Multidisciplinary Treatment on a Case of ROSAH Syndrome / 罕见病研究

A 15-year-old female was referred to the hospital with intermittent fever, where multiple systemic abnormalities were found, such as splenomegaly, secondary hypersplenism, retinitis pigmentosa, and ectodermal dysplasia. Medical history revealed that she had suffered recurrent respiratory infections, blurred vision at night, and dysplasia of teeth and nail beds since childhood. Then she was suspected to be experiencing ROSAH syndrome, a rare disease newly recognized in recent years, which was finally confirmed by gene sequencing results. During a course of treatment with tumor necrosis factor inhibitors, recurrent fever with elevated inflammatory markers reappeared, and the child developed headaches. To guide the comprehensive treatment and improve the patient's quality of life, the multidisciplinary team in Peking Union Medical College Hospital discussed together and directed the following treatment.

Pancreatic serous microcystic neoplasm with atypical clinical and image features / 中华肝胆外科杂志

Objective:To summarize the atypical manifestations and treatment strategies of serous microcystic neoplasm of the pancreas.Methods:Review the case data of 11 cases of pancreatic serous microcystic adenoma with atypical preoperative clinical imaging findings admitted to Peking Union Medical College Hospital from July 2008 to October 2019, and summarize their clinical manifestations, CT/MRI features, and preoperative clinical diagnosis, surgical methods, postoperative conditions.Results:The median age of the 11 patients was 50 (46-66) years old, 7 females, and 4 males. There were 8 cases with back pain and 6 cases with weight loss. The preoperative imaging examination found that the lesion was located in the head and neck in 8 cases, with pancreaticobiliary duct dilatation in 7 cases, suspected vascular invasion in 3 cases, and pancreatic parenchymal atrophy in 2 cases, 3 cases showed rich blood supply and solid space, and 2 cases were connected to the pancreatic duct. Preoperative diagnosis of pancreatic malignant tumors accounted for 4 cases, neuroendocrine tumors in 3 cases, solid pseudopapillary tumors in 3 cases, and intraductal papillary mucinous tumors in 1 case. All patients underwent surgical treatment, 3 cases of distal pancreatectomy (2 cases of using Kimura method to protect the spleen), 3 cases of pancreaticoduodenectomy, 1 case of total pancreatectomy, 2 cases of pancreatic head resection with duodenum preservation, 2 cases of local resection. All patients had no early postoperative deaths. There were 2 cases of grade B pancreatic fistula, 2 cases of biochemical leakage, 1 case of postoperative abdominal hemorrhage, and 2 cases of delayed gastric emptying, all of which were cured after active treatment. The median postoperative hospital stay was 18 (7-63) days.Conclusions:Pancreatic serous microcystic neoplasm could be accompanied by atypical features such as pancreatic/bile duct dilation, parenchyma atrophy, or even present vessel invasion. Understanding the atypical clinic and image features would help improving differential diagnosis and treatment. For those with invasive features, surgical exploration should be recommended.

Metformin inhibits pancreatic cancer metastasis caused by SMAD4 deficiency and consequent HNF4G upregulation

Pancreatic ductal adenocarcinoma (PDAC) has poor prognosis due to limited therapeutic options. This study examines the roles of genome-wide association study identified PDAC-associated genes as therapeutic targets. We have identified HNF4G gene whose silencing most effectively repressed PDAC cell invasiveness. HNF4G overexpression is induced by the deficiency of transcriptional factor and tumor suppressor SMAD4. Increased HNF4G are correlated with SMAD4 deficiency in PDAC tumor samples and associated with metastasis and poor survival time in xenograft animal model and in patients with PDAC (log-rank P = 0.036; HR = 1.60, 95% CI = 1.03-2.47). We have found that Metformin suppresses HNF4G activity via AMPK-mediated phosphorylation-coupled ubiquitination degradation and inhibits in vitro invasion and in vivo metastasis of PDAC cells with SMAD4 deficiency. Furthermore, Metformin treatment significantly improve clinical outcomes and survival in patients with SMAD4-deficient PDAC (log-rank P = 0.022; HR = 0.31, 95% CI = 0.14-0.68) but not in patients with SMAD4-normal PDAC. Pathway analysis shows that HNF4G may act in PDAC through the cell-cell junction pathway. These results indicate that SMAD4 deficiency-induced overexpression of HNF4G plays a critical oncogenic role in PDAC progression and metastasis but may form a druggable target for Metformin treatment.

Selection strategy of neoadjuvant therapy for pancreatic cancer / 中华消化外科杂志

Pancreatic cancer is a common malignancy with the worst prognosis.Radical surgery has been the only curative treatment for pancreatic cancer.With the advancement of surgical techniques and the implementation of the concept of comprehensive treatment for cancer in recent years,neoadjuvant therapy for pancreatic cancer has received more attention.There are continuing controversies in the hotspots and difficulties,with opportunities and challenges coexisting.Four famous experts and their teams in pancreatic surgery discussed selection strategy of neoadjuvant therapy for pancreatic cancer based on clinical experiences.Professor Wang Chunyou proposed that surgery was prior for patients with a higher likelihood of achieving R0 resection for pancreatic cancer to avoid the possibility of tumor progression and loss the opportanity of radical resection during neoadjuvant therapy.For patients with less chance of radical resection for pancreatic cancer and unresectable pancreatic cancer,neoadjuvant therapy is worthy of a positive attempt.Professor Jin Gang and his team believed that neoadjuvant therapy played an important role in improving the survival time of patients with pancreatic head cancer,especially with borderline resectable pancreatic head cancer.After neoadjuvant therapy,pancreatic surgeons should pay attention to improvement of surgery safety and R0 resection rate.Professor Dai Menghua and his team suggested that patients with resectable pancreatic cancer and borderline resectable pancreatic cancer could benefit from neoadjuvant therapy,which required proof from clinical trials.Surgeons should choose the appropriate treatment strategy based on guidelines and individual conditions for patients with pancreatic cancer.Professor Shao Cheghao and his team suggested that surgical treatment after neoadjuvant therapy or translational therapy for locally advanced pancreatic head cancer is safe,effective and feasible,especially for pancreaticoduodenectomy with combined revascularization.For the treatment of patients with pancreatic head cancer after neoadjuvant chemotherapy,the choice of next treatment options,evaluation indicators,timing of surgery and surgical methods need to be further studied.

Imaging anatomy and clinical significance of the inferior pancreaticoduodenal veins / 中华消化外科杂志

Objective To investigate the imaging anatomy and clinical significance of the inferior pancreaticoduodenal veins (IPDVs).Methods The retrospective and descriptive study was conducted.The clinicopathological data of 42 patients with pancreatic head ductal adenocarcinoma who were admitted to Peking Union Medical College Hospital from January to June 2018 were collected.There were 24 males and 18 females,aged from 41 to 78 years,with an average age of 61 years.Patients received preoperative contrast-enhanced computed tomography (CT) examination with 1 mm slice thickness,and underwent corresponding surgery according to the preoperative evaluation.Observation indicators:(1) results of preoperative CT examination;(2) surgical situations.Normality of measurement data was analyzed using Shapiro-Wilk test.Measurement data with skewed distribution were described as M (QR) or M (range),and comparison between groups was analyzed by the Mann-Whitney U test.Count data were described as absolute number or percentage,and comparison between groups was analyzed by the chi-square test.Results (1) Results of preoperative CT examination:42 patients received preoperative contrast-enhanced CT examination with 1 mm slice thickness.① The first jejunal venous trunk was identified in all the 42 patients.The first jejunal venous trunk crossed dorsal to the superior mesenteric artery (SMA) in 34 patients and ventral to the SMA in 8 patients.② Of 42 patients,2 showed no IPDV,and 40 showed IPDV including 23 with 1 IPDV,13 with 2 IPDVs,3 with 3 IPDVs,and 1 with 4 IPDVs.A total of 62 IPDVs were identified in the 42 patients,with an average IPDV number of 1 (range,0-4).There were 43 IPDVs drained into first or second jejunal venous trunks and 19 IPDVs drained into superior mesenteric vein (SMV).③ Of 42 patients,type Ⅰ IPDV was identified in 32 patients including 20 with 1 IPDV drained into jejunal venous trunk at dorsal side of SMA,7 with 2 IPDVs drained into jejunal venous trunk at dorsal side of SMA,2 with 3 IPDVs drained into jejunal venous trunk at dorsal side of SMA,and 3 with 1 IPDV drained into jejunal venous trunk at ventral side of SMA,and non-type Ⅰ IPDV was identified in 10 patients;type Ⅱ IPDV was identified in 18 patients including 17 with 1 IPDV drained into SMV and 1 with 2 IPDVs drained into SMV,and non-type Ⅱ IPDV was identified in 24 patients.Some patients can simultaneously had type Ⅰ and type Ⅱ IPDV.(2) Surgical situations:42 patients underwent pancreatoduodenectomy,14 of which underwent laparoscopic surgery and 28 underwent open surgery.There were 5 cases with SMV or portal vein reconstruction,and 18 with intraoperative blood transfusion.All the 42 patients were diagnosed as pancreatic ductal adenocarcinoma by postoperative pathological examination,including 30 of R0 resection and 12 of R1 resection.The volume of intraoperative blood loss,cases with intraoperative blood transfusion,cases with R0 and R1 resection (situation of surgical margin),cases with SMV or portal vein reconstruction were 650 mL(853 mL),15,20,12,4 in the 32 patients with type Ⅰ IPDV,aod 475 mL (480 mL),3,10,0,1 in the 10 patients with non-type Ⅰ IPDV;there were significant differences in the volume of intraoperative blood loss and situation of surgical margin (Z=94.000,x2=5.250,P＜ 0.05).There was no significant difference in the cases with intraoperative blood transfusion,cases with SMV or portal vein reconstruction between patients with type Ⅰ and non-type Ⅰ IPDV (x2 =0.045,0.886,P＞0.05).Conclusions IPDVs can be distinguished on the contrast-enhanced CT with slice thickness,and classified as IPDVs drained into SMV or jejunal venous trunk.It is necessary to carefully deal with IPDVs drained into jejunal venous trunk in the pancreaticoduodenectomy due to its more volume of intraoperative blood loss and lower R0 resection rate.

Progress in neoadjuvant and transformation therapy of pancreatic cancer / 中华外科杂志

Pancreatic adenocarcinoma is a malignant disease with considerable metastatic potential.While surgical resection can be potentially curative, tumor recurrence remains an important cause of treatment failure.Neoadjuvant chemotherapy can increase rate of resectability by decreasing tumor burden and decrease recurrence rate by clearing microscopic disease in lymph nodes and vessels.Currently, neoadjuvant therapy is recommended for patients with resectable who has signs of high risks or borderline resectable pancreatic adenocarcinoma.However, no consensus exists in current literature on the evaluation of treatment response or operative timing.FOLFIRINOX has recently emerged as an effective chemotherapy regimen in several large clinical trials.

Impact of the 2016 new definition and classification system of pancreatic fistula on the evaluation of pancreatic fistula after pancreatic surgery / 中华外科杂志

Objective@#To discuss the impact of updated definition and classification system of pancreatic fistula published in 2016 on the postoperative classification of pancreatic fistula.@*Methods@#Retrospective analysis was made on patients who underwent pancreatic surgery at ward 1 in Department of General Surgery, Peking Union Medical College Hospital from January 2015 to December 2016.A total of 408 patients were included in this retrospective study, male/female was 184/224, aged from 9 to 81 years with mean age of 51.6 years.One hundred and fifty-two cases were performed pancreaticoduodenectomy, 125 cases for distal pancreatectomy, 43 cases for spleen preservation distal pancreatectomy, 61 cases for partital pancreatectomy or enucleation, 8 cases for middle pancreatectomy, 6 cases for pancreaticojejunostomy and 13 cases for other procedures.Clinical data including postoperative drainage fluid volume, amylase concentration, duration of hospitalization and drainage were obtained, revaluated and re-analyzed, classified grounded on 2005 edition and 2016 edition, respectively.t-test was adopted for data analysis.@*Results@#According to the previous standards, the incident rate of pancreatic fistula was 57.4%, and the incident rate of B-level plus C-level pancreatic fistula was 35.8%, which decreased to 13.7% based on 2016 edition.Nine patients who received percutaneous puncture or endoscopic drainage was regraded from C-level to B-level. The average duration of postoperative hospitalization of patients without pancreatic fistula was (12.5±6.0)days, demonstrating no significant difference compared to (14.1±7.7)days, duration of postoperative hospitalization of A-level(under 2005 edition of criteria) pancreatic fistula group(t=1.66, P=0.09) and (12.4±6.1)days, duration of postoperative hospitalization of biochemical leakage group(t=0.14, P=0.89). Nevertheless, there was statistical significant difference between the average postoperative duration of hospitalization(30.7±16.9) days of B-level(under 2016 criteria) pancreatic fistula patients and pancreatic fistula-free patients as well as the biochemical leakage group patients (t=7.10, 7.13; both P<0.01).@*Conclusions@#Based on the new diagnostic criteria, the incidence of postoperative pancreatic fistula decreased dramatically.New classification system downgraded part of cases graded C-level pancreatic fistula to B-level and some B-level to biochemical fistula.The new diagnostic classification and criteria facilitated clinical practice, accomplished better conformity to clinical reality and potentially enacted clinical outcome.

Laparoscopic segmental gastrectomy for early gastric cancer / 中华胃肠外科杂志

<p><b>OBJECTIVE</b>To investigate the feasibility of segmental pylorus-reservation gastrectomy in patients with early gastric cancer.</p><p><b>METHOD</b>A retrospective cohort study on clinical data of 6 patients strictly met the criteria of early gastric cancer locating in the middle of the stomach undergoing laparoscopic segmental gastrectomy from January 2014 to April 2016 at Department of General Surgery, Peking Union Medical College Hospital was carried out. Preoperative clinical staging revealed T1N0M0 for all the cases. One case received endoscopic mucosa resection(EMR) first, and postoperative pathology showed moderate differentiated adenocarcinoma invading substratum of mucosa, so a complementary laparoscopic segmental gastrectomy was performed. Surgical procedure was laparoscopic segmental gastrectomy with D1 or D2 lymph node dissection. Vagus nerve was not reserved during lymph node dissection in lesser curvature side. Number of resected lymph node, postoperative complication and long-term gastric function were observed.</p><p><b>RESULT</b>Of 6 cases, 3 were male and 3 were female with age ranging from 55 to 59 years old. The distal resection margin was (4.6±0.5) cm away from pylorus. The average number of resected lymph node was 18.3±7.5 without metastasis. Follow-up time was 1 to 29 months for all the 6 cases and no relapse or metastasis was found during the follow-up. In 4 cases with follow-up beyond 1 year, 3 cases had slight distension in superior belly after meal and dyspepsia; another one case had vomiting nocturnal occasionally. Gastroscope examination one year after operation found food residue in all the cases. Images indicated the decrease of stomach size in all the cases. Two cases had esophagogastric reflux. All the patients had delayed gastric emptying symptoms after operations and were relieved within one year.</p><p><b>CONCLUSIONS</b>It is technically feasible to perform laparoscopic segmental gastrectomy in patients with early gastric cancer. Whether vagus nerve should be reserved requires further investigation.</p>

Retrospective study on post-operative glucose level and insulin dose in patients undergoing total pancre-atectomy / 中华临床营养杂志

Objective To evaluate the post-operative glucose level and insulin dose of patients undergoing total pancreatectomy.Methods From September 1980 to September 2014, 21 patients underwent total pancrea-tectomy in Peking Union Medical College Hospital, who were enrolled in our study.We reviewed the changes in their insulin dosage and glucose levels after operation, also summarized type and dose of insulin as well as glucose level in stable period.Results The required insulin dose reached peak within 4 days after surgery ( maximum dose 300 U/d).The average dose was (143.5 ±62.8) U/d and decreased gradually.During the perioperative period (needing parenteral nutrition), the blood glucose level fluctuated markedly (1.52-29.06 mmol/L) and the average level was (11.18 ±0.95) mmol/L.During the stable period ( without parenteral nutrition) , patients on average had (5.3 ±2.0) U of preprandial rapid-acting insulin and (8.1 ±2.9) U of long-acting insulin be-fore sleeping;the average fasting blood glucose was (6.69 ±1.48) mmol/L, 2 h postprandial blood glucose was (9.08 ±2.84) mmol/L, bedtime blood glucose was (9.66 ±2.49) mmol/L, and blood glucose level at night was (8.15 ±2.78) mmol/L.67%of the patients had 13 hypoglycemic episodes monthly on average.For those five followed-up patients, the average hemoglobin A1c was (6.15 ±1.20)%.Conclusions Patients undergoing total pancreatectomy may experience marked fluctuation of blood glucose level and short-term increase of insulin need which gradually decreases afterwards.After entering the stable period, the glucose level could be well-con-trolled but with frequent hypoglycemia.There is no diabetic ketoacidosis.

Combination of intraoperative ultrasonography for localizing insulinoma under Da Vinci robotic surgical system: experience of a single center in 50 cases / 中华外科杂志

<p><b>OBJECTIVE</b>To evaluate the effect and safety of enucleation of insulinoma under the Da Vinci robotic surgical system combination with intraoperative ultrasonography(IOUS) for the localization.</p><p><b>METHODS</b>The clinical materials of 50 insulinoma cases which underwent IOUS and assisted by the robotic surgical system from September 2012 to September 2014 in Peking Union Medical College Hospital were reviewed retrospectively. The patients were followed up by outpatient review and telephone until October 2014. The diagnostic accuracy rate, operation time, blood loss, complications and cure rate were analyzed by t-test.</p><p><b>RESULTS</b>The locations of tumors were 13 in the head, 21 in the body and 13 in the tail of pancreas, 2 were multiple insulinoma, 1 was ectopic to mesenterium.The average operation time was 142 minutes; the average blood loss was 165 ml.Three(6.0%) patients were transformed to open.One patient experienced postoperative bleeding about 300 ml on the 7(th) day after operation and no infection and perioperative death.Thirty-five cases were of class A and 14 of class B according to the clinical grading of postoperative pancreatic fistula.The blood glucose 60 minutes after tumor dissection was significantly elevated than that before operation ((6.2±1.8)mmol/L vs.(3.7±1.2)mmol/L)(t=-6.89, P<0.01). The cure rate was 100% as all the patients' symptoms were disappeared during follow-up time.</p><p><b>CONCLUSIONS</b>Combination IOUS is a highly sensitive method for the localization of insulinoma, which is helpful in localizing tumors precisely in insulinoma cases assisted by robotic surgical system and shortening operation time.It is safe and effective for insulinoma enucleation.</p>

To study the variation of glucose and its management in patients with insulinomas / 中华肝胆外科杂志

Objective To retrospectively study the variation of glucose in patients with insulinomas. Methods The medical records of patients who underwent operations in the last 20 years at Peking Union Medical College Hospital were reviewed retrospectively. Patients who received repeated operations, or just exploratory laparotomy or liver biopsy were excluded. The clinical data were collected and analyzed. Results There were 245 patients, including 103 males and 142 females. The mean age was (42.2+ 14.6) years old. The mean value of preoperative fasting glucose was (1.47+0. 57) mmol/L, and the ratio of insulin to glucose was greater than 0.3 in 87.4% patients. The glucose level rose gradually after removal of the tumor(s). The blood sugar rising more than 1 time over the baseline value at one hour after resection was observed in 79.6% patients. For all the other patients, the glucose level rose to this level in the morning of the first postoperative Day 1. Rebound hyperglycemia was common after operations, and the glucose gradually returned to normal, with a mean level of 5.56 mmol/L at the time when the patients were discharged home. Conclusions Patients with insulinomas had a serum fluctuation in glucose (low→high→normal) during the perioperative period.Intraoperative glucose test combined with ultrasound and frozen section helped to localize and to remove all the lesions. Rebound hyperglycemia after surgery should be treated with insulin pump actively to minimize the incidence of complications.

Affecting factors for diagnosis of benign or malignant pancreatic cystic lesions / 中华肝胆外科杂志

Objective To determine the effecting factors for diagnosis of binign or malignant in cystic lesions of pancreas(CLP).Methods One hundred twenty-six patients undergoing operations for CLP or suspected CLP in this hospital from January 1984 to June 2008 were reviewed.Patients were divided into two groups according to lesion's histological features after operation.The predictive effect of various preoperative factors on the malignant potential of CLP was evaluated.Results One hundred twenty-six patients underwent operations for suspected pancreatic cystic neoplasms.There were 89 benign and 37 malignant CLPs.The univariate analysis showed that gender, clinical symptoms(jaundice and weight loss), elevated serum CA199, and presence of one or more of three morphologic features such as solid component, nodule or septation were significantly different between benign and malignant cystic neoplasm of pancreas.The multivariate analysis indicated that imaging features and gender were independent predictors of malignancy.Conclusion In patients with suspected pancreatic cystic neoplasms, elevated serum CA199, clinical symptoms(jaundice and weight loss)and presence of suspicious morphologic features on imaging are predictors of malignant potential of CLP.Patients with a high likelihood of a potentially malignant or malignant lesion based on these three factors should undergo operation without additional investigations.

Diagnosis and treatment of multiple insulinom / 中华普通外科杂志

Objective To summarize the diagnostic and therapeutic experiences for multiple insulinoma. Methods Clinical data of 34 cKsefl of multiple insulinoma treated in Peking Union Medical College Hospital between 1984 and 2007 were analyzed retrospectively. Results Multiple insulinoma was identified in these 34 cases for 37 instances.Malignant insulinoma was found in 2 cases.Three cases suffered from postoperative recurrent multipie tumors.35.3% cases belonged to MEN1;13.5% cases were of insulinoma combined with islet hyperplasia;43.2% cases had 3 or more than 3 insulinomas;Fifteen cases (40.5%)had had a misdiagnosis.45.2%tumors were smaller than 1 cm in diameter:88.9%multiple insulinonla located at the body and tail of the pancreas.Enucleation of multiple tumors was performed for 48.7 percent of cases. Conclusions Most multiple insulinomas were small,it was difficult for preoperative examination to locate all the tumors therefore.Being on the alert against multiple insulinoma and such measures as careful exploration,intraoperative blood glucose determination.fine needle aspiration biopsy,frozen sections helps to avoid missing multiple imuhnoma.

Diagnosis and surgical treatment of nonfunctioning pancreatic neuroendocrine carcinoma / 中国普通外科杂志

0.05). Positive expression of 2 or all 3 markers of neuroendocrine cell was detected in all 16 samples; negative expression of all 5 peptide hormones was observed in only 25% of samples. At a mean follow-up of 44.2 months (range 4 months-7 years ), the mean time from resection to relapse or metastasis was 29.8 months; 7 cases survived more than 5 years, of which 2 have survived for 7 years. Two cases, who subjected non-operative treatment, survired 39,61 months, respectively.Conclusions Clinical manifestations of NPNEC are non-specific. Only a few tumors in NPNEC patients do not yield peptide hormones. Treatment of NPNEC (including patients with liver metastasis) with aggressive surgical resection followed by interventional methods can result in excellent overall long-term survival.