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BACKGROUND Mirizzi syndrome (MS) is defined as an extrinsic compression of the extrahepatic biliary system by an impacted stone in the gallbladder or the cystic duct leading to obstructive jaundice. Endoscopic retrograde cholangiopancreatography (ERCP) could serve diagnostic and therapeutic purposes in patients with MS in addition to revealing the relationships between the cystic duct, the gallbladder, and the common bile duct (CBD). Cholecystectomy is a challenging procedure for a laparoscopic surgeon in patients with MS, and the presence of a cholecystocholedochal fistula renders preoperative diagnosis important during ERCP. AIM To evaluate cholecystocholedochal fistulas in patients with MS during ERCP before cholecystectomy. METHODS From 2004 to 2018, all patients diagnosed with MS during ERCP were enrolled in this study. Patients with associated malignancy or those who had already undergone cholecystectomy before ERCP were excluded. In total, 117 patients with MS diagnosed by ERCP were enrolled in this study. Among them, 21 patients with MS had cholecystocholedochal fistulas. MS was further confirmed during cholecystectomy to check if cholecystocholedochal fistulas were present. The clinical data, cholangiography, and endoscopic findings during ERCP were recorded and analyzed. RESULTS Gallbladder opacification on cholangiography is more frequent in patients with MS complicated by cholecystocholedochal fistulas (P < 0.001). Pus in the CBD and stricture length of the CBD longer than 2 cm were two additional independent factors associated with MS, as demonstrated by multivariate analysis (odds ratio 5.82, P = 0.002; 0.12, P = 0.008, respectively). CONCLUSION Gall bladder opacification is commonly seen in patients with MS with cholecystocholedochal fistulas during pre-operative ERCP. Additional findings such as pus in the CBD and stricture length of the CBD longer than 2 cm may aid the diagnosis of MS with cholecystocholedochal fistulas.

Gastrectomy is commonly performed for both benign and malignant lesions. Although the incidence of post-gastrectomy acute pancreatitis (PGAP) is low compared to other well-recognized post-operative complications, it has been reported to be associated with a high mortality rate. In this article, we describe a 70-year-old man with asymptomatic pancreatic divisum who underwent palliative subtotal gastrectomy for an advanced gastric cancer with liver metastasis. His post-operative course was complicated by acute pancreatitis and intra-abdominal sepsis. The patient eventually succumbed to multiple organ failure despite surgical debridement and drainage, together with aggressive antibiotic therapy and nutritional support. For patients with pancreas divisum or dominant duct of Santorini who fail to follow the normal post-operative course after gastrectomy, clinicians should be alert to the possibility of PGAP as one of the potential diagnoses. Early detection and aggressive treatment of PGAP might improve the prognosis.

AIM: To determine the timing of mortality after onset of severe acute pancreatitis (SAP) and the course of the disease in a large series of patients. METHODS: From July 1996 to June 2005, all patients diagnosed with acute pancreatitis at Chang Gung Memorial Hospital, Taipei, Taiwan were retrospectively studied. Three thousand two hundred and fifty episodes of acute pancreatitis were recorded in 2248 patients (1431 males and 817 females; median age, 55.6 years; range, 18-97 years). Mortality was divided into two groups: early death (≤ 14 d after admission), and late death (> 14 d after admission). The clinical features of patients in these two groups were compared. RESULTS: Although the overall mortality rate of acute pancreatitis was 3.8% (123/3250), mortality rate of SAP was as high as 16.3% (105/643). Of those 105 SAP mortalities, 44 (41.9%) deaths occurred within the first 14 d after admission and 61 (58.1%) occurred after14 d. Incidence of early death did not significantly differ from that of late death. The co-morbidities did not contribute to the timing of death. Early deaths mainly resulted from multiple organ failure. Late deaths were mainly caused by secondary complication of infected necrosis. Intra-abdominal bleeding significantly caused higher mortality in late death. CONCLUSION: Approximately half (42%) of SAP deaths occur within 14 d and most were due to multiple organ failure. The late deaths of SAP were mostly due to infected necrosis.

Primary squamous cell carcinoma (SCC) of the liver is rare. Totally nine such cases have been reported in the literature. Primary SCC of the liver has been reported to be associated with hepatic teratoma, hepatic cyst, or hepatolithiasis. Complete remission of poorly differentiated SCC of the liver could be achieved by systemic chemotherapy followed by surgery or remarkably respond to hepatic arterial injection of low dose chemotherapeutic drugs. Here we report the first case of primary SCC of the liver presenting as a solid tumor and receiving successful hepatic resection with 9-mo disease free survival.

We described two members in a family with gastroin-testinal stromal tumors (GISTs) without cutaneous hyperpigmentation. The patients were father and son who did not have cutaneous hyperpigmentation. Histological examination showed that these tumors were GISTs expressing CD34 and CD117. Tumor DNA extracted from paraffin-embedded specimens revealed somatic mutation with a deletion mutation at different codons in exon 11 of c-kit gene after direct sequencing analysis. No germline mutation was detected in DNA extracted from peripheral leukocytes obtained from the father and son. We propose that GISTs could be caused by sporadic somatic mutation in a family without germline mutation and hyperpigmentation.

AIM: To report preliminary results of the efficacy and safety of sunitinib in the management of Taiwanese gastrointestinal stromal tumors (GIST) patients facing imatinib mesylate (IM) intolerance or failure. METHODS: Between 2001 and May 2010, 199 Taiwanese patients with metastatic GIST were treated at Chang Gung Memorial Hospital. Among them, 23 (11.6%) patients receiving sunitinib were investigated. RESULTS: Sixteen male and 7 female patients with a median age of 59 years (range: 24-83 years) received sunitinib. Twenty-two GIST patients changed to sunitinib because of IM failure and 1 because of intolerance. The median duration of sunitinib administration was 6.0 mo (range: 2-29 mo). The clinical benefit was 65.2% [2 complete response (CR), 4 partial response (PR), and 9 stationary disease (SD); 15/23]. In 12 patients harboring mutations of the kit gene at exon 11, the clinical benefit rate (CR, PR, and SD) was 75.0% and 6 patients with tumors containing kit exon 9 mutations had a clinical benefit of 50.0% (not significant, P = 0.344). The progression free survival (PFS) and overall survival (OS) did not differ between patients whose GISTs had wild type, KIT exon 9, or KIT exon 11 mutations. Hand-foot syndrome was the most common cause of grade III adverse effect (26.1%), followed by anemia (17.4%), and neutropenia (13.0%). During the median 7.5-mo follow-up after sunitinib use, the median PFS and OS of these 23 GIST patients after sunitinib treatment were 8.4 and 14.1 mo, respectively. CONCLUSION: Sunitinib appears to be an effective treatment for Taiwanese with IM-resistant/intolerant GISTs and induced a sustained clinical benefit in more than 50% of Taiwanese advanced GIST patients.

AIM: To establish the prognosis and feasibility of en-bloc vascular resection of stage II pancreatic adenocarcinoma of the head and uncinate process. METHODS: We retrospectively analyzed 87 patients with stage II pancreatic adenocarcinoma, who were subjected to pancreaticoduodenectomy (PD) and pylorus-preserving PD (PPPD) between 1996 and 2006 in Chang Gung Memorial Hospital, Taiwan. Twelve and 75 patients underwent PD/PPPD with and without resection of portal vein/superior mesenteric vein (PV/SMV), respectively. RESULTS: The overall 1- and 3-year survival rates of patients undergoing PD/PPPD with and without vascular resection were 50.0% and 16.7%, and 44.4% and 12.2%, respectively. Morbidity and mortality rates in the PV/SMV resection vs non-resection group were 50.0% and 0.0%, and 40.0% and 2.7%, respectively. In multivariate analysis, serum bilirubin, histological differentiation and adjuvant chemotherapy were independent prognostic factors that influenced survival. CONCLUSION: In stage II adenocarcinoma of the pancreatic head and uncinate process, serum bilirubin, histological differentiation and adjuvant chemotherapy were independent prognostic factors, and en-bloc vascular resection is a feasible option in carefully selected patients.

Cystic neoplasms of the pancreas are relatively rare, comprising 10 percent of pancreatic cysts and only 1 percent of pancreatic cancers. Cystic neoplasms include mucinous cystic neoplasms, serous cystadenomas, papillary cystic tumors, cystic islet cell tumors and intraductal papillary mucinous neoplasms of the pancreas (IPMNs). IPMN was first described in 1982. It has been most commonly described in 60 to 70 years old males, and represents a relatively “new” but increasingly recognized disease. The improvement and widespread use of modern imaging equipments and heightened awareness of physicians contribute to the increasing incidence of IPMN. The majority of IPMNs are located in the pancreatic head (75%) while the rest involves the body/tail regions. Multifocal IPMNs have been hypothesized, but the true presence of multifocality is unknown. Here we present a 72-year-old male diagnosed with IPMN (carcinoma in situ) in the pancreatic head and a branch duct type IPMN (duct atypia) in the pancreatic body and tail. The patient underwent a Whipple intervention and a distal pancreatectomy. A three-year disease-free survival has been observed so far.

AIM: To study the behavior as well as optimal treatment of gallbladder sarcomatoid carcinoma, we reviewed the results of treatment of gallbladder sarcomatoid carcinoma from Chang Gung Memorial Hospital. METHODS: From 1987 to 2005, six patients were diagnosed with gallbladder sarcomatoid carcinoma and treated at our institution. Tumor staging was based on 2002 revised tumor-node-metastasis (TNM) staging for gall bladder cancer from the American Joint Committee on Cancer. The clinical presentation, laboratory data and preoperative workup were reviewed retrospectively. RESULTS: Five patients were female and one was male. The age ranged from 51 to 66 years (median, 58 years). Surgical procedures included three curative resections, two palliative resections and one biopsy. There were two surgical complications (33.3%) and one case of surgical mortality (16.7%). The follow-up time ranged from 30 d to 5 mo. The median survival was 2.5 mo. The prognosis was extremely poor, even after curative resection and postoperative chemotherapy. CONCLUSION: The prognosis of gallbladder sarcomatoid carcinoma was not dependent on TNM stage and was always dismal. The clinicopathological features were different from those of gall bladder cancer.

We reported a 55-year-old man who suffered from chest pain and dyspnea on exertion for two weeks associated with night sweating, general malaise, poor appetite, and body weight loss. Physical examination revealed friction rub with distant heart sound, bilateral clear breathing sound, no abdomen tenderness, and normal bowel sound. Subsequent chest X-ray revealed cardiomegaly and cardiac echo showed massive pericardial and pleural effusion with normal left ventricular function. Constrictive pericarditis was diagnosed based on clinical information. Tuberculosis (TB), malignancy, autoimmune disease, infection, hypothyroidism, and idiopathic could be the causes but excluded by further study. High-resolution lung CT scan after reconstruction revealed a moderate amount pericardial effusion with possible superimposed infection. Thickness of pericardium and left lobe liver abscess were found. A straight tubular structure about 6 cm in length transverses the lateral segment of liver to pericardial space and unknown foreign body was suspected. Laparotomy was performed, 6.5 cm toothpick was found through the liver into pericardium. Post-operative course was uneventful and he discharged one week later. The patient could not remember swallowing the toothpick before. He had no chest pain and dyspnea on exertion during a 6-mo follow-up period.

To examine the impact of imatinib mesylate (Glivec) on patient survival and response and its safety, and the correlation of the response rate with the kit gene mutation status.Thirty-three of 74 (44.6%) small bowel gastrointestinal stromal tumor (GIST) patients who developed recurrence after curative resection and not treated with Glivec were classified as group A patients. Twenty-two advanced small bowel GIST patients treated with Glivec were classified as group B patients. Clinicopathological features, post-recurrence and overall survival rates were compared. Each tumor in group B patients was investigated for mutations of kit or platelet-derived growth factor alpha (PDGFRA). The mutation type was correlated with clinical outcomes. The anti-tumor effect and safety of Glivec in group B patients were also assessed.Advanced small bowel GIST patients treated with Glivec had substatntially longer post-recurrence survival and higher overall survival rates than those not treated with Glivec. A total of 15 patients had a partial response (PR) (67.8%). Activated mutations of c-kit were found in 16 of 19 tested patients and no PDGFRA mutant was identified. In 13 patients with GISTs harboring exon 11 kit mutations, the partial response rate (PR) was 69.3%, whereas two of three patients with tumors containing an exon 9 kit mutation had an overall response rate (ORR) of 66.7% (not significant).Glivec significantly prolongs the post-recurrence and overall survival of Asian patients with advanced GISTs. Glivec induces a sustained objective response in more than half of Asian patients with advanced small bowel GISTs. Activated mutations of kit exon 11 are detectable in the vast majority of GISTs. There is no difference in the PR rate for patients whose GISTs have kit exon 9 and exon 11 mutations.

AIM: To analyze the prognostic factors influencing the overall survival of peripheral cholangiocarcinoma (PCC) patients undergoing surgical treatment during 25 years at a single institution. METHODS: This study retrospectively reviewed prospectively collecting data about 373 patients with histologically proven PCC who underwent surgical treatment between 1977 and 2001. RESULTS: Three hundred and seventy-three PCC patients (159 men and 214 women) underwent surgical treatment from 1977 to 2001. Among them, 187 PCC patients underwent hepatectomy and 135 had curative resection (curative resectability rate: 36.2%). The follow-up duration ranged from 1.05 to 167.6 mo (mean/median = 14.1/7.2 mo). Overall cumulative survival rates at 1, 3, and 5 years were 32.5%, 9.2%, and 4.1%, respectively. Univariate log-rank analysis identified the following as adverse influences on overall survival: presence of symptoms, absence of mucobilia, elevated CEA and CA 19-9 levels, non-papillary tumor type, receiving non-hepatectomy, advanced tumor staging, lack of post-operative chemotherapy, and radiotherapy. Meanwhile, multivariate Cox’s proportional hazard analysis demonstrated that absence of mucobilia, non-papillary tumor type, advanced tumor staging, non-hepatectomy, and lack of post-operative chemotherapy were the five independent prognostic factors that adversely affected overall survival. CONCLUSION: Favorable overall survival of PCC patients undergoing surgical treatment depends on early tumor stage, presence of mucobilia, papillary tumor type, hepatic resection, and post-operative chemotherapy.

Primary splenic angiosarcoma is a very rare, aggressive neoplasm with a high metastatic rate and dismal prognosis. This neoplasm usually presents with abdominal pain, splenomegaly, anemia, and thrombocytopenia. Splenic angiosarcoma with bleeding gastrointestinal metastases is extremely rare. The literature contains only two case reports. This study reported a 44-year-old male patient with splenic angiosarcoma with sustained repeated gastrointestinal bleeding due to small bowel metastases. Salvage surgery was performed by splenectomy and resection of the metastatic small bowel tumors. The post-operative course was uneventful; the patient survived with the disease and had no GI bleeding, 7 mo after surgery.