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Objective:To investigate the clinical imaging features and prognosis of von Hippel-Lindau (VHL) syndrome associated with pancreatic lesions.Method:The retrospective case-control study was conducted. The clinicopathological data of 161 patients with VHL syndrome who were admitted to Peking University First Hospital from September 2010 to August 2022 were collected. There were 83 males and 78 females, with age of onset as 27.0(range, 8.0-66.0)years. Observation indicators: (1) imaging results of VHL syndrome associated with pancreatic lesions; (2) clinical characteristics of VHL syndrome associated with pancreatic lesions; (3) comparison of clinicopathological factors in patients with VHL syndrome associated with pancreatic cystic lesions; (4) comparison of clinicopathological factors in patients with VHL syndrome associated with pancreatic neuroendocrine neoplasms (pNENs). (5) Treatment and prognosis of patients with VHL syndrome associated with pancreatic lesions. Measurement data with normal distribution were represented as Mean± SD, and comparison between groups was conducted using the independent sample t test. Measurement data with skewed distribution were represented as M(range), and comparison between groups was conducted using the non-parameter test. Count data were described as absolute numbers, and comparison between groups was conducted using the chi-square test. Results:(1) Imaging results of VHL syndrome associated with pancreatic lesions. Of the 161 patients with VHL syndrome, there were 151 patients associated with pancreatic lesions and 10 patients not associated with pancreatic lesions. Of the 151 patients with VHL syndrome associated with pancreatic lesions, there were 136 patient with pancreatic cystic lesions and 34 patients with pNENs, 22 patients with both pNENs and pancreatic cystic lesions, and the type of pancreatic lesions could not be accurately determined in 3 cases. (2) Clinical characteristics of VHL syndrome associated with pancreatic lesions. The age of onset in 151 patients with VHL syndrome associated with pancreatic lesions was 33.0(range, 14.0-68.0)years. Cases with gene site mutation of exon 1, exon 2, exon 3 and other types of gene site was 51, 16, 43 and 41, respectively. There were 116 patients of VHL type 1 and 35 patients of VHL type 2. There were 92 patients with family history of VHL syndrome and 59 patients without family history of VHL syndrome. There were 127 patients combined with renal cell carcinoma, 112 patients combined with central nervous system lesions, 46 patients combined with retinal hemangioblastoma. Patients may combined with multiple lesions. (3) Comparison of clinicopathological factors in patients with VHL syndrome associated with pancreatic cystic lesions. The age of onset, VHL syndrome type (VHL1 type, VHL2 type) and cases combined with renal cell carcinoma were 32.5(range, 14.0-68.0)years, 110, 26 and 115 in 136 patients with VHL syndrome associated with pancreatic cystic lesions, versus 22.0(range, 8.0-64.0)years, 13, 12 and 14 in 25 patients with VHL syndrome not associated with pancreatic cystic lesions, showing significant differences in the above indicators between them ( Z=-3.384, χ2=9.770, 10.815, P<0.05). (4) Comparison of clinicopathological factors in patients with VHL syndrome associated with pNENs. The age of onset, gene mutation sites (exon 1, exon 2, exon 3, other types of gene site) and VHL syndrome type (VHL1 type, VHL2 type) were 33.5(range, 14.0-64.0)years, 12, 5, 14, 3 and 18, 16 in 34 patients with VHL syndrome associated with pNENs, versus 27.0(range, 9.0-66.0)years, 41, 12, 32, 42 and 105, 22 in 127 patients with VHL syndrome not associated with pNENs, showing significant differences in the above indicators between them ( Z=-4.030, χ2=8.814, 13.152, P<0.05). (5) Treatment and prognosis of patients with VHL syndrome associated with pancreatic lesions. Of the 161 patients with VHL syndrome, 3 patients underwent surgical treatment, and the remaining patients were followed up. All 161 patients with VHL syndrome were followed up for 6 (range, 1-12)years, in which 15 patients died and 146 patients alive during the follow-up. The follow-up time of 3 patients undergoing surgical treatment was 4, 14, 9 years, respectively, and all of them were alive. Conclusions:The clinical imaging features of pancreatic lesions related to VHL syndrome are cystic lesions and pNENs, which with the characteristics of multiple lesions and benign tumors. Such patients usually do not requiring surgical treatment and have good prognosis.
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Objective To explore the surgical indication,diagnosis and treatment of suspected pancreatic cystic lesions.Methods To-tally 341 patients were admitted into our hospital because of pancreatic cystic lesions from October 2010 to October 2015.Except the 278 confirmed cases,the clinical data of the rest 63 patients with vague diagnosis were retrospectively analyzed.Results The surgical indication of pancreatic cystic lesions were:the lesion diameter was more than 4 cm;the imaging diagnosis was malignant;the lesion was combined with obvious clinical symptoms which can not explain with other diseases;there were asymptomatic pancreatic cystic lesion and main pancre-atic duct dilation without surgical risk factors;the follow-up showed that the desease is in development.Endoscopic ultrasonography guided fine needle aspiration is a useful supplement for more accurate diagnosis.However,it is an uncertain diagnostic value currently.Conclusion Diameter of lesion,uncertain imaging diagnosis,severe abdominal symptoms,obstructive jaundice and abnormal serum tumor markers are the surgical indication for patients.
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Objective To investigate the value of contrast-enhanced ultrasound (CEUS) in differential diagnosis of pancreatic pseudocysts,cystadenomas and cystadenocarcinomas,so as to improve diagnostic accuracy of the condition.Methods The results of CEUS and conventional ultrasound for 71 patients with pancreatic cystic lesions were analyzed retrospectively,and compared with the finally obtained surgical-pathological examination to assess the ability of qualitative diagnosis between two methods.Results In 71 pancreatic cystic lesions,there were 32 pancreatic pseudocysts,24 cystadenomas and 15 cystadenocarcinomas.The sensitivity and accuracy of CEUS for pancreatic pseudocysts and cystadenocarcinomas were 84.4％,93.0％ and 86.7％,88.7％,which were higher than those of conventional ultrasound 56.3 ％,80.3 ％ and 53.3 ％,74.6％ (x2 =6.06,4.92,3.97,4.71,all P ＜ 0.05).Conclusion There are some similarities in terms of imaging manifestations among pancreatic pseudocysts,cystadenomas and cystadenocarcinomas.Combining conventional ultrasound with CEUS can help the differential diagnosis of pancreatic cystic lesions.
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Pancreatic cystic lesions are being recognized increasingly due to the development of computed tomography and magnetic resonance imaging.Differential diagnosis between pseudocysts and cystic tumors is the key point for pancreatic cystic lesions.Patients with pancreatic pseudocysts generally have a history of acute or chronic pancreatitis,whereas patients with cystic tumors did not have such a history.Some pancreatic cystic lesions did not have typical imaging presentations,and they were difficult to be diagnosed.Pancreatic cystic lesions could be divided into unilocular cysts,microcystic lesions,macrocystic lesions and cysts with a solid component according to the presentations under computed tomography and magnetic resonance imaging.This typing system is helpful for the diagnosis and differential diagnosis of pancreatic cystic tumors,as well as for the guidance of therapy.