A blastoma is a type of cancer, which is common in children; it is caused by malignancies derived from in the precursor cells, often called blasts. Examples are nephroblastomas, retinoblastomas, pleuropulmonary blastomas, and pancreatoblastomas. Pancreatoblastomas are extremely rarely in adults. It is difficult preoperatively to distinguish this tumor from other pancreatic tumors including solid and papillary epithelial neoplasm of the pancreas (SPEN), acinar cell carcinoma, islet cell tumor, and ductal adenocarcinoma with cystic degeneration. To our knowledge, this case may be the second report of a pancreatoblastoma occurring in an adult in Korea. We report a case of a pancreatoblastoma that was confirmed by pathology, despite the radiologic finding that assumed it was a SPEN.
Adenocarcinoma ; Adenoma, Islet Cell ; Adult* ; Carcinoma, Acinar Cell ; Child ; Humans ; Korea ; Neoplasms, Glandular and Epithelial ; Pancreas ; Pathology ; Retinoblastoma ; Wilms Tumor

OBJECTIVETo investigate the diagnosis and treatment of pancreatic islet cell tumors.

METHODSFifty-one patients with islet cell tumors treated in our department from January 1991 to April 2011 were included in this study. The data of clinical features, diagnosis and treatment were retrospectively analyzed.

RESULTSAmong the 51 cases, 38 cases showed typical Whipple's triad, and the other 13 cases were non-functional islet cell tumors. In these 13 cases, 5 patients had no specific clinical symptoms, and 8 patients had abdominal distending pain. The positive rates of imaging were: B-ultrasound 43.1%, multi-slice spiral CT 69.8%; MRI 62.5%, endoscopic ultrasonography (EUS) 64.7% (11/17), and intraoperative ultrasound (IOUS) 96.3%, the differences among them were statistically significant (P<0.05). All patients underwent surgical treatment. Postoperative pancreatic leakage happened in 6 cases. Finally all the patients recovered after effective external drainage, anti-infection treatment and nutritional support.

CONCLUSIONSIntraoperative ultrasonography (IOUS) has a higher accuracy in the diagnosis of pancreatic islet cell tumors, compared with preoperative B-ultrasonography, CT, MRI, and endoscopic ultrasound (EUS). The most effective treatment of this disease is surgery.

Adenoma, Islet Cell ; diagnosis ; diagnostic imaging ; surgery ; Adult ; Anastomotic Leak ; etiology ; Endosonography ; Female ; Humans ; Insulinoma ; diagnosis ; diagnostic imaging ; surgery ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multidetector Computed Tomography ; Pancreatectomy ; adverse effects ; Pancreatic Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Retrospective Studies

No abstract available.
Adenoma, Islet Cell* ; Adult* ; Diabetes Mellitus, Type 2* ; Humans ; Islets of Langerhans* ; Nesidioblastosis*

PURPOSE: Insulinoma is a rare disease for which early diagnosis followed by proper surgical management provides a chance for cure. Analyses of clinicopathological features of patients can help optimize the surgical approach in the treatment of insulinoma. METHODS: The records of 13 patients (seven male, six female mean age 44.3 years; age range 17~62 years) who were diagnosed clinically and pathologically with insulinoma and who underwent surgery between March 1997 and April 2007 at the Department of Surgery, Seoul National University Hospital. Hospital in English please were retrospectively examined. RESULTS: All patients had findings compatible with Whipple's triad. Mean fasting blood sugar was 40.5 mg/dl, serum insulin level was 33.5µU/ml, and insulin-to-glucose ratio was 0.6. A prolonged starvation test was performed on six patients. Tumors were localized in 10 patients with a computed tomography (CT) scan and in three patients with CT angiography. Five tumors were located in the pancreas head and uncinate process, five in the body, and four at the body-tail border and tail. Patients underwent resection of tumorby enucleation, distal pancreatectomy, pylorus-preserving pancreaticoduodenectomy, laparoscopic distal pancreatectomy, and duodenum-preserving resection of pancreas head. Four immediate postoperative complications (fluid collection, pancreatic fistula, delayed gastric emptying) occurred. No symptoms or recurrences were apparent during the median 15 month follow-up. CONCLUSION: Insulinoma is difficult to diagnose correctly without a prolonged duration of symptoms. Localization of insulinoma can be aided by a CT scan and/or CT angiography. Less aggressive operative procedures such as simple enucleation might be a sufficient and feasible procedure for curative resection of benign insulinomas.
Adenoma, Islet Cell ; Angiography ; Blood Glucose ; Early Diagnosis ; Fasting ; Female ; Follow-Up Studies ; Head ; Humans ; Insulin ; Insulinoma* ; Male ; Pancreas ; Pancreatectomy ; Pancreatic Fistula ; Pancreaticoduodenectomy ; Postoperative Complications ; Rare Diseases ; Recurrence ; Retrospective Studies ; Seoul ; Starvation ; Surgical Procedures, Operative ; Tail ; Tomography, X-Ray Computed

We report the case of a 16-year-old boy with a solid pancreatic mass which proved to be a nonfunctioning, malignant pancreatic neuroendocrine tumor (PNET). In pediatric patients, malignant pancreatic tumors are rare, especially malignant PNET. When dynamic contrast enhanced MRI showed a well enhancing solid pancreatic tumor on arterial and delayed phases and combined with malignant features, such as vascular invasion, invasion of adjascent organs, and lymphadenopathy, we should include malignant pancreatic neuroendocrine tumor in the differential diagnosis of childhood pancreatic tumors.
Adenoma, Islet Cell ; Adolescent ; Child ; Diagnosis, Differential ; Humans ; Lymphatic Diseases ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors

OBJECTIVETo evaluate the methods of diagnosis and surgical treatment for nonfunctional islet cell tumor (NICT).

METHODSForty-four patients with non-functional islet cell tumor treated at the First Affiliated Hospital of Nanjing Medical University during January 1968 to June 2008 were analyzed retrospectively. There were 9 males and 35 females, aged from 7- to 70-years-old. Clinical manifestation: 15 cases (34.1%) of abdominal masses, 17 patients (38.6%) with epigastric or back pain, 5 cases of jaundice, 5 cases (11.4%) for upper abdominal fullness or vomiting, 10 cases (22.7%) of pancreatic tumor noticed by routine health checkups or imaging examinations. Imaging examination: CT scan, sonography, ERCP, MRI, upper GI series were performed in 33 (75.0%), 16 (36.4%), 6 (13.6%), 2 (4.5%), and 10 cases (22.7%) respectively. Operation methods: 39 patients (88.6%) underwent surgical resection and the other 5 patients did not.

RESULTS

COMPLICATIONSpancreatic fistula in 7 patients (15.9%), intra-abdominal bleeding in 4 (9.1%), gastrojejunal anastomosis outlet obstruction in 1 (2.3%), biliary fistula in 2 (4.5%) and incisional infection in 3 (6.8%). Surgery related mortality happened in 2 patients (4.5%), both treated before 1999. Twenty-five patients underwent operation between January 1999 and June 2008 were followed up for 6 to 108 months. All survive except one died 75 months after the surgery for unknown reason.

CONCLUSIONSNo specific clinical manifestation is recognized for non-functional islet cell tumor. Spiral CT is an optimal diagnostic method, while surgery is the first choice for treatment. Middle segmental pancreatectomy has become an alternative surgical protocol for NICT.

Adenoma, Islet Cell ; diagnosis ; surgery ; Adolescent ; Adult ; Aged ; Child ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Pancreatectomy ; methods ; Pancreatic Neoplasms ; diagnosis ; surgery ; Prognosis ; Retrospective Studies ; Young Adult

BACKGROUND/AIMS: Since pancreatic endocrine tumors (PET) are rare and heterogeneous diseases, their survival and prognosis are not well known. Due to recent advances in CT/MRI technology, incidentalomas of the pancreas are detected with increasing frequency. This study presents results of clinical manifestations of PET and predictive factors associated with survival. METHODS: From year 1990 through 2006, medical records of 98 patients (56 men, 42 women) who were diagnosed as PET pathologically at Seoul National University Hospital were reviewed retrospectively. RESULTS: Ages ranged from 17 to 76 years (mean 51.6+/-1.3 years) with a mean follow-up of 3.6+/-0.4 years (range 0-10.1 years). Overall 5-year survival rate was 68.1%, and 5-year survival rate of the patients who had distant metastases at initial diagnosis was 43.9%. Functioning tumors [hazard ratio (HR) 0.229, 95% confidence interval (CI) 0.056-0.943, p=0.041] and lymph node or liver metastases (HR 5.537, 95% CI 2.106-14.555, p<0.001) were the significant prognostic factors associated with survival rate. However, tumor size and pathology showed no significant association with survival. CONCLUSIONS: Because small and pathologically benign nature do not predict good prognosis in PET, aggressive treatment such as curative resection would be considered initially even in the case of incidental PET.
Adenoma, Islet Cell/*diagnosis/epidemiology/*mortality ; Adolescent ; Adult ; Aged ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Liver Neoplasms/diagnosis/secondary ; Lymph Nodes/pathology ; Male ; Middle Aged ; Multivariate Analysis ; Pancreatic Neoplasms/*diagnosis/epidemiology/*mortality ; Predictive Value of Tests ; Prognosis ; Retrospective Studies ; Survival Rate ; Tomography, X-Ray Computed ; Treatment Outcome

OBJECTIVETo summarize the surgical technique and clinical experience of total laparoscopic resection of the pancreatic islet cell tumors.

METHODSFrom July 2002 to December 2007, 30 cases including 12 males and 18 females were diagnosed as pancreatic islet cell tumor. There were at least one positive imaging examination of each patient preoperatively. The location of the tumors included 4 in proximal pancreas and 26 in distal pancreas.

RESULTSThe tumors were successfully found and removed in 28 cases and unsuccessfully located in the other 2 cases. The procedures included local resections in 13 cases and distal pancreatectomies in 15 cases (spleen reserved in 7 cases). The mean operation time was 165 min (range, 65 - 465 min). The mean blood loss was 145 ml (range, 50 - 800 ml). Pancreatic leakage occurred in 3 cases, 2 of which were cured conservatively. And the other one were cured by endoscopic retrograde cannulation of the pancreatic duct. The mean postoperative hospital stay was 5.6 days (range, 2 - 17 d). There were no conversions and death. After follow up of (14.3 +/- 16.7) months (range, 4 - 62 months), there were no recurrences.

CONCLUSIONTotal laparoscopic resection is a safe and effective method for pancreatic islet cell tumors.

Adenoma, Islet Cell ; surgery ; Adult ; Aged ; Female ; Follow-Up Studies ; Humans ; Laparoscopy ; Male ; Middle Aged ; Pancreatectomy ; methods ; Pancreatic Neoplasms ; surgery ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo analyze the clinical and pathological features in order to investigate appropriate way of diagnosis and treatment for non-functional islet cell tumors of the pancreas (NFICT).

METHODSThe data and experience of surgically treated 43 patients with pathologically confirmed NFICT over the last 30 years were retrospectively reviewed. The survival rate was estimated using Kaplan-Meier method and the potential risk factors affecting survival were compared with Log rank test.

RESULTSThere were 7 males and 36 females in this series with a mean age of 31.6 years ranged from 8 to 67 years. Twenty-eight patients were diagnosed as having non-functional islet cell carcinomas of the pancreas (NFICC) and 15 patients benign islet cell tumors. The most common symptoms in NFICT were abdominal pain 55.8%, nausea and/or vomiting (32.6%), fatigue (25.6%) and abdominal mass (23.3%). Preoperatively, all of those were found to have a mass in their pancrease by ultrasonic and computed tomography examination, with 21 in the head, 10 in the body and 6 in the tail of the pancreas. Multicemtric tumor were found in one patient. Thirty-nine of these 43 patients (90.7%) underwent surgical resection, with a curative resection in 30 (69.8%) and palliative in 9 (20.9%). The resectability and curative resection rate in 28 patients with nonfunctioning islet cell carcinomas of the pancreas was 78.6% and 60.7%, respectively. None of the 15 patients with benign nonfunctioning islet cell tumor of the pancreas died of this disease. While the overall cumulative 5- and 10-year survival rate in 28 patients with non-functional islet cell carcinomas of the pancreas was only 58.1% and 29.0%, respectively. Curative resection, female, younger than 30 years old and mass diameter < 10 cm were found to be positive prognostic factors. But multivariate Cox regression analysis indicated that radical resection was the only independent prognostic factor (P = 0.007).

CONCLUSIONNonfunctioning islet cell tumor of the pancreas is frequently found in young female. Surgical resection, especially curative resection can achieve satisfactory long-term survival.

Adenoma, Islet Cell ; diagnosis ; therapy ; Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoma, Islet Cell ; diagnosis ; therapy ; Chemotherapy, Adjuvant ; methods ; statistics & numerical data ; Child ; Combined Modality Therapy ; Doxorubicin ; therapeutic use ; Female ; Fluorouracil ; therapeutic use ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Mitomycin ; therapeutic use ; Multivariate Analysis ; Pancreatic Neoplasms ; diagnosis ; therapy ; Pancreaticoduodenectomy ; methods ; statistics & numerical data ; Proportional Hazards Models ; Regression Analysis ; Retrospective Studies

Adenosquamous carcinoma of the pancreas is a rare tumor; its clinical features and radiologic findings are not well known, so the definite preoperative diagnosis of this tumor is quite difficult. We report a case of adenosquamous carcinoma of the pancreas. A 47-year-old woman was admitted on April 2005 to our hospital with a complaint of left upper quadrant pain. The serum CA 19-9 and CEA level were increased up to 4019.08 U/ml and 13.13 ng/ml, respectively. An abdominal computed tomographic (CT) revealed a 10x9 cm sized well-defined homogenous lobulated mass in the pancreas tail. Under the impression of solid-pseudopapillary neoplasm (SPN) or nonfunctioning islet cell tumor, distal pancreatectomy and splenectomy with wedge resection of stomach were performed. The specimen consisted of a huge pancreatic mass that was attached to the posterior wall of stomach. The cut surface of pancreatic mass revealed a huge whitish infiltrative mass. Microscopically, glandular elements of ductal adenocarcinoma were admixed with solid squamoid complexes or squamous cell carcinoma components. Although the origin of the adenosquamous carcinoma has not been well proven, the histopathological findings of our case support the theory of squamous metaplasia.
Adenocarcinoma ; Adenoma, Islet Cell ; Carcinoma, Adenosquamous* ; Carcinoma, Squamous Cell ; Diagnosis ; Female ; Humans ; Metaplasia ; Middle Aged ; Pancreas* ; Pancreatectomy ; Splenectomy ; Stomach