ABSTRACT
Objective:To investigate the clinicopathological features and treatment strategies of intraductal papillary neoplasm of the bile duct (IPNB).Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 40 patients with IPNB who were admitted to Peking Union Medical College Hospital from August 2000 to April 2020 were collected. There were 19 males and 21 females,aged (60±14) years. Patients underwent preoperative imaging examination and blood test for evaluation of tumor location, range and resectability. The treatment strategies of patients depended on preoperative examination and their own willingness. Observation indicators: (1) preoperative examinations and tests; (2) treatment; (3) pathological examination; (4) follow-up. Follow-up using outpatient examination, telephone interview and online diagnosis was performed to detect tumor recurrence and survival of patients up to April 2021. Count data were represented as absolute numbers and percentages. Measurement data with normal distribution were represented as Mean± SD, and measurement data with skewed distribution were represented as M(range). The Kaplan-Meier method was used to calculate the cumulative survival rate and draw survivla curve. Results:(1) Preoperative examinations and tests: 40 patients received preoperative imaging examination and blood test. Of 40 patients, 33 cases underwent abdominal ultrasonography, 31 cases underwent abdominal computed tomography (CT) examina-tion, 21 cases underwent magnetic resonance imaging (MRI), 15 cases underwent endoscopic retrograde cholangiopancreatography (ERCP), 8 cases underwent position emission tomography CT examination, 6 cases underwent endoscopic ultrasonography; some patients underwent multiple examinations. The main imaging features of IPNB were bile duct dilatation, and intraluminal tumor. Enhanced CT scan showed tumor reinforcement. Preoperative blood tests showed of the 40 patients, 21 cases with abnormal liver function, 17 cases with increased bilirubin, 9 cases with increased carcinoembryonic antigen, and 24 cases with increased CA19-9. (2) Treatment: 35 of 40 patients underwent surgery, 5 patients underwent ERCP and biopsy and didn′t undergo surgery based on their willings. Of 35 patients with surgeries,20 cases underwent hemihepatectomy or lobectomy, 8 cases underwent pancreatico-duodenectomy, 7 cases underwent bile duct tumor resection. The operation time was (262±91)minutes, and volume of intraoperative blood loss was 300 mL(range, 50?2 000 mL). Postopera-tive complications occurred in 6 of 35 patients, including 3 cases with Grade Ⅰ complications and 3 cases with Grade Ⅱ complication according to Clavien-Dindo classi-fication system. (3) Pathological examination: 40 patients were diagnosed as IPNB by pathological examinations. There were 19 and 21 patients with extrahepatic and intrahepatic lesions, respectively. There were 20 benign lesions (15 cases of low or intermediate-grade intraepithelial neoplasia and 5 cases of high-grade intraepithelial neoplasia) and 20 malignant lesions of invasive carcinoma. There were 18 cases with mucus secretion and 22 cases without mucus secretion or information. Five of 35 patients with surgeries had positive margin and the rest of 30 patients had negative margin. A total of 154 lymph nodes were dissected in 21 patients, including 3 positive lymph nodes. (4) Follow-up: 35 of 40 patients were followed up for (53±35)months. Seventeen of 35 patients survived without tumor, and 3 patients survived with tumor of which the time to tumor recurrence were 12, 17, 37 months. Fifteen patients died, with the time interval to death of (30±19)months. The 1-, 3-, and 5-year cumulative survival rates of 35 patients were 88.6%, 73.6%, and 50.7%, respectively.Conclusions:IPNB is rare, with the main imaging features as bile duct dilatation, and intraluminal tumor. The tumor is reinforce-ment after enhanced scan. Surgery is the main treatment for IPNB and lymph node metastasis is rare.
ABSTRACT
Objective To analysize the clinicopathologyic features of intraductal papillary neoplasm of the bile duct (IPNB),aiming to increase clinic reorganization of it.Methods Nine patients with IPNB were treated at Department of General Surgery,Beijing Friendship Hospital,Capital Medical University from April 2009 to March 2019,including 5 males and 4 females,aged from 53 to 72 years old.All patients' clinical characteristics,diagnostic methods,treatment and prognosis were retrospectively analyzed.All the patients were followed up from 1 to 119 months (medium 31 months).Results The pathologic diagnosis of all the 9 cases were obtained.Seven cases had occured recurrent abdominal painand fever and 2 cases presented painless jaundice.Five patients had single lesion (4 cases at the distal of common bile duct,1 cases at the hilum),whereas 4 cases had multiple lesions (2 cases diffuse lesionsin the biliary tree,2 cases multicentriclesionsin the common bile duct).Eight patients were underwent surgical procedure,including 7 cases of pancreticoduodenectomy and 1 cases radical resection of hilar cholangiocarcinoma.One patient with diffuse lesions was treated by chemotherapy after pathologic diagnosis was confirmed.One was died at 88 months after surgery,others were survival.Conclusions IPNB is a rear disease.The multicentric lesions have more chances to progress to invasive lesions.Different operative approaches should be chosen for different tumor sites and extents.
ABSTRACT
Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. The asymptomatic patient was treated with liver segmentectomy.