Updated Principles of Surgical Management of Pancreatic Neuroendocrine Tumours (pNETs): What Every Surgeon Needs to Know

Pancreatic neuroendocrine tumours (pNETs) represent 1 to 2% of all pancreatic neoplasm with an increasing incidence. They have a varied clinical, biological and radiological presentation, depending on whether they are sporadic or genetic in origin, whether they are functional or non-functional, and whether there is a single or multiple lesions. These pNETs are often diagnosed at an advanced stage with locoregional lymph nodes invasion or distant metastases. In most cases, the gold standard curative treatment is surgical resection of the pancreatic tumour, but the postoperative complications and functional consequences are not negligible. Thus, these patients should be managed in specialised high-volume centres with multidisciplinary discussion involving surgeons, oncologists, radiologists and pathologists. Innovative managements such as “watch and wait” strategies, parenchymal sparing surgery and minimally invasive approach are emerging. The correct use of all these therapeutic options requires a good selection of patients but also a constant update of knowledge. The aim of this work is to update the surgical management of pNETs and to highlight key elements in view of the recent literature.

Case Report: Development of Type 1 Autoimmune Pancreatitis in an Adolescent With Ulcerative Colitis Mimicking Pancreatic Cancer

Introduction: Autoimmune pancreatitis (AIP) is a rare extraintestinal manifestation of inflammatory bowel disease (IBD) which is typically responsive to corticosteroid treatment.Case Presentation: We report a case of a 17-year-old male diagnosed with ulcerative colitis who subsequently developed acute pancreatitis. Blood tests demonstrated elevated pancreatic enzyme levels of amylase (1319 U/L) and lipase (809 U/L). Abdominal computed tomography revealed peripancreatic fat stranding and the presence of a perisplenic pseudocyst. Azathioprine and mesalazine were stopped as possible causes of drug-induced pancreatitis. However, pancreatic enzymes remained elevated and corticosteroid treatment was started. Despite corticosteroid therapy, amylase and lipase levels continued to increase. Infliximab was started due to a flare in gastrointestinal symptoms of ulcerative colitis. Follow-up abdominal ultrasonography revealed a pancreatic tail mass. Tumor markers, including CA 19-9, were elevated and atypical cells were seen on histological examination of an endoscopic ultrasonography-guided fine needle aspiration biopsy. Surgical pancreaticosplenectomy was performed for suspected pancreatic neoplasm. Surprisingly, histology revealed chronic pancreatitis with storiform fibrosis and infiltration of IgG4-positive cells, compatible with AIP type 1. Thereafter, pancreatic enzymes gradually decreased to normal levels and the patient has been in remission for 9 months on infliximab monotherapy.Conclusion: Pediatric gastroenterologists should keep in mind that AIP may develop during the natural course of pediatric IBD. Moreover, the development of pancreatic fibrosis may be non-responsive to corticosteroid treatment and mimic pancreatic neoplasia.

(A Clinicopathological Aspects of Resectable Pancreatic Neoplasm)

Aim Pancreatic cancer is one of the deadliest malignant neoplasms. As with many malignant neoplasms, survival rates depend on the histopathological type of cancer, its stage, tumor size, and treatment. In this study, we aimed to classify pancreatic cancer according to clinicopathological features and histological subtypes. Material and method The data of all adult patients diagnosed and treated for pancreatic neoplasm in our clinic were collected retrospectively from the hospital's computerized database and medical files. Patients were categorized according to their clinicopathological features. Chi-square test and Fisher's exact test were used for between-group comparisons, and t-test was used for independent samples for quantitative data. Data were expressed as mean ± SD for continuous variables and numbers and percentages for categorical variables. A value of p<0.05 was considered significant. Results The mean age of the patients was 60.5 years, 70.8% were male. There were five types of tumors defined histopathologically, and the most common diagnosis was adenocarcinoma (76.9%). The most common localization of the tumor was head and neck (44.4%). Whipple surgery was performed predominantly in 69.2% of patients, and distal pancreatectomy in 29.0%. Postoperative complications were observed in more than one third (34%) of the patients. The main complications were pancreatic cyst (16.3%). In the survival analysis performed with the Kaplan-Meier test, median survival of 30.5 months, and overall survival (OS) at 1.2 and 5 years were 67.8%, 40.5%, and 16.6%, respectively. Discussion However, survival analysis results were optimistic compared to population-based studies as all patients had resectable tumors.

Rare Pseudopapillary Neoplasm of the Pancreas: A 10-Year Experience

Introduction. The solid pseudopapillary epithelial neoplasm (SPN) is a rare form of pancreatic neoplasm with an incidence of 2-3% of all pancreatic tumours. The recent increase in incidence is attributed to the increasing use of imaging techniques for nonspecific abdominal complaints. We report our institutional experience in the management of this tumour over the last decade. Method. We retrospectively analyzed from a prospectively maintained database of patients from January 2011 to December 2020 who were operated upon for SPN. All the patients were followed till date. Results. Of 479 patients operated on for various types of pancreatic tumours during this period, 15 (3.1%) had SPN. The mean age of presentation was 28 years with a female preponderance (12/15, 80%). The most common location was the body and tail of the pancreas (66%), and the mean size was 6.4 cm (2–15 cm). The tumour extent was defined as ‘borderline resectable’ in 20% of cases. Distal pancreatectomy was done in 11 patients with spleen preservation in 3. R0, R1, and R2 resection were done in 12, 2, and 1 patient(s), respectively. The operative mortality was 6.7%. All the patients are doing well on follow-up. Conclusion. SPN is a low-grade malignant tumour with a strong female predilection. Clinical manifestations have no specificity, imaging examination only contributes tumour location, and the final diagnosis rests on pathology. Surgery is the main modality of treatment and carries a good prognosis.

Polarising Frantz Tumors – Benign And Malignant Solid Pseudopapillary Epithelial Neoplasm Of Pancreas – Twin Case Reports

Solid pseudo-papillary epithelial neoplasm (SPEN), also known Hamoudi tumors or Franz tumors, are rare pancreatic neoplasm which are almost always seen in young women predominately of non-Caucasian descent with only a small minority of cases diagnosed in men [1]. The first published description of an SPN was by Frantz in 1959[2]. . It is a rare tumor comprising of less than 3 percent of all pancreatic tumor. It is seen most often in the region of tail of pancreas. This tumor is mostly asymptomatic and usually detected when it reaches large size. These are tumors with low malignant potential and rarely vascular invasion and metastatic disease can be seen in aggressive cases. Here we report two cases, one each of benign SPEN and malignant SPEN with contrasting imaging findings and polar outcomes.

Role of Imaging in a Case of Toxoplasmosis Presenting as Generalized Lymphadenopathy

Toxoplasmosis is caused by Toxoplasma gondii an obligate protozoan intracellular parasite. The disease has variable prevalence globally and is usually asymptomatic. Pregnant and immunocompromised people are at risk of getting infected. Enlarged lymph nodes are the most frequently observed clinical form of Toxoplasma in humans, mostly affecting posterior cervical nodes. Other organs usually affected are the brain and eyes. We present a case of toxoplasmosis with generalized lymphadenopathy mimicking metastasis in a lady with a previous history of operated pancreatic neoplasm.