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Cysts are sacs or capsules that form in the skin or inside the body. They may contain fluid or semisolid material. Although cysts can appear anywhere in the body, most frequently they live in the skin, ovaries, breasts or kidneys. Most cysts are not cancerous.
Pancreatic cystic lesions are being found more frequently on cross-sectional imaging studies (e.g. conventional imaging such as CT/MRI/Ultrasound). Although many of these cysts are discovered incidentally in asymptomatic patients, many have a malignant potential. Coupled with the lack of a single diagnostic test for accurate risk stratification and the risks of surgical resection, this leads to diagnostic and management challenges for clinicians, highlighting the need for a multidisciplinary approach to optimize patient care.
What is the incidence rate for pancreatic cysts?
The incidence of pancreatic cystic lesions is increasing, partly due to the aging population, the increased use of cross-sectional imaging studies, and, perhaps, other reasons that are currently unclear. According to Reference 1, the prevalence of pancreatic cysts increases with age; it has an approximate overall incidence of 15% on MRI, with meta-analysis data showing this number increases from 9% in people aged 50 to 59 years to as high as 38% in those aged 80 years and older. It follows that many people diagnosed with a Neuroendocrine Neoplasms will have pancreatic cysts incidentally found during work up and surveillance.
Types of pancreatic cysts
Pancreatic cystic lesions represent a heterogeneous group of lesions, and their malignancy potential varies based on cyst type. Typically, cysts are divided into two categories: inflammatory fluid collections and pancreatic cystic neoplasms (PCNs).
Inflammatory fluid collections
Inflammatory fluid collections occur as a complication of pancreatitis or pancreatic injury and lack a true epithelial lining, although they can be challenging to distinguish from PCNs on the basis of imaging alone. This type can be divided further into acute peripancreatic fluid collections and pseudocysts. The latter is defined as collections of leaked pancreatic fluids and they may form next to the pancreas during pancreatitis.
Pancreatic pseudocysts are different from true pancreatic cysts. Both cysts and pseudocysts are collections of fluid. A true cyst is a closed structure. It has a lining of cells that separates it from the nearby tissue. A pseudocyst isn’t closed and doesn’t have a lining of epithelial cells separating it from the nearby tissue. This difference is important. The cause of these conditions and treatments may vary. Reference 1 states that pancreatic pseudocysts are rarely cancer. However, the later publication (Reference 3) states they are non-neoplastic.
Pancreatic cystic neoplasms (PCNs)
PCNs are divided into mucinous and non-mucinous lesions. Mucinous lesions are considered premalignant and are lined by a columnar epithelium that secretes mucin, as is seen in intraductal papillary mucinous neoplasms (IPMNs), and mucinous cystic neoplasms (MCNs). Serous cystadenomas (SCAs) are benign collections of multiple smaller cysts lined by a cuboidal epithelium arising from pancreatic acinar cells. Other serous cysts of the pancreas include rarer lesions such as solid serous adenoma and von Hippel-Lindau disease–associated cystic lesions.
This summary was abstracted from References 1 and 3 below but includes some additional wording from the blog author.
Above, I listed two main categories of pancreatic cysts. I’ll now focus on PCNs but on the basis that some inflammatory fluid collections can become a cancer, I’ll include that as a rare possibility of Pancreatic Neuroendocrine Tumour (Pan NET). PCNs can be mucinous and non-mucinous lesions.
Mucinous lesions
These are considered premalignant and are lined by a columnar epithelium that secretes mucin, as is seen in intraductal papillary mucinous neoplasms (IPMNs), and mucinous cystic neoplasms (MCNs). Mucinous neoplasms would be highly unusual in Neuroendocrine disease and as you will see below, NETs are described under the heading of non-mucinous Serous Cystic Neoplasms (SCNs).
Non-mucinous lesions
Serous Cystic Neoplasm (SCNs)
SCNs, also known as serous cystadenomas, are benign pancreatic tumors with an exceptionally low malignancy potential of 0.1%. SCNs represent about 30% of all cystic neoplasms of the pancreas.
From References 1 and 3, it’s clear that most pancreatic cysts are unrelated to NET. Mucinous lesions are not normally part of the Neuroendocrine Neoplasms family, so the focus moves to non-mucinous.
In reference 1, five separate pancreatic cyst case studies were presented. One of the 5 explained a Pan NET. One also identified a VHL associated lesion but there was no indication this was a true pancreatic NET (although other texts may describe it as such in the context of VHL). Another separate case study was diagnosed as a pseudocyst but as per above, a cancer is a rare find in a cyst, so I can only assume it was a benign issue unrelated to pancreatic NET (i.e. not all pseudocyst neoplasms are NET). The other two case studies in Reference 1, were unrelated to NET.
Reference 4 confirms a subtype of cystic pancreatic NETs which is said to account for up to 17% of Pan NETs.
NET or NOT – Ronny Allan – Living with Neuroendocrine Cancer
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