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pancreatic cancer is well known to be an aggressive and highly malignant condition with varied ways of presentation .
pancreatic cystic neoplasms ( pcns ) are very uncommon causes of pancreatic malignancy and are often ignored or missed , especially in the early stages .
they can be considered as confusing diagnostic problems that are being encountered with greater frequency , especially with modern and advanced imagining technology [ 1 , 2 ] .
pcns account for < 10% of all pancreatic malignancies , but for up to 30% of pancreatic resections performed , and they encompass a spectrum of benign , premalignant ( borderline ) and malignant lesions [ 1 , 2 , 3 ] .
although relatively rare , it is important to distinguish cystic neoplasms from the far more prevalent entity of benign pseudocysts , which are usually associated with a clinical history and radiographic evidence of pancreatitis .
cystic neoplasms of the pancreas can be classified into four major categories as discussed below .
these categories possess varying potentials for malignancy and prognostic outcomes , hence the need for proper diagnosis .
we bring to light our experience with a 49-year - old male with no past medical history who was initially diagnosed with acute pancreatitis without any obvious cause at an outside facility after he presented with sudden epigastric pain .
he was initially treated conservatively and subsequently discharged home . however , following discharge , his symptoms persisted .
two weeks later , he was readmitted to the outside facility where a laparoscopic cholecystectomy was performed for cholelithiasis that was presumed to be the cause of his persistent symptoms . at the same time
he was also found to have a pancreatic pseudocyst on ct of the abdomen / pelvis ( fig . 1 , fig
he was subsequently seen by gastroenterology at our hospital , where he had a eus - fna biopsy of the pancreatic pseudocyst , and was discharged home .
however , on discharge , he started experiencing unremitting daily fevers ( 101104f ) , nausea and diffuse abdominal pain . at this time
he presented to the emergency department as his symptoms continued to worsen . on presentation , he was found to be in painful distress .
physical examination revealed a moderately distended and tender abdomen with guarding in the epigastrium and left lower quadrant .
labs showed a hemoglobin of 11.4 g / dl , a white blood cell count of 10.2  10/l , ast of 85 u / l , alt of 119 u / l and alkaline phosphate of 136 u / l .
he was admitted and treated conservatively for presumptive persistent pancreatitis . a ct scan of the abdomen / pelvis with contrast revealed sequela of pancreatitis with complex peri - pancreatic fluid and likely necrosis involving the distal pancreatic body and tail .
a fluid level was also identified , raising concern for debris / necrotic tissue versus blood products secondary to recent bleeding into these collections .
multiple ill - defined hypoattenuating lesions scattered throughout the hepatic parenchyma were also identified , although they were considered too small to characterize ( fig .
the patient was placed on vancomycin and zosyn from admission without any relief of his symptoms .
given these worsening symptoms , he was taken to the operating room for an exploratory laparotomy and possible pancreatic necrosectomy . upon exploration of the abdomen ,
on pathological examination , the tumor was found to be composed of highly pleomorphic , discohesive cells with eosinophilic cytoplasm and frequent multinucleated giant cells ( fig .
the tumor was also positive for epithelial membrane antigen , confirming the epithelial nature of the malignant cells .
they range from inflammatory pseudocysts that arise from acute or recurrent pancreatitis to pcns that possess varying malignant potential .
cystic neoplasms are a broad group with various subtypes that are responsible for about 1% of pancreatic cancers .
presenting symptoms and signs are nonspecific and are no basis for diagnosis . in most cases ,
they remain asymptomatic until they are incidentally discovered or up to very late stages , especially in malignant subtypes .
they are typically benign , multicystic lesions formed by glycogen - rich , periodic acid - schiff - positive epithelial cells with a central scar that can be found throughout the pancreas .
abdominal discomfort is the major presenting feature and is mainly secondary to expanding tumor size .
mucinous cyst adenomas are a second subtype and just like their serous counterparts are common among middle - aged females , with a higher potential for malignancy . on imaging they
may be seen as one or more cysts with thick , irregular walls along the body and/or tail of the pancreas .
cystic fluid analysis of mucinous cystadenomas reveals a positive mucin stain with an elevated cea level .
intrapapillary mucinous neoplasia is a third subtype and unlike the subtypes discussed earlier , it is more common in middle - aged and older males .
lastly , papillary cystic neoplasms are a rare form of cystic neoplasms found in young females .
they have a malignant potential , are locally aggressive and can be found along the body and/or tail of the gland . the most important factor to keep in mind with cystic neoplasms
this will help assess the potential for malignancy and hence guide appropriate therapy . according to grobmyer et al .  
the risk of malignancy is directly proportional to the size of the cysts , especially in mucinous subtypes .
asymptomatic cysts that are < 5 cm at their widest diameter without concerning features such as nodularity and/or calcifications can be serially monitored at least yearly with imaging .
eus - fna is usually added to obtain cystic fluid for analysis and cytology , thereby enhancing diagnostic accuracy .
molecular markers and cystic dna are other methods that can be employed in proper diagnosis . in a prospective , multicenter study , khalid et al .
[ 7 , 8 ] and jones et al .   concluded that elevated amounts of pancreatic fluid dna , high - amplitude mutations and specific mutation acquisition sequences are indicators of malignancy .
lesions with a high risk of malignancy and caught early are usually managed by surgery .
lesions in the pancreatic head are resected by a pancreaticoduodenectomy while those in the distal body and tail are resected by a pancreatectomy .
serous cyst adenomas that are asymptomatic and do not raise concern of malignancy do not need to be resected .
cysts that are suspicious for malignancy in patients who are not surgical candidates can be managed with eus - guided ethanol lavage with paclitaxel injection .
cysts with widespread metastases have a very poor prognosis and palliative options may need to be explored .
our experience shows the potential extent of malignant pcns if left untreated or undiscovered . in a matter of 12 weeks
, our patient went from being diagnosed with what was initially thought to be acute pancreatitis to metastatic malignant mucinous cystic pancreatic neoplasm that eventually cost him his life .
it is our hope that our experience and this article bring to light the importance of proper diagnosis of pcns .
pancreatic cancer is well known to be an aggressive and highly malignant condition with varied ways of presentation . 
 pancreatic cystic neoplasms are very uncommon causes of pancreatic malignancy and can often be ignored or missed , especially in the early stages . 
 we present the case of a 49-year - old caucasian male with no past medical history presenting to an outside facility with sudden epigastric pain that was eventually diagnosed as acute pancreatitis . on transfer to our facility , he was eventually found to have metastatic malignant mucinous cystic pancreatic neoplasm . 
 barely 12 weeks after his initial presentation and following an aggressive hospital course , he passed away .