[A Case of Intraductal Papillary Mucinous Adenocarcinoma with Hepatic Dysfunction Due to Tumor Perforation into the Bile Duct].

A 58-year-old woman presented with a complaint of weight loss. Abdominal computed tomography showed dilatation of the biliary and pancreatic ducts and a mural nodule in the pancreatic duct. The diagnosis was intraductal papillary mucinous neoplasm(IPMN). Endoscopic retrograde cholangiopancreatography(ERCP)and cholangioscopy revealed a fistula between the common bile duct and the IPMN. A sudden increase in hepatobiliary enzymes was noted preoperatively. ERCP showed that the common bile duct was obstructed by mucus. A nasobiliary drainage tube was inserted into the bile duct endoscopically and kept open by daily tube washing, and the liver dysfunction improved. Total pancreatectomy, splenectomy, and regional lymph node dissection were performed. Histological examination confirmed that the primary tumor was mixed invasive intraductal papillary mucinous adenocarcinoma. The patient remains alive and well with no evidence of recurrence 18 months after resection.

[Pseudomyxoma Peritonei Caused by Intraductal Papillary Mucinous Neoplasm-A Three Case Series].

Pseudomyxoma peritonei(PMP)is a rare condition caused by intraductal papillary mucinous neoplasm(IPMN). At our institution, 3 cases, along with literature review, of PMP caused by IPMN were treated with cytoreductive surgery. In case 1, a 70-year-old man was pathologically diagnosed with low-grade PMP with intraductal papillary mucinous carcinoma. Recurrence was discovered 68 months after surgery, and the patient died after 78 months. In case 2, a 69-year-old man was pathologically diagnosed with high-grade PMP with signet-ring cell carcinoma caused by intraductal papillary mucinous carcinoma. The patient died 8 months post-surgery. In case 3, a 77-year-old woman was pathologically diagnosed with low- grade(partially high-grade)PMP with intraductal papillary mucinous adenoma. There was a recurrence 14 months post- surgery. The patient is still alive because of systemic chemotherapy 32 months after surgery.

[A Case of Resection for Bleeding from a Gallbladder Tumor].

A 77-year-old woman was taking anticoagulant medication for pulmonary hypertension. Black stools were noted in August 2018. Close abdominal ultrasonography revealed a broad-based tumor at the base of the gallbladder. Lower endoscopy showed no obvious digestive tract hemorrhage but hemorrhage from a gallbladder tumor was suspected. The resected procedure was suspected for gallbladder cancer(cT2N0M0, cStageⅡ), but dizziness 5 days before operation. The patient complained of severe anemia, and underwent laparoscopic cholecystectomy for hemorrhage control. In the macroscopic examination of the specimens, tumorous lesions and blood clots were found adhered to the bottom of the gallbladder. The histopathological diagnosis was papillary adenocarcinoma.

Hemobilia due to intracholecystic papillary neoplasm.

We report the case of a 39-year-old patient who presented an episode of upper gastrointestinal bleeding due to hemobilia. The imaging tests showed the gallbladder occupied by solid tissue, with a diagnosis of intracholecystic papillary neoplasm after the cholecystectomy. The intracholecystic papillary neoplasm of the gallbladder is a newly established entity and it is considered a subtype of intraductal papillary neoplasm of the bile duct. Its presentation in the form of hemobilia has barely been described in the literature.

Intracholecystic papillary-tubular neoplasm in a patient with choledochal cyst: a link between choledochal cyst and gallbladder cancer?

BACKGROUND: Intracholecystic papillary-tubular neoplasms are rare precursor lesions of gallbladder cancer. They were proposed as a separate pathologic entity in 2012 by Adsay et al. for the unification of a variety of mass-forming precursor lesions including papillary adenomas, tubulopapillary adenomas, intestinal adenomas, and others. They are considered homologous to intrapapillary mucinous neoplasms of the pancreas and intrabiliary papillary neoplasms of the common bile duct. In contrast with the commoner flat-type precursor gallbladder cancer lesions, they follow a more indolent clinical course and probably different genetic pathways to carcinogenesis. They are largely uninvestigated with only a handful of studies providing biological and clinical information. Choledochal cysts are dilation of the common bile duct. Diagnosis is usually established during childhood, and only a minority of patients are diagnosed at adulthood. They are of major clinical importance as they are known predisposing factors for biliary carcinogenesis. CASE PRESENTATION: The current report describes a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. The patient underwent excision of the extrahepatic biliary tree for a Todani I choledochal cyst, and histological examination of the specimen revealed an intracholecystic papillary-tubular neoplasm of the gallbladder. Authors describe diagnostic and clinical course of the patient alongside clinical and biological characteristics of these rare lesions. CONCLUSIONS: To the best of our knowledge, this is the first report of a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. Those rare lesions shed light on different forms of gallbladder cancer carcinogenesis and its relationship with choledochal cysts and cholestasis.

[About 2 cases of ectopic sebaceous glands in esophagus: Endoscopic and histological correlations from biopsies and resected specimens]. / À propos de 2 cas de glandes sébacées ectopiques œsophagiennes : corrélations endoscopiques, et anatomopathologiques sur biopsies et pièces opératoires.

Sebaceous glands are cutaneous annexes located in the dermis. Focal spots of ectopy of these glands are frequently identified in ectodermal tissues: they represent Fordyce's disease. However, only a few cases of ectopic sebaceous glands have been mentioned in non-ectodermic tissue. Fordyce spots of esophageal location are unusual, and most of them have been diagnosed from biopsy specimens. We report two cases of ectopic sebaceous glands in esophagus, the first diagnosed from a resected specimen, the second from biopsies. A literature review is carried out.

[A Radically-Resected Case of Ruptured IPMC of the Pancreas after Abscess Drainage Using an Endoscopic Naso-Pancreatic Drainage Tube].

A 79-year-old man was referred to our hospital because of sudden upper abdominal pain. Enhanced computed tomography revealed a huge multilocular cystic tumor with a thickened wall, which was connected with the main pancreatic duct of the pancreatic body. Abscess formation was seen inside the omental bursa; however, there were no signs of direct invasion of the cystic tumor into the stomach or transverse colon. Therefore, we performed emergency endoscopic naso-pancreatic drainage(ENPD)under the diagnosis of an intraperitoneal abscess caused by rupture of intraductal papillary mucinous carcinoma(IPMC). Four weeks later, distal pancreatectomy with omentectomy was performed to achieve curative resection of the ruptured IPMC. The postoperative course was uneventful and the patient was discharged on postoperative day 14. The pathological diagnosis was noninvasive IPMC. No signs of recurrence were seen until 12 months after surgery. Rupture of IPMC into the intraperitoneal space is rare; however, the prognosis is relatively poor because of the difficulty of curative resection. ENPD drainage before surgery is potentially useful for patients with ruptured IPMC to control local inflammation, which improves surgical curability.

Autoimmune pancreatitis type-1 associated with intraduct papillary mucinous neoplasm: report of two cases.

Chronic pancreatitis lesions usually embrace both intraduct papillary mucinous neoplasm (IPMN) and pancreatic ductal adenocarcinoma (PDAC). Patients at genetically-determined high risk of PDAC often harbor IPMN and/or chronic pancreatitis, suggesting IPMN, chronic pancreatitis and PDAC may share pathogenetic mechanisms. Chronic autoimmune pancreatitis (AIP) may also herald PDAC. Concurrent IPMN and AIP have been reported in few patients. Here we describe two patients with IPMN who developed type-1 AIP fulfilling the Honolulu and Boston diagnostic criteria. AIP diffusively affected the whole pancreas, as well as peripancreatic lymph nodes and the gallbladder. Previous pancreatic resection of focal IPMN did not show features of AIP. One of the patients carried a CFTR class-I mutation. Of notice, serum IgG4 levels gradually decreased to normal values after IPMN excision. Common risk factors to IPMN and AIP may facilitate its coincidental generation.

Diabetes mellitus and ovarian cancer: more complex than just increasing risk.

OBJECTIVE: Diabetes mellitus (DM) is a risk factor for endometrial cancer and is associated with poorer outcomes in breast and colon cancers. This association is less clear in epithelial ovarian cancer (EOC). We sought to examine the effect of DM on progression-free (PFS) and overall survival (OS) in women with EOC. METHODS: A retrospective cohort study of EOC patients diagnosed between 2004 and 2009 at a single institution was performed. Demographic, pathologic and DM diagnosis data were abstracted. Pearson chi-square test and t test were used to compare variables. The Kaplan-Meier method and the log rank test were used to compare PFS and OS between non-diabetic (ND) and DM patients. RESULTS: 62 (17%) of 367 patients had a diagnosis of DM. No differences in age, histology, debulking status, or administration of intraperitoneal chemotherapy between ND and DM patients were present, although there were more stage I and IV patients in the ND group (p=0.04). BMI was significantly different between the two groups (ND vs. DM, 27.5 vs. 30.7kg/m(2), p<0.001). While there were no differences in survival based on BMI, diabetic patients had a poorer PFS (10.3 vs. 16.3months, p=0.024) and OS (26.1 vs. 42.2months, p=0.005) compared to ND patients. Metformin use among diabetic patients did not appear to affect PFS or OS. CONCLUSIONS: EOC patients with DM have poorer survival than patients without diabetes; this association is independent of obesity. Metformin use did not affect outcomes. The pathophysiology of this observation requires more inquiry.

Computed tomography and magnetic resonance imaging findings of malignant solid pseudopapillary tumors of the pancreas with macroscopic venous tumor thrombosis: a report of 4 cases.

PURPOSE: The purpose of this study was to report the imaging findings of malignant pancreatic solid pseudopapillary tumors (SPTs) with macroscopic venous tumor thrombi. METHODS: The clinical features and imaging findings of 4 cases of malignant pancreatic SPT with venous tumor thrombi were retrospectively reviewed. RESULTS: The tumor thrombi were located in the splenic vein (n = 3) or the main portal vein and the proximal splenic vein (n = 1). Venous thrombi were connected with the main pancreatic tumors and showed venous filling defects on computed tomography and magnetic resonance imaging. Tumor thrombi primarily consisting of necrotic component and/or hemorrhage displayed no enhancement after contrast injection (n = 3). The enhancement pattern of the tumor thrombi that consisted mainly of tumor nests was consistent with pancreatic SPT (n = 1), that is, a slight enhancement in the arterial phase and a progressive enhancement in the portal venous phase and the equilibrium phase. Venous tumor thrombi associated with hemorrhage were hyperintense on both T1-weighted and T2-weighted images. CONCLUSIONS: It is uncommon for pancreatic SPTs to spread by invading the venous system and forming macroscopic venous tumor thrombi.

[A case of small bowel cancer with positive peritoneal cytology and five-year recurrence-free survival].

Small bowel cancer is frequently detected at an advanced stage and its prognosis is poor. We report on a patient with small bowel cancer with positive peritoneal cytology who survived for 5 years without recurrence after surgery.The case involved a 73-year-old woman who had undergone partial resection of the small intestine and lymphadenectomy for a small bowel tumor with obstruction. Pathological examination confirmed papillary adenocarcinoma with partial serosal invasion. Ascites cytology indicated a class V tumor. Adjuvant chemotherapy with TS-1 was administered for 20 months, and the patient has survived without evidence of disease for over 5 years.In this case, it is possible that TS-1 chemotherapy was effective for prevention against small bowel cancer recurrence.Furthermore , peritoneal cytology in patients with small bowel cancer should be evaluated as a predictor of prognosis.

The clinicopathological spectrum and management of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B).

BACKGROUND: Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is a rare but increasingly diagnosed clinical entity. Typical cholangioscopic findings usually include intraductal protruding papillary tumors that secrete mucus. METHODS: Clinical, radiological and histopathological data of seven consecutive patients who were found to have IPMN-B were analyzed. RESULTS: Six of the seven patients presented with obstructive jaundice/cholangitis as the presenting complaint. ERCP and other imaging were equivocal in five of these patients and peroral cholangioscopy (POCS, single-operator cholangioscopy system) was performed. This revealed mucin-producing intraductal tumors with numerous frond-like papillary projections; a macroscopic appearance consistent with IPMN-B. Preoperative biopsy revealed adenoma, with low-grade dysplasia in two patients and high-grade dysplasia in three. Three patients underwent Whipple resection; one underwent total pancreatectomy with left hepatectomy, one patient a pancreas preserving duodenectomy with common bile duct reimplantation and one patient an extended right hepatectomy. These patients were found to have IPMN-B with adenomatous changes with varying grades of dysplasia and even cholangiocarcinoma on final histopathology. One patient first underwent endoscopic papillectomy and on follow-up was found to have cholangiocarcinoma with metastases to the liver. CONCLUSION: POCS can be a key diagnostic investigation in the evaluation of patients with papillary tumors of the bile duct. IPMN-B has a heterogenous pathology and varying grades of dysplasia and even carcinoma may exist in the same patient. Surgical management should be radical and based on tumor extent.

Pulmonary metastases of a papillary adeno-carcinoma of ovary in a pregnant lady.

A 28 years young lady admitted to National Institute of Diseases of Chest and Hospital (NIDCH), Dhaka with the complaints of respiratory distress and cough for 2 months. She was in her last trimester of first pregnancy when she becomes dyspneic on exertion and at night. Gradually it increases in time and she become unable to walk when she consulted with specialists in Barisal. She was examined clinically and radiographically and found some opacity on both lung fields. She was on several antibiotics, but got no improvement. In the meantime, an emergency caesarean section was done to have a healthy baby and patient came to NIDCH for this unexplained breathlessness and cough. She was severely dyspneic, tachypnoic, cyanosed. There were bilateral coarse crepitations, bilateral opacities predominantly on both lower and middle zones of both lungs obscuring costo-phrenic angles on chest X-rays. CT scan of chest shows bilateral reticulo-nodular shadows with a homogenous opacity on right lower lung which enhances after contrast scanning. A CT guided FNAC was done from that opacity which revealed a papillary adeno-carcinoma with psammoma bodies on cytopathological study. Thereafter, to explore the primary site, thyroid gland, abdominal organs was assessed adequately and only positive finding was raised CA-125 (706 IU/ml) which was consistent with ovarian cancer. In this way, a primary ovarian carcinoma in a pregnant young lady with normal sized ovary that metastasizes to lung causing bilateral pleural effusion and lymphangitis carcinomatosa was explored. This was an unusual presentation of ovarian papillary adeno-carcinoma with cough and breathlessness at the last trimester of pregnancy in absence of any abdominal mass.

[Lung cancer accompanied by active pulmonary tuberculosis].

We report 2 patients with lung cancer accompanied by active pulmonary tuberculosis. Case1 was a 82-year-old woman with stage I A bronchioloalveolar carcinoma and tuberculosis in right upper lobe. Right upper lobectomy was performed after the histological diagnosis of lung cancer by intraoperative frozen section. Case2 was a 69-year-old man with papillary adenocarcinoma in right lower lobe and tuberculosis in bilateral upper lobe. Partial resection in right lower lobe was performed for diagnosis of lung cancer. Smear-positive tuberculosis was diagnosed by sputum examination after the operation. Post-operative anti-tuberculosis chemotherapy was added in both patients.