Nonfunctional pancreatic neuroendocrine tumours misdiagnosed as autoimmune pancreatitis:A case report and review of literature

BACKGROUND Nonfunctional pancreatic neuroendocrine tumours are difficult to diagnose in the early stage of disease due to a lack of clinical symptoms,but they can rarely manifest as autoimmune pancreatitis.Autoimmune pancreatitis is an uncommon disease that may cause recurrent acute pancreatitis and is therefore often regarded as a special type of chronic pancreatitis.CASE SUMMARY We report a case of a 42-year-old female who had nonspecific upper abdominal pain for 4 years and radiological abnormalities of the pancreas that mimicked autoimmune pancreatitis.The symptoms and pancreatic imaging did not improve following 1 year of steroid therapy.Finally,pancreatic biopsy was performed through endoscopic ultrasonography-guided fine-needle aspiration biopsy,and nonfunctional pancreatic neuroendocrine tumours were ultimately diagnosed.Pancreatectomy has resolved her symptoms.CONCLUSION Therefore,the differentiation of nonfunctional pancreatic neuroendocrine tumours from autoimmune pancreatitis is very important,although it is rare.We propose that endoscopic ultrasonography-guided fine-needle aspiration biopsy should be performed if imaging characteristics are equivocal or the diagnosis is in question.

Borderline resectable pancreatic cancer:Certainties and controversies

Borderline resectable(BR)pancreatic ductal adenocarcinoma(PDAC)is currently a well-recognized entity,characterized by some specific anatomic,biological and conditional features:It includes patients with a stage of disease intermediate between the resectable and the locally advanced ones.The term BR identifies a tumour with an aggressive biological behaviour,on which a neoadjuvant approach instead of an upfront surgery one should be preferred,in order to obtain a radical resection(R0)and to avoid an early recurrence after surgery.Even if during the last decades several studies on this topic have been published,some aspects of BR-PDAC still represent a matter of debate.The aim of this review is to critically analyse the available literature on this topic,particularly focusing on:The problem of the heterogeneity of definition of BR-PDAC adopted,leading to a misinterpretation of published data;its current management(neoadjuvant vs upfront surgery);which neoadjuvant regimen should be preferably adopted;the problem of radiological restaging and the determination of resectability after neoadjuvant therapy;the post-operative outcomes after surgery;and the role and efficacy of adjuvant treatment for resected patients that already underwent neoadjuvant therapy.

Cystic Tumours of the Pancreas: A Challenging Pathology, Diagnosis and Management

Background: Cystic tumours of the pancreas are relatively uncommon tumours but there is an increasing awareness of their importance. Modern advances in imaging had resulted in a more prompt diagnosis of these tumours. The understanding of the pathology, clinical features, diagnosis and management of these tumours is continuously evolving. Data Sources: Systematic literature review. A PubMed database research was performed. Relevant articles published in English were identified and scrutinized. Duplications of information and persistently unsolved uncertainties were excluded. Results: Cystic tumours of the pancreas should be differentiated from pancreatic pseudocysts. Cystic tumours could be classified into: cystic serous neoplasms (SCN), mucinous cystic neoplasms (MCN), intraductal papillary neoplasms (IPMN), solid pseudopapillary neoplasms (SPPN) and the rarer tumours cystic pancreatic endocrine neoplasms (PEN). Except for SCN, all these tumours should be regarded, at least, as potentially malignant but they may be frankly malignant. The age of the patient and site of the lesion may be helpful in the diagnosis. In particular, MCN is always encountered in females in their middle age and often in the body and tail of the pancreas. Many of these tumours are diagnosed incidentally. Pancreatitis and hyperamylasaemia should be interpretted with caution as IPMN may present as pancreatitis. CT is the primary diagnostic tool although MRI, EUS, PET, abdominal ultrasound and ERCP have all been utilised. Biochemical markers are of limited value in the diagnosis. Conclusions: Management decision depends primarily on the understanding of pathology and on how confident the preoperative diagnosis is. SCN may be observed. Partial pancreatectomy is the usual operation performed for most other tumours according to the location of the lesion which means that distal pancreatectomy is usually the operation performed for MCN. Total pancreatectomy for IPMN, enucleation and central pancreatectomy have all been described in the literature. Laparoscopy is more suitable for benign or low grade malignant tumours in the context of distal pancreatectomy. Spleen should be preserved unless splenectomy is indicated and splenic vessels should be preserved wherever possible when preserving the spleen.

Adult pancreatoblastoma: Current concepts in pathology

Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas,which may pose diagnostic difficulties.Because of its rarity,little is known about its clinical and pathologic features.This article reviews the clinical and pathologic features of pancreatoblastoma in adults including differential diagnosis,treatment,and follow-up.Although pancreatoblastoma commonly occurs in childhood,there have now been more than 70 adult pancreatoblastomas described in the literature.There is a slight male predominance.There are no symptoms unique to pancreatoblastomas and adult patients are frequently symptomatic.The most common presenting symptom is abdominal pain.Grossly,the tumours are often large and well-circumscribed.Microscopically,pancreatoblastomas are composed of neoplastic cells with predominantly acinar differentiation and characteristic squamoid nests.These tumours are positive for trypsin,chymotrypsin,lipase,and BCL10.Loss of heterozygosity on chromosome 11p is the most common molecular alteration in pancreatoblastomas.Adult pancreatoblastomas are aggressive tumours with frequent local invasion,recurrence,and distant metastasis.Treatment consists of surgical resection.Chemotherapy and radiotherapy may have a role in the treatment of recurrent,residual,unresectable,and metastatic disease.It is important to distinguish pancreatoblastomas from morphological mimics such as acinar cell carcinomas,solid pseudopapillary neoplasms,and pancreatic neuroendocrine neoplasms.

Solid pseudopapillary neoplasm of the pancreas

Solid pseudopapillary neoplasms are rare.This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas,including the epidemiology,cytology,molecular pathology,differential diagnosis,treatment,and prognosis.Solid pseudopapillary neoplasms are low-grade malignant tumours of the pancreas characterized by poorly cohesive epithelial cells with solid and pseudopapillary patterns.Solid pseudopapillary neoplasms occur predominantly in young women.Although solid pseudopapillary neoplasms can occur throughout the pancreas,they arise slightly more frequently in the tail of the pancreas.The aetiology is unknown.Extremely rare cases have been reported in the setting of familial adenomatous polyposis.There are no symptoms unique to solid pseudopapillary neoplasms,however,the most common symptom is abdominal pain or discomfort.The features of solid pseudopapillary neoplasms on computed tomography imaging are indicative of the pathologic changes within the tumour.Typically,well-demarcated masses with variably solid and cystic appearances.Microscopically,these tumours are composed of epithelial cells forming solid and pseudopapillary structures,frequently undergoing haemorrhagic cystic degeneration.Typically,these tumours express nuclear and/or cytoplasmicβ-catenin.Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1,the gene encodingβ-catenin.The overall prognosis is excellent,and most patients are cured by complete surgical resection.

Octreotide acetate long-acting release in treatment of pancreatic neuroendocrine tumors

Pancreatic neuroendocrine tumours are uncommon neoplasms of the pancreas, accounting for around 2% of all primary pancreatic tumors. This specific group of tumours includes insulinoma, gastrinoma, glucagonoma, VIPoma, somatostatinoma and others. They may cause a clinical syndrome due to hormone overproduction. These tumours tend to be less aggressive than pancreatic adenocarcinoma, however, more than 50% of them have metastasized to the liver at the time of diagnosis. Under this circumstance, surgery is impossible to resect all metastases. Thus other various medical measures have been explored in the treatment of these tumors.