurrent abdominal pain that doesn't releive on any kind of treatment, the multislices computerised tomography scan showed compress of the median arcuate ligament with an incidental mass in the tail of pancreas turned out to be a pancreatic neuroendocrine tumor.ConclusionsThe Dunbar syndrome and the Pancreatic neuroendocrine tumors must be kept in mind of phyciciens while the differential diagnosis of any recurrent abdominal pain.
Project description:BackgroundMixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) of the pancreas are extremely rare. Their pathogenesis and molecular landscape are largely unknown. Here, we report a case of mixed pancreatic intraductal papillary mucinous neoplasm (IPMN) and well-differentiated neuroendocrine tumor (NET) and identify its genetic alterations by next-generation sequencing (NGS).Case presentationA fifty-year-old male was admitted into the hospital for evaluation of a pancreatic lesion detected during a routine examination. Abdominal ultrasound indicated a hypoechoic mass of 2.6 cm at the head of the pancreas. Malignancy was suspected and partial pancreatectomy was performed. Thorough histopathological examination revealed a mixed IPMN-NET. In some areas, the two components were relatively separated, whereas in other areas IPMN and NET grew in a composite pattern: The papillae were lined with epithelial cells of IPMN, and there were clusters of NET nests in the stroma of papillary axis. NGS revealed shared somatic mutations (KRAS, PCK1, MLL3) in both components. The patient has been uneventful 21 months after the surgery.ConclusionsOur case provides evidence of a common origin for mixed IPMN-NET with composite growth features. Our result and literature review indicate that KRAS mutation might be a driver event underlying the occurrence of MiNEN. We also recommend the inclusion of mixed non-invasive exocrine neoplasms and neuroendocrine neoplasms into MiNEN.
Project description:Neuroendocrine neoplasms are a rare and heterogeneous group of neoplasms. Small sized (≤ 2 cm) pancreatic neuroendocrine tumors (pNETs) are of particular interest, as they are often associated with aggressive behavior, with no specific prognostic or progression markers. This article describes a clinical case characterized by a progressive growth of non-functional pNET requiring surgical treatment, in a patient with a germline FANCD2 mutation, previously not reported in pNETs.