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

Abstract

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 re search 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.