solid pseudopapillary tumor ( spt ) of the pancreas is a rare enigmatic tumor of low malignant potential that most frequently occurs in young non - caucasian women . are cured . because of the degenerative changes that commonly accompany these tumors , they must be considered in the differential diagnosis with other cystic neoplasms and pancreatic pseudocysts . we present a case of spt in a caucasian patient investigated by endoscopic ultrasonography ( eus ) , computed tomography ( ct ) and magnetic resonance imaging ( mri ) . a 32-year - old female caucasian patient presented to the emergency department of our hospital complaining of postprandial epigastric pain during the last 4 months . she had no history of ulcer or any other pathology of the upper gastrointestinal tract . physical examination and laboratory tests were unremarkable . on eus , a well - circumscribed lesion measuring approximately 3 cm was confirmed in the pancreatic body . the lesion presented a mixed solid and cystic composition , with frond - like papillary projections into the cystic areas ( fig . 1 ) . no surrounding lymphadenopathy was noted , and the pancreatic duct appeared to have a normal course and contour . on contrast - enhanced ct , a well - circumscribed mass with solid and cystic components was revealed in the pancreatic body . mri demonstrated a well - defined lesion with heterogeneous ( mixed low and high ) signal intensity on t1- and t2-weighted images . on t1-weighted image a low intensity area this corresponded to a cystic area that was evident on t2-weighted images presenting high signal intensity . after contrast administration the lesion enhanced partially and gradually but remained hypointense in relation to the normal pancreatic parenchyma ( fig . 3 ) . the patient underwent eus - guided fine needle aspiration ( eus - fna ) , which revealed a homogeneous population of small cells with central the cell block revealed a papillary architecture , and immunohistochemical stains on the eus - fna specimen were strongly positive for vimentin and moderately strongly positive for progesterone receptors . intraoperatively there was no evidence of local tumor adhesions with adjacent structures , the mesenteric and splenic vessels . the postoperative course was uneventful and she was discharged from hospital after 6 days of recovery . six months after the operation the patient is free of disease or any kind of symptoms and in excellent general condition . at gross examination of the surgical specimen a soft , round , well - circumscribed mass with a pseudocapsule was identified . at histologic analysis the tumor was composed of uniform polygonal cells arranged in solid nests with areas of cystic degeneration . immunohistochemical analysis revealed : vimentin ( + ) , nse ( + ) , progesterone receptors ( + ) , cd10 mild ( + ) , pankeratin (  ) , cytokeratin 7 (  ) , chromogranin a (  ) , synaptophysin (  ) , ki67 2% of the neoplastic cells . spt of the pancreas is rare and was first described by franz in 1959 . over time many terms have been used to describe the same tumor , such as frantz 's tumor , solid and papillary tumor , papillary cystic tumor , solid - cystic tumor , solid - cystic and papillary epithelial neoplasm , benign or malignant papillary tumor of the pancreas , papillary epithelial neoplasm of pancreas in a child and adenocarcinoma of the pancreas in childhood . who in 1996 proposed the name this term reflects the two most conspicuous histological features : the solid and pseudopapillary areas . spts constitute about 5% of cystic pancreatic tumors and about 12% of exocrine pancreatic neoplasms . they occur predominantly in girls ( not before the age of 9 years ) and in younger women ( mean age 35 years ) . there is a predilection for asian and african - american women , but some cases have also been reported in men and children . although most tumors show benign behavior , malignant degeneration may occur . spts with clear criteria of malignancy ( vascular and nerve sheath invasion or lymph node and liver metastases ) are designated as solid pseudopapillary carcinomas . nevertheless , the tumor is indolent , with long patient survival , even in the presence of extension into adjacent organs or metastases . the tumor may present with a gradually enlarging abdominal mass causing vague abdominal pain or discomfort . obstructive symptoms may occur if the neoplasm grows large enough or is located in the pancreatic head . small tumors are usually incidental findings on abdominal imaging studies or during laparotomy performed for other purposes . although spts may affect any portion of the pancreas , they are slightly more common in the pancreatic tail . this predilection has recently been debated by some authors . in some patients they appear as extrapancreatic tumors with only a localized connection to the gland . in these cases there are no known associations between spt and other neoplasms or heritable or acquired medical conditions . serum amylase levels and cancer markers ( ca199 , carcinoembryonic antigen , -fetoprotein ) are usually normal . they are also not associated with paraneoplastic syndromes because of the production of hormones or enzymes by the tumor . on imaging well - encapsulated cystic and solid masses can be seen , but sometimes spt may present as a solid - looking mass or having internal septa and calcifications . ct usually demonstrates a well - encapsulated lesion with varying solid and cystic components owing to hemorrhagic degeneration . following contrast material administration , enhancing solid areas are typically noted at the periphery , whereas cystic spaces are usually more centrally located . in our case cystic spaces were located in the anterior aspect of the tumor and solid areas in the posterior . in some instances the presence of a thick peritumoral fibrous capsule results in a radiological similarity to pancreatic pseudocysts . eus also typically demonstrates a well - demarcated , encapsulated lesion with mixed solid and cystic components . the diagnostic utility of eus has been greatly augmented by the ability to perform fine needle aspiration ( eus - fna ) of the lesion . the specimen obtained on eus - fna can also be stained immunohistochemically , as was seen in the case presented . however areas of high signal intensity on t1-weighted images and low or inhomogeneous signal intensity on t2-weighted images can help identify blood products . moreover spts do not demonstrate the hypervascularity typically seen in islet cell tumors . in our case hemorrhagic areas on the initial contrast - phase the mass was hypointense but showed progressive filling , a finding that has also been described by other authors . overall , between imaging findings and pathology results , there was agreement in the characterization of cystic areas and in the presence of a peripheral pseudocapsule . fine needle aspiration or biopsy and cytologic or histologic analysis are needed for definitive diagnosis in certain cases . a solid , cystic , solid - cystic and/or hemorrhagic appearance may be possible , depending on tumor size . histologically spt is generally characterized by solid areas with a pseudopapillary pattern and cystic spaces due to degenerative changes . in some cases extensive hemorrhage into the cystic spaces results in the appearance of an organized thrombus . however the immunohistochemical pattern of spts is distinctive and differs from that of other primary pancreatic tumors . cytokeratin expression is rare ( in our case it was negative ) and markers of acinar differentiation are often negative . presence of neuroendocrine markers is not significant ( chromogranin a is not detected and synaptophysin has a patchy immunoreactivity on 22% ) . in our case there was neither chromogranin nor synaptophysin expression . many investigators favor the theory that spts originate from multipotent primordial cells , while others suggest an extrapancreatic origin from genital ridges of ovarian - related cells attached to pancreatic tissue during early embryogenesis . the presence of progesterone receptors like in our case favors the second theory , but spts also occur in men without sex hormone abnormalities . a low pool of ki67-reactive tumor cells has also been referred , explaining the good clinical outcome of these tumors , and was also noticed in our case . patients with spts can be adequately managed by pylorus - preserving pancreatoduodenectomy or spleen - preserving distal pancreatectomy , as in our case . selected neoplasms of the body can be managed by corpus resection by experienced surgeons dedicated in pancreatic surgery . the efficacy of chemotherapy or radiation therapy for spt has yet to be shown , although a single case was reported to be resectable after preoperative chemotherapy . they comprise a wide range of differing underlying pathologies from completely benign through premalignant to frankly malignant . the exact diagnostic and management pathway of these lesions remains challenging and algorithms for the diagnosis and management have been suggested by the international association of pancreatology [ 17 , 18 ] . spt should be encountered in the differential diagnosis of a cystic pancreatic lesion with or without a solid component in young patients . in view of the relative benign behavior of spts , the young age of patients and the complete alleviation of all symptoms after resection , accurate preoperative diagnosis is very important . radiologists should be familiar with typical and atypical presentation of spts and use all available imaging methods in difficult cases . a team of experienced radiologists , gastroenterologists , surgeons and pathologists is essential for the appropriate management of these rare tumors .
solid pseudopapillary tumor of the pancreas is a rare pancreatic tumor that predominantly occurs in young non - caucasian women . 
 although most tumors show benign behavior , malignant degeneration may occur . a case of solid pseudopapillary pancreatic tumor in a caucasian woman 
 is presented that was investigated by endoscopic ultrasonography ( eus ) , computed tomography , magnetic resonance imaging and eus - guided fine needle aspiration . 
 the patient underwent surgery and radiological findings are correlated with histopathology . 
 the preoperative diagnosis of solid pseudopapillary tumor of the pancreas is challenging , frequently leading to imaging by multiple different modalities .