Somatostatinomas are rare neuroendocrine tumors usually associated with Von Recklinghausen's disease, multiple endocrine neoplasia, or Von Hippel-Lindau syndrome. Nevertheless, it may be associated with sporadic occurrence in healthy patients (1) . Approximately 55% of somatostatinomas are pancreatic tumors and the remainder arises in ampullary and periampullary region or, rarely, in jejunum (2) . A patient with pancreatic somatostatinoma usually presents a triad of steatorrhea, weight loss and diabetes mellitus, due to inhibition of gastrointestinal peptides (1,3) . In duodenal papillary somatostatinomas (DPS), these symptoms occur in less than 10%. Endoscopic retrograde cholangio-pancreatography (ERCP), magnetic resonance or computed tomography can aid in diagnosis, which is only confirmed by psammoma bodies on histologic studies, a rare finding in pancreatic tumors (4) . Surgical resection (duodenopancreatectomy) is the treatment of choice. The authors present a case of DPS with cholestasis, endoscopic treated with excellent results and follow-up for 7 years.44-year-old woman with biliary colic for 1 month and evolving with 1-week jaundice. Bilirubin =14 mg/dL (conjugated =12 mg/ dL), AST, ALT and GGT increased and chromogranin A =6 U/l. ERCP revealed choledochal dilatation, presence of spontaneous choledocoduodenal fistula and a papillary tumor. A 10F plastic prosthesis was inserted through the fistulous orifice and biopsy was performed. The pathology report revealed neuroendocrine tumor. Magnetic Resonance Imaging unveiled a tumor on duodenal papilla (2.0 cm) and three peri duodenal lymph nodes (LN). PET/CT (FDG) revealed hypermetabolic area on duodenal papilla without LN involvement (FIGURE 1). Endosonography
<b><i>Introduction:</i></b> Imaging diagnosis of pancreatic solid-pseudopapillary neoplasms (SPNs) is difficult. Preoperative diagnosis by endosonography-guided fine-needle aspiration (EUS-FNA) is possible and has been reported in the literature in pancreatic tumors. However, its usefulness is still controversial. The aim of this study was to determine the accuracy of the EUS-FNA in the diagnosis of patients with SPN and describe the findings in computerized tomography (CT), magnetic resonance cholangiopancreatography imaging (MRI/MRCP), and EUS therefore comparing the imaging methods alone to the findings of microhistology (McH) obtained by EUS-FNA. <b><i>Materials and Methods:</i></b> We retrospectively reviewed the medical records of patients undergoing EUS-FNA with suspected SPN in imaging studies in 5 Brazilian high-volume hospitals (two university hospitals and three private hospitals). The demographic data; findings in CT, MRI/MRCP, and EUS; and McH results obtained by EUS-FNA were noted prospectively. The final diagnosis was obtained after the anatomopathological examination of the surgical specimen in all patients (gold standard), and we compared the results of CT, MRI/MRCP, EUS, and the McH with the gold standard. <b><i>Results:</i></b> Fifty-four patients were included in the study, of which 49 (90.7%) were women with an average age of 33.4 (range 11–78) years. The most common symptom presented was abdominal pain, present in 35.2% patients. SPN was detected incidentally in 32 (59%) patients. The average size of the tumors was 3.8 cm (SD: 2.26). The most common finding at EUS was a solid, solid/cystic, and cystic lesion in 52.9%, 41.1%, and 7.8% patients, respectively. The final diagnosis was 51 patients with SPN and 3 with nonfunctioning pancreatic neuroendocrine tumors (NF-NET). The correct diagnosis was made by CT, MRI/MRCP, EUS isolated, and EUS-FNA in 21.9%, 28.88%, 64.71%, and 88.24%, respectively. EUS-FNA associated with CT and MRI increased diagnostic performance from 22.72% to 94.11% and from 29.16% to 94.11%, respectively. <b><i>Conclusions:</i></b> SPN are rare, incidentally identified in most cases, and affect young women. Differential diagnosis between SPN, NF-NET, and other types of tumors with imaging tests can be difficult. EUS-FNA increases preoperative diagnosis in case of diagnostic doubt and should be used whenever necessary to rule out NF-NET or other type of solid/cystic nodular lesion of the pancreas.
▶ Fig. 3 a Fluoroscopy image of SpyGlass positioned on the pancreatic tail. b Internal view of main pancreatic duct obtained by SpyGlass with altered vascularization and diminutive mucous membrane projections. ▶ Fig. 4 Hematoxylin and eosin stain images of fragments obtained with SpyGlass, seen as pancreatic head (a), body (b) and tail (c) Spybites.All revealed presence of high-grade dysplasia (HGD). ▶ Fig. 2 a Endoscopic image of the duodenal papilla with eruption of mucoid secretion.b Pancreatography revealing defective filling of the pancreatic head and main pancreatic duct tortuosity with guidewire positioned on the pancreatic tail.
Purpose Imaging diagnosis of SPN is difficult. Preoperative diagnosis by EUS-FNA) is possible, safe, and has been reported in the literature. However, its usefulness is still controversial. The aim of this study was to determine the accuracy of the EUS-FNA and imaging findings in CT and MRI/MRCP exams in the diagnosis of patients with SPN. Methods We retrospectively reviewed the medical records of patients undergoing EUS-FNA with suspected diagnosis of SPN on imaging studies in 5 high-volume hospitals. The final diagnosis was obtained after the histological examination of the surgical specimen. Demographic data, CT, MRI and EUS findings, anatomopathological specimen and McH results obtained by EUS were analyzed. Results Fifty-four patients were included, of which 49 (90.7%) were women with an average age of 33.4 (range 11–78) years. The most common symptom presented was abdominal pain, present in 35.2%. SPN was detected incidentally in 32 (59%). The mean size of the tumors was 3.8 cm (SD: 2.26). The most common findings at EUS were a solid, solid/cystic, and cystic lesion in 52.9%, 41.1% and 7.8%, respectively. The final diagnosis was SPN in 51 patients and NF-NET in 3. The correct diagnosis was made by CT, MRI, EUS and EUS-FNA in 21.9%, 28.9%, 64.7% and 88.2%, respectively. EUS-FNA associated with CT and MRI increased diagnostic performance to 94.11% and 94.11%, respectively. Conclusion Differential diagnosis between SPN and NF-NET with imaging tests can be difficult. EUS-FNA increases preoperative diagnosis in such cases and should be routinely used to rule out NF-NET.
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