Microcystic Adenoma Research Articles

Rate of growth of pancreatic serous cystadenoma as an indication for resection

The purpose of this study was to examine the natural history and growth rate of pancreatic serous cystadenomas (SCAs) to determine which factors lead to resection for these benign neoplasms. We reviewed retrospectively a prospectively maintained database, identifying patients diagnosed with SCAs of the pancreas. The diagnosis was made via a combination of classic imaging features with or without cyst aspiration results consistent with SCA. To determine growth rates, gamma regression models were used and the average was modeled using the log function. A prospectively maintained database of 1,241 pancreatic cystic neoplasms was queried from 1998 to 2010. A total of 219 patients (18%) were diagnosed with SCA, 194 in the surveillance group and 25 in the resection group. Twenty patients underwent resection after initial imaging principally for presence of symptoms and indeterminate diagnosis, and 5 underwent resection after surveillance for development of symptoms and/or rapid rate of growth. Rate of growth increased at a steady state over time, with an estimated doubling time of 12 years (95% confidence interval, 7.8-21.5). This study shows that growth patterns are similar for SCAs of the pancreas regardless ofinitial size. When doubling time is faster than 12 years, resection should be considered.

Clinical characteristics and treatment of 57 patients with serous cystadenoma of pancreas

Objective To analyse the diagnosis and treatment options of serous cystadenoma of the pancreas.Method The clinical data of 57 patients operated in the Tianjin Medical University Cancer Institute & Hospital from August 1996 to December 2011 with pathologically confirmed serous cystadenoma of pancreas after the operation were retrospectively studied.Results There were 13 males (22.8％) and 44 females (77.2％).The median age was 56.8 years.The patients were asymptomatic in 31.6％.CT was accurate in the diagnosis in 70.6％.All patients received surgical resection,inluding pancreaticoduodenectomy (n =17,29.8％),distal pancreatectomy (n =38,66.7％),palliative resection (n=1),and tumor enucleation (n=1).Postoperative complications developed in 6 patients.Histopathologically,there were 50 cases of serous microcystic adenoma (87.7％) and 7 cases of serous oligocystic adenoma (12.3 ％).One of these patients had developed into serous cystadenocarcinoma.At a follow-up of 12 months to 15 years,one patient with serous cystadenocarcinoma died 13 months after the operation.The remaining patients were all alive.Statistical analysis was performed based on the postoperative histopathological type and tumor size.The mean postoperative hospital stay of the group of patients with serous microcystic adenoma were significantly longer than the patients with serous oligocystic adenoma [(17.39±7.61) d vs (19.43±0.98) d,P=0.002].The incidence of patients with clinical symptoms was higher in the group of patients with tumor size ≥4 cm when compared with the patients with tumour size ＜4 cm.There was no significant difference on the other parameters.Conclusions Pancreatic serous cystadenoma is a rare pancreatic tumor,and it often happens in elderly women.Indications for surgical resection included symptomatic tumours,tumor diameter more than 4 cm,malignant biological behavior,malignancy could not be ruled out,and potentially malignant tumors.For asymptomatic patients and tumor size less than 4 cm,surgical resection should also be considered if the tumour progresses on follow-up. Key words: Pancreatic neoplasms; Neoplasms, cystic, mucinous, and serous; Surgical procedures, operative

Fine‐needle aspirations of pancreatic serous cystadenomas: Improving diagnostic yield with cell blocks and α‐inhibin immunohistochemistry

The diagnosis of serous cystadenoma (SCA), a rare benign pancreatic neoplasm, can alter the management of patients with pancreatic masses. Although characteristic imaging findings and fluid chemical analysis have been described, SCAs are not always recognized preoperatively. Furthermore, scant cellular yield on fine-needle aspiration (FNA) often leads to a nondiagnostic or nonspecific benign diagnosis. α-Inhibin (AI), a sensitive marker for SCA, is infrequently required for diagnosis in surgical specimens due to their characteristic histologic appearance. The objective of the current study was to determine whether AI staining can improve SCA diagnosis on FNA specimens. Fifteen confirmed cases of SCA with prior FNA specimens were selected for this study. FNAs were evaluated for cellularity, cellular arrangement, and cytomorphology. Resection specimens were reviewed. Of the 15 FNA cases, approximately 75% demonstrated scant cellularity (11 of 15 cases). On smears, the cells were arranged as flat sheets, corresponding to strips of cells on cell block sections. The cells were small and round to cuboidal, with clear cytoplasm; occasional plasmacytoid cells and oncocytic cells were identified. Flattened cells, corresponding to attenuated epithelial cells lining macrocysts on the resections, were also noted. Stromal fragments were present in 5 FNAs and correlated with the hyalinized stroma in the resection specimens. AI immunostaining was positive in 88% of cases (7 of 8 of cases), thereby supporting the diagnosis of SCA. The results of the current study indicate that low cellularity and bland cytology are inherent to SCAs. Performing cell blocks and AI staining on FNA specimens is useful for establishing the diagnosis of SCA. An immunohistochemical panel including AI, chromogranin, and synaptophysin may enhance the diagnostic accuracy of pancreatic FNA specimens.

Cystic Pancreatic Neoplasms: Imaging Features and Management Strategy

utmost importance. 5-8 The primary purpose of this review is to highlight the key imaging findings for an array of cystic pancreatic neoplasms. These include the relatively common masses: intraductal papillary mucinous neoplasm (IPMN), serous microcystic adenoma (SMA), and mucinous cystic neoplasm (MCN), as well as less common lesions including cystic endocrine tumors, solid pseudopapillary tumor (SPT), cystic metastases, and lymphangiomas. Finally, this article also provides a comprehensive management algorithm based on lesion size and patient’s symptoms, with recommendations when to reimage patients with those lesions.

Pancreatic serous cystadenoma with signs of compression

Pancreatic serous cystadenoma with signs of compression

Clinical presentation of Von Hippel Lindau syndrome type 2B associated with VHL p.A149S mutation in a large Turkish family

Von Hippel Lindau (VHL) syndrome is an autosomal dominant disorder characterized by benign and malignant tumors. This study presents the clinical and genetic features of VHL syndrome in a Turkish family. For the diagnosis of pheochromocytoma-related diseases, 49 family members from three generations were evaluated between March 2008 and January 2013. Family members were examined to identify components of pheochromocytoma-related genetic syndromes through physical examination, laboratory tests, and imaging methods. For the causative mutation, sequence analysis of VHL gene was performed. Nine patients were diagnosed with pheochromocytoma. Lumbal spinal hemangioblastoma and pancreatic neuroendocrine tumor without pheochromocytoma were detected in one patient. In patients with pheochromocytoma, additional tumors, such as retinal angioma, renal cell carcinoma, pancreatic serous cystadenoma, and pancreatic neuroendocrine tumors were detected. All patients were diagnosed as VHL syndrome type 2B. Sequence analysis of VHL gene revealed heterozygous p.A149S mutation in all symptomatic patients and in seven of the asymptomatic family members. This is the first study that identified VHL p.A149S mutation in a Turkish family with VHL syndrome. However, VHL p.A149S mutation was identified in an American family by Atuk et al. (J Clin Endocrinol Metab, 83:117-120, 14) and the family was defined as VHL type 2A. In our study, the family was identified as VHL type 2B. This variability in the phenotypic features suggests that further studies are required to beter assess the genotype-phenotype correlation in such cases.

Tu1226 In Vivo Characterization of Pancreatic Serous Cystadenomas by Needle-Based Confocal Laser Endomicroscopy (nCLE). Intra and Inter Observer Agreement - Contact Study

diagnosis. We requested further information from the authors if any clarification was needed. Pooled estimates of sensitivity, specificity, likelihood ratio (LR) and diagnostic odds ratio (DOR) were calculated using random-effects models. Summary receiver-operating characteristic (sROC) curves were used to analyze the diagnostic accuracy of cystic fluid CEA in distinguishing benign and malignant pancreatic cysts. Results: Of 842 articles, 8 studies (n= 504) met the inclusion criteria. The cut-off for determining malignant cyst ranged from 109.9 6,000 ng/ml among various studies. The pooled estimates of intracystic CEA level in prediction of malignant pancreatic cysts were poor, with a pooled sensitivity of 63% (95% CI, 55-69), pooled specificity of 63% (95% CI, 57-68). The positive LR was 1.89 (95% CI, 1.13-3.16) and the negative LR was 0.62 (95% CI, 0.40-0.97). The DOR was 3.84 (95% CI, 1.37-10.74). Heterogeneity was observed (I2 = 76%, p , 0.0001). The area under the sROC curve was 0.70 with an SE of 0.09. The maximum joint sensitivity and specificity (Q* value) was 0.66 with an SE of 0.07. In subgroup analysis of patients with mucinous cysts (mucinous cystic neoplasm and IPMN) (5 studies, 227 patients), the pooled sensitivity of intracystic CEA level in prediction of malignant pancreatic cysts was 65% (95% CI, 5773), with the pooled specificity of 66% (95% CI, 59-72) and DOR of 4.74 (95% CI, 1.4615.37). Conclusion: This meta-analysis suggested that the accuracy of intracystic CEA level in differentiating benign and malignant pancreatic cysts was poor. The decision to perform surgical resection for pancreatic cystic lesions including IPMN should not be based solely on a very high intracystic CEA level.

Pancreatic Cystic Neoplasms: Management and Unanswered Questions

Approximately 10% of persons 70 years old or older are now diagnosed with pancreatic cysts, but it is not clear which ones require additional analysis, interventions, or follow-up. Primary care doctors rely on gastroenterologists for direction because no one wants to miss a diagnosis of pancreatic cancer, but meanwhile there is pressure to limit use of diagnostic tests and limit costs. We review the different cystic neoplasms of the pancreas and diagnostic strategies based on clinical features and imaging data. We discuss surgical and nonsurgical management of the most common cystic neoplasms, based on the recently revised Sendai guidelines. Intraductal papillary mucinous neoplasm (particularly the branch duct variant) is the lesion most frequently identified incidentally. We report what is known about its pathology, its risk of developing into pancreatic ductal adenocarcinoma, the pros and cons of current guidelines for management, and the potential role of endoscopic ultrasound in determining cancer risk. We also review surgical treatment and strategies for surveillance of pancreatic cysts.

Imaging appearances of von Hippel-Lindau disease

Objective To assess imaging appearances of von Hippel-Lindau disease (VHLD). Methods The clinical and imaging data of ten patients who met VHLD gene diagnostic criteria were retrospectively analyzed.Seven underwent CT abdominal scan,three underwent MRI abdominal scan.All patients underwent MRI cranial scan.Eight patients were treated by surgery,and three of them underwent endoscopic ultrasound guided aspiration biopsy of pancreatic tumors. Results All patients had multiple cysts of the pancreas,eight patients suffered multiple cysts of kidneys.Three patients suffered multiple islet cell tumors of pancreas: one was accompanied with pancreatic serous cystadenoma,another was accompanied with left adrenal pheochromocytoma.Three patients had bilateral renal cell carcinoma (one of them had the right kidney cut off),one patient had right renal cell carcinoma.Two patients suffered cerebellar hemangioblastomas,and one of them was accompanied with mutiple spinal hemangioblastomas and the left retinal hemangioblastoma.Two patients with multiple cysts of pancreas and kidneys had not been found any tumor. Conclusion Multiple cysts of pancreas and kidney are highly suggestive of VHLD.At the same time,endocrine tumors of pancreas,serous cystadenoma of pancreas,adrenal pheochromocytoma,renal carcinomas,multiple hemangioblastomas of central nervous system and retinal hemangioblastoma can also occur in VHLD. Key words: Pancreatic neoplasm; Hemangioblastoma; Renal neoplasms

Various Abdominal and Endoscopic Ultrasonographic Appearances of Pancreatic Serous Cystadenoma and Pancreatic Neoplasms Mimicking Serous Cystadenoma

Various Abdominal and Endoscopic Ultrasonographic Appearances of Pancreatic Serous Cystadenoma and Pancreatic Neoplasms Mimicking Serous Cystadenoma

Lipid-rich variant of pancreatic endocrine tumour with inhibin positivity and microscopic foci of microcystic adenoma-like areas: emphasis on histopathology

Pancreatic endocrine tumours (PETs) are uncommon tumours with typical morphology characterised by relatively uniform cuboidal cells arranged in nests and festoons, with distinctive nuclear salt-and-pepper chromatin. A lipid-rich variant poses diagnostic difficulties in the midst of other pancreatic tumours and metastatic goblet cell carcinoid. A 22-year-old man presented with symptoms of abdominal pain and jaundice. His liver function test and blood glucose level were normal, but computed tomography of the abdomen suggested the presence of a tumour in the head of the pancreas. Specimen obtained by pancreaticoduodenectomy revealed an infiltrating yellow-tan tumour composed of nests and a cribriform arrangement of polygonal vacuolated cells with pyknotic nuclei, along with focal classical areas of PET. Two foci of early serous microcystic adenoma were seen. Immunohistochemistry contributed to the arrival of a conclusive diagnosis. Von Hippel-Lindau disease was excluded in our patient, as other supportive classical features of the syndrome were absent.

微小膵漿液性嚢胞腺腫の1例

症例は43歳の男性で，繰り返す膵炎とCTにて限局的な尾側膵の腫大と膵管の拡張を認めたため当院紹介となった．保存的加療で急性膵炎が軽快した後，原因精査を行った．膵炎軽快後のCTでも尾側膵管の拡張は残存していたが，腫瘤像は認めず，EUSでも明らかな腫瘤像は認めなかった．PET-CTでも膵にFDGの有意な取り込みは認めなかった．ERCPでは膵尾部主膵管に狭窄像とその尾側膵管の拡張が認められた．膵管擦過細胞診ではclass II，膵液細胞診では疑陽性の結果を得た．小膵癌を否定できず膵体尾部・脾合併切除を施行した．病理組織学的検査所見では，膵尾部の膵管内に管腔状，一部乳頭状に発育する立方状膵管上皮を認め，PAS染色にて一部陽性であり膵漿液性嚢胞腺腫と診断した．今回，われわれは繰り返す膵炎で発症し術前画像診断では捉えられなかった主膵管内の2mm大の膵漿液性嚢胞腺腫の1例を経験したので報告する．

Automatic differential diagnosis of pancreatic serous and mucinous cystadenomas based on morphological features

Generally, pathological diagnosis using an electron microscope is time-consuming and likely to result in a subjective judgment, because pathologists perform manual screening of tissue slides at high magnifications. Recently, the advent of digital pathology technology has provided the basis for convenient screening and quantitative analysis by digitizing tissue slides through a computer system. However, a screening process with high magnification still takes quite a long time. To solve these problems, recently the use of computer-aided design techniques for performing pathologic diagnosis has been increasing in digital pathology. For pathological diagnosis, we need different diagnostic methods for different regions with different characteristics. Therefore, in order to effectively diagnose different lesions and types of diseases, a quantitative method for extracting specific features is required in computerized pathologic diagnosis. This study is about an automated differential diagnosis system to differentiate between benign serous cystadenoma and possibly-malignant mucinous cystadenoma. In order to diagnose cystic tumors, the first step is identifying a cystic region and inspecting its epithelial cells. First, we identify the lumen boundary of a cyst using the Direction Cumulative Map considering 8-ways. Then, the Epithelial Nuclei Identification algorithm is used to discern epithelial nuclei. After that, three morphological features for the differential diagnosis of mucinous and serous cystadenomas are extracted. To demonstrate the superiority of the proposed features, the experiments compared performance of the classifiers learned by using the proposed morphological features and the classical morphological features based on nuclei. The classifiers in the simulations are as follows; Bayesian Classifier, k-Nearest Neighbors, Support Vector Machine, and Artificial Neural Network. The results show that all classifiers using the proposed features have the best classification performance.

Morphological Analysis and Differentiation of Benign Cystic Neoplasms of the Pancreas Using Computed Tomography and Magnetic Resonance Imaging

To evaluate morphologic characteristics and establish a standardized diagnostic algorithm to differentiate benign cystic pancreatic tumors (CPTs) in non-pancreatitis patients using multidetector computed tomography (CT) and magnetic resonance imaging (MRI). Patients with histopathologically proven CPTs who had undergone MRI and/or CT and subsequent tumor resection in our institution were retrospectively identified. Images were analyzed for morphology and enhancement patterns by three independent blinded observers. Preoperative image findings were correlated with histopathological results. Based on the evaluated morphologic parameters, a standardized diagnostic algorithm was designed to help characterize the lesions. A total of 62 consecutive patients with 64 CPTs were identified from the surgical database (21 intraductal papillary mucinous neoplasms; 10 mucinous cystic neoplasms; 12 serous microcystic adenomas; 3 serous oligocystic adenomas; 6 solid pseudopapillary tumors; 12 neuroendocrine neoplasms). The overall averaged accuracy for the 3 observers was 89.9 % for CT and 93.1 % for MRI with increasing overall accuracy in relation to the experience of the observer (88.2 %, 91.5 %, and 93.8 %, respectively). Overall, the generalized kappa value was 0.69 (CT, 0.64; MRI, 0.76); p < 0.001). The accuracy of the standardized diagnostic algorithm was 91.1 %. It is possible to characterize benign CPTs with MRI and CT, while MRI appears to be superior to CT. Diagnostic accuracy depends on the observer's experience. The standardized algorithm can aid in the differential diagnosis but still needs to be tested in other patient populations.

Endoscopic Ultrasound Fine Needle Aspiration Biopsy of Serous Cystadenoma of the Pancreas in Patients with Surgical Excision

Endoscopic Ultrasound Fine Needle Aspiration Biopsy of Serous Cystadenoma of the Pancreas in Patients with Surgical Excision

Von Hippel-Lindau disease–associated solid microcystic serous adenomas masquerading as pancreatic neuroendocrine neoplasms

Patients with von Hippel-Lindau disease (VHL) commonly develop pancreatic cysts and neuroendocrine neoplasms (PNENs or PNETs). Solid microcystic serous adenoma (SMSA), a rare neoplasm described in VHL patients, can be mistaken for PNEN on imaging. Clinical, pathologic, and radiologic data were reviewed on VHL patients who underwent surgery for a preoperative diagnosis of PNEN since 1994 at 1 institution. Blinded to the pathologic diagnoses, radiologists reassessed available imaging. For 55 patients, 79 pancreatectomies were performed for presumed PNENs. Ten (18%) patients underwent 12 (15%) resections for neoplasms diagnosed as SMSA on final pathology. The average size of a SMSA leading to operation was 3.6 ± 0.4 cm. Four out of 11 SMSAs were still mistaken for PNENs when imaging was reassessed. The mean FDG-positron emission tomography (PET) standardized uptake value was greater for 17 PNENs (12.1 ± 1.2) compared with 6 SMSAs (4.2 ± 0.5; P = .002). The mean doubling time of SMSAs and PNENs was similar. Seven (15%) patients with pathologically proven PNENs had malignant disease. SMSAs can mimic PNENs on nonfunctional imaging; FDG-PET may help to differentiate them. A high index of suspicion is needed to minimize operations performed for SMSA and to counsel VHL patients of their risks of undergoing operation for a lesion with no known malignant potential.

Giant serous microcystic pancreas adenoma

Serous cystadenomas are rare tumors comprising 1–2% of exocrine pancreas tumors. They are mostly known as benign conditions but malign transformation as serous cystadenocarcinoma is also reported. It is usually seen in females. Non-specific symptoms, such as abdominal pain or symptoms due to mass affect, are usually seen. A 64-year old female patient was investigated for abdominal pain. Physical and laboratory findings were normal. Abdomen ultrasonography confirmed an 11×9.5 cm solid cystic lesion and abdomen computed tomography scan confirmed a 12×11 cm lobulated cystic solid lesion which had central cystic necrotic areas extending from liver hilus inferiorly. Fine needle biopsy confirmed benign cytology and trucut biopsy of the pancreatic mass reported chronic inflamation. Nevertheless, this mass could have malignant contents and transformation potential. A laparatomy was decided due to patient's symptoms and mass effect. Due to vascular invasion of the tumor, Whipple procedure was performed. The pathology report confirmed serous microcystic adenoma. These rare tumors are usually benign but pre-operative malignity criterias are not identified. There are few differential diagnostic tools for excluding malignity. We suggest surgical resection as best treatment approach for selected cases.

DOG1 (Clone K9) is Seldom Expressed and Not Useful in the Evaluation of Pancreatic Neoplasms

DOG1, a transmembrane calcium-regulated chloride channel protein, is a sensitive and specific marker for gastrointestinal stromal tumors compared with other spindle cell and epithelioid neoplasms. Overexpression has also been described in a variety of both benign and malignant epithelial neoplasms. Recently, DOG1 immunoreactivity has been reported in pancreatic solid pseudopapillary tumors (SPT), suggesting a role as a marker for SPT. Utilizing immunohistochemistry, we evaluated DOG1 expression in pancreatic neoplasms to determine the prevalence of staining and establish diagnostic utility. Multiple tissue microarrays (TMA) were created from cores of formalin-fixed paraffin-embedded blocks containing pancreatic adenocarcinomas (n=112), neuroendocrine tumors (n=99), serous cystadenomas (n=28), and SPT (n=14) as well as normal pancreas (n=12). Immunoreactivity for DOG1 (clone K9) was assessed for intensity (1 to 3+), percentage of tumor positivity and location. Of the 99 cases of neuroendocrine tumors, only 2 (2%) were focally positive. Patchy staining was identified in 8 cases (7%) of adenocarcinoma of 1 to 2+ intensity, involving 15% to 80% of the tumor cells and primarily seen in a membranous and luminal distribution. In contrast to a previous report, no DOG1 positivity was observed in SPT, evaluated by both TMA and full sections. The TMAs of serous cystadenomas and normal pancreas were negative for DOG1. Rarely, pancreatic islets displayed granular, cytoplasmic staining. DOG1 antibody clone K9 is not a useful marker for SPT or other primary pancreatic neoplasms. Additional studies may be helpful to evaluate differences between clones of DOG1.

Internal pancreatic stent causing irreversible dilatation of pancreatic duct.

A 28-year-old woman underwent a pylorus preserving Whipple procedure for pancreatic serous cystadenoma located on the head of the pancreas. During the operation, an internal stent (7F silastic catheter, 9cm in length) was placed within the pancreatic duct in the area of pancreaticojejunal end-to-end Dunking type anastomosis to prevent development of fistula. The stent was positioned so that one third of its length would lie into the pancreatic duct, and it was anchored to the periductal pancreatic tissue with only one rapidly absorbable chromic suture. Leakage from the anastomosis was not observed, and she was discharged without any complaint. Early postoperative abdominal CT examination revealed that the stent was retained within the normal caliber pancreatic duct (Fig.1a). Six months after the operation, she began to complain to epigastric pain triggered by the meals. The laboratory analysis was normal, particularly liver biochemical tests and serum amylase. The internal pancreatic stent within the dilated pancreatic duct was detected by an additional CT examination (Fig.1b). The stent was removed endoscopically at the third attempt. The pain was resolved after its removal. Control CT examination which was taken at the 18th month after removal of the stent showed dilatation of the pancreatic duct (Fig.2a). The patient remained free of any complaint, although regressed pancreatic duct dilatation has persisted over 4years of follow-up (Fig.2b).

PMO-140 Analysis of EUS-guided cyst aspirate has no impact on surgical management of suspected pancreatic cystic tumour

IntroductionPreferred strategies for evaluation and management of patients with pancreatic cysts remain controversial. EUS-guided fine needle aspiration (FNA) of suspected pancreatic cyst tumours for CEA and cytology is often recommended...