Nonfunctioning Islet Cell Tumors Research Articles

Solid pseudopapillary carcinoma of pancreas: A rare case report

A 20-year-old female presented with abdominal pain and loss of weight of 3 months duration, initially diagnosed as acid peptic disorder ultra sound examination revealed pancreatic mass and contrast-enhanced computed tomography abdomen confirmed distal pancreatic mass, which was excised and sent for histopathological examination, which revealed solid pseudopapillay tumor of pancreas. Solid pseudopapillary tumors most commonly occur in young women. Till recently the tumors are misclassified as nonfunctioning islet cell tumors or solid cystic tumor of pancreas or papillary cystadeno carcionoma. The tumors are well-encapsulated and cut surfaces show solid and hemorrhagic necrotic patterns. The neoplasms usually behave like a very low grade malignancy; hence complete surgical removal is treatment of choice.

Clinical application of binding pancreaticogastrostomy in the central pancreatectomy

Objective To investigate the clinical application of binding pancreaticogastrostomy (BPG) in the central pancreatectomy (CP). Methods The clinical data of 62 patients with benign and low-grade malignant lesions in the neck and body of pancreas who received CP combined with BPG from January 2010 to October 2014 were retrospectively analyzed. Fifty-six patients with space-occupying lesions of the head and neck of pancreas were confirmed by postoperative pathological examinations, including 21 solid pseudopapillary tumors of pancreas (SPTPs), 19 pancreatic neuroendocrine neoplasms (PNENs) (13 non-functional islet cell tumors), 16 pancreatic cystic tumors (12 serous cystadenomas and 4 mucinous cystadenomas) and 6 ruptures in the head and neck of pancreas. CP combined with BPG was performed. The central pancreas was resected via upper and anterior approaches after surgical exploration, and digestive tract reconstruction was applied using BPG. The operation time, volume of intraoperative blood loss, time of postoperative gastrointestinal function recovery, drainage tube removed time, duration of hospital stay and postoperative complications were recorded. Patients were followed up by outpatient examination and telephone interview up to January 2015, and follow-up included the level of blood glucose, conditions of pancreatic exocrine function and with or without pancreatic pseudocyst. Results All the patients underwent successful operation without perioperative death. The average operation time, average volume of intraoperative blood loss, average time of postoperative gastrointestinal function recovery, average drainage tube removal time and average duration of postoperative hospital stay were 155 minutes (range, 125-230 minutes), 300 mL (range, 210-425 mL), 3.0 days (range, 2.0-5.0 days), 6.0 days (range, 4.0-10.0 days) and 10.5 days (range, 9.0-21.0 days), respectively. Seven patients with delayed gastric emptying were cured by non-surgical treatment. Of 6 patients complicated with pancreatic fistula, 4 patients (Grade A) had healed pancreatic fistulas during hospitalization, 2 patients (Grade B) with drainage tubes were discharged from hospital and then drainage tubes were removed after confirming healed pancreatic fistula by imaging examination. Of 2 patients with intraperitoneal hemorrhage, 1 underwent under gastroscope cauterisation for hemostasis and 1 underwent open reoperation for hemostasis. All the patients were followed up for 3-36 months with a median time of 25 months and without high blood glucose, pancreatic exocrine function insufficiency and pancreatic pseudocyst. Conclusions CP with the advantages of minimal invasion and quick recovery can be used as a priority surgical method for benign or low-grade malignant tumors and injures in the neck and body of pancreas. BPG is safe and feasible as well as reduce the incidence of pancreatic fistula after CP, and it is an ideal reconstruction. Key words: Pancreatic neoplasms; Pancreatic trauma; Central pancreatectomy; Binding pancreatico-gastrostomy; Pancreatic fistula

Intrapancreatic accessory spleen. A case report and review of literature.

Accessory spleen is a congenital focus of healthy splenic tissue that is separated from the main body of spleen. Although an accessory spleen usually appears as an isolated asymptomatic abnormality, it may have clinical significance in some situations. We report the case of 53-year-old woman with a 2-year history of upper abdominal discomfort after meals and weight loss. The pathologic lesion was diagnosed by the abdominal sonography and the magnetic resonance tomography in the pancreatic tail. The patient was operated with suspicion of a solid pseudopapillary neoplasm or a nonfunctioning islet cell tumor. Histopathological examination found an intrapancreatic accessory spleen, which is a congenital abnormality consisting of normal splenic tissue in ectopic sites. We present possibilities of differential diagnosis of this entity.

A Critical Approach to Clinical Biochemistry of Chromogranin A.

Chromogranin A (CGA) is a member of the granin family of proteins which are widespread in endocrine, neuroendocrine, peripheral, and central nervous tissues, where they are typically found in secretory granules. It is well accepted that CGA cooperates to regulate synthesis and secretion of these various granule signaling molecules.Because of its ubiquitous distribution within neuroendocrine tissues, CGA can be a useful diagnostic marker for neuroendocrine neoplasms, including carcinoids, pheochromocytomas, neuroblastomas, medullary thyroid carcinomas (MTC), some pituitary tumors, functioning and nonfunctioning islet cell tumors and other amine precursor uptake and decarboxylation (APUD) tumors. It is also useful as a prognostic marker for detection of recurrence and monitoring of response to different treatments. As other tumor markers, it is imperative to know its physiology and pathophysiology, its sensitivity and specificity in different neuroendocrine tumors (NETs), and carefully integrate these data with the clinical data of the single patient, to maximize its diagnostic/prognostic index.

The Transformation of a Nonfunctioning Islet Cell Tumor of the Pancreas into a Proinsulinoma under Conditions of Lung Metastasis

We herein report the first case of a nonfunctioning islet cell tumor that transformed into a proinsulinoma during the process of metastasis to the lungs. This phenomenon was confirmed in a 69-year-old woman with an advanced pancreatic islet cell tumor and multiple liver metastases who later developed multiple lung metastases. She underwent pancreatic resection followed by the administration of chemotherapy and survived for seven years. Although the patient initially had hyperglycemia due to diabetes mellitus, she conversely began to manifest hypoglycemic attacks 63 months postoperatively with the concomitant development of multiple lung metastases. An autopsy revealed that only the tumor in the lungs produced proinsulin; no other hormones were detected.

Nonfunctional Islet Cell Tumor of the Pancreas in a Patient with Tuberous Sclerosis: A Case Report with Literature Review

Islet cell tumors (ICTs) are rare tumors of the pancreas. Association of this type of tumor with tuberous sclerosis is extremely rare. Only 13 cases of pancreatic ICT with tuberous sclerosis have so far been documented in the literature. However, awareness of the association of tuberous sclerosis and ICT is important for early diagnosis and appropriate treatment of this condition. This article presents the case of a 63-year-old female with angiomyolipoma (AML) of the kidney and liver, calcified subependymal nodules and a large mass in the pancreas, which was proven to be an ICT on histopathological examination.

Analysis of clinicopathologic characteristics and prognostic factors in non-functioning islet cell tumor: A report of 70 cases

Analysis of clinicopathologic characteristics and prognostic factors in non-functioning islet cell tumor: A report of 70 cases

Diagnosis and treatment of 51 patients with pancreatic islet cell tumors

To investigate the diagnosis and treatment of pancreatic islet cell tumors. Fifty-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. Among 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. Intraoperative 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.

A case of nonfunctioning pancreatic islet cell tumor in a young female

Pancreatic endocrine tumors are uncommon and represent 1-2% of all pancreatic neoplasms. Nonfunctioning pancreatic endocrine tumors constitute 30-40% of pancreatic endocrine tumors. They can occur at any age, but are rare in childhood. About 65-80% of nonfunctioning pancreatic endocrine tumors are associated with clear-cut evidence of malignant behavior. A 14-year-old girl presented with abdominal pain of 2 weeks duration with occasional vomiting. Abdominal examination did not reveal any abnormality. Ultrasound and computed tomography (CT) scan of the abdomen showed solid mass lesion located in the head of pancreas. The patient underwent Whipple's pancreaticodudenectomy. Microscopically, the mass was circumscribed, containing oval to polygonal cells. There was no pleomorphism, significant mitotic activity or evidence of capsular or vascular invasion, and a diagnosis of benign pancreatic islet cell tumor was made. Although benign nonfunctioning pancreatic islet cell tumor is rare in children, such diagnosis should be considered when there is an isolated mass in the pancreas. Appropriate resection of such mass is attended with good outcome.

Nesidioblastosis and Pancreatic Non-functioning Islet Cell Tumor in an Adult with Type 2 Diabetes Mellitus

Nesidioblastosis is a clinically, pathologically, and genetically heterogenous disease observed in neonates with persistent hyperinsulinemic hypoglycemia of infancy, but it is very rare in adults.1 Although progression from nesidioblastosis and islet hyperplasia to pancreatic tumors has been observed in an animal study using transgenic mice,2 islet cell tumors associated with nesidioblastosis in human are exceedingly rare.3,4 Herein we report a very unusual case of nesidioblastosis coexisting with a pancreatic non-functioning islet cell tumor in an adult with type 2 diabetes mellitus (type 2 DM).

An Interesting Association Between Rhabdomyosarcoma and Pancreas

Purpose: Introduction: Alveolar rhabdomyosarcoma (ARMS) is a malignant tumor arising from skeletal muscles which is common in children but very rare in adults. Here we present a case with ARMS metastatic to pancreas. Case: A 21 year old man presented with epigastric pain and yellow discoloration of eyes for 3 days. A month prior, he noticed progressively increasing swelling over left forearm with two other swellings in the arm and axilla. He denied fever, changes in appetite, weight or trauma. Vitals were stable. Scleral icterus was noted. A 6-cm immobile, hard, non-tender mass was felt on distal forearm. Another 5 cm mass was felt over distal humerus with axillary and supraclavicular lymphadenopathy. Abdominal examination revealed epigastric tenderness, negative Murphy's sign and no organomegaly. Testes were normal with absent inguinal adenopathy. Laboratories revealed a normal hemogram, elevated lipase and mixed hepatocellular and cholestatic picture on hepatic panel. Serologies for viral hepatitis and autoimmune diseases were negative. CT abdomen showed a diffuse mass in head, body, and tail of pancreas with biliary ductal dilatation. MRI of left upper extremity showed multiple bulky infiltrative masses invading fascial planes. Initial impression of lymphoma or non-functioning islet cell tumor was made. However, the hormonal work-up and tumor markers were negative. Biopsies from the hand mass, supraclavicular node and EUS guided pancreatic biopsy confirmed the tumor to be metastatic ARMS. With conservative management, the pancreatitis improved. Common bile duct stent was placed via ERCP. For further management patient was referred to another hospital equipped with pediatric oncology services. Discussion: It is postulated that ARMS arising from extremities can be particularly aggressive with metastasis to unusual sites like breast, testes or pancreas. Only eight cases of clinically overt pancreatic metastasis have been reported so far. However autopsy studies show incidence of subclinical pancreatic metastasis from ARMS as high as 67%. The involvement of pancreas even in the absence of other abdominal metastasis, raises the possibility of a biological explanation for this spread. Reports state that PAX/FKHR fusion protein, characteristic of ARMS, induces expression of IGF-I molecule. Pancreas is highly influenced by IGF-like proteins which make it a target organ for metastasis from ARMS. Thus, the high likelihood of pancreatic metastasis in ARMS makes it vital for the oncologist to screen the abdomen with PET-CT. Treatment with IGF-IR monoclonal antibodies in metastatic disease may achieve favorable outcomes.

Misdiagnosis of Intrapancreatic Accessory Spleen as an Endocrine Tumor of the Pancreatic Tail

a ( a p s a l p i a A man without any symptoms or signs had a health examination. An enhancing round mass of approxmately 2 cm in diameter was delineated in the tail of the ancreas by an abdominal computed tomography scan (Figure ). A nonfunctioning islet cell tumor or solid pseudopapillary eoplasm without necrosis of pancreatic tail was suspected. hysical examination and laboratory data were all unremarkble. At operation, palpation of the pancreas did not reveal an bvious mass. On the basis of the computed tomography findngs, partial pancreatectomy was performed, and spleen was reserved. On section, the cut surface shows a relatively wellemarcated brownish-red solid mass, measuring 1.6 1.5 cm n size (Figure B). Microscopic examination of the mass showed he typical structure of normal splenic tissue with well-develped red-and-white pulp (Figure C). An accessory spleen is a relatively common congenital efect. In an autopsy study of 3000 patients, there were 364

Nonfunctional islet cell tumor: a report on 26 cases and review of the literature

Objective To review our experience in the diagnosis and treatment of nonfunctional islet cell tumors(NICT).Method The clinicopathological data of 26 cases of NICT were retrospectively analysed and the medical literature was reviewed.Results In addition to the 26 cases seen in our center,there were 569 cases published in 37 articles in the medical literature.In China,NICT was more common in women.The male to female ratio was about 3 ∶ 7.The mean age of onset of the disease was 35 years old.Most NICT were solitary and malignant,surgery was effective in prolonging long-term survival.Conclusions NICT is rare and it had no specific clinical presentation.BUS and CT are useful for diagnosis and surgery is effective. Key words: Nonfunctional; Islet cell tumor; Pancreatic endocrine tumor

Middle segment pancreatectomy for the benign tumors of the neck and body of the pancreas (report of 15 cases)

To study the clinical application value of middle segment pancreatectomy in the treatment of benign tumors of the amphi-neck of the pancreas. Fifteen cases were retrospectively analyzed treated from November 2005 to June 2009. There were 3 male and 12 female aging from 30 to 50 years. They all received middle segment pancreatectomy for benign tumors of the amphi-neck of the pancreas. There was no death during perioperative period. All the 15 patients received middle segment pancreatectomy. Fourteen of them received the closure of broken ends of pancreatic head, pancreaticojejunostomy (mono-anastomosis) and the rest one received dipl-anastomosis. Postoperative pathology showed that in the 15 patients, 1 got solid-pseudopapillary tumor of the pancreas, 3 got non-functional islet cell tumor, 11 got cystadenoma of pancreas. Three of them got pancreatic fistula and were self cured in 3 months. Follow-up visits to all the patients kept in the following 2 to 45 months. There was no death. No patients got new-onset diabetes and pancreatic pseudocyst. And their tumors were not relapsed. There is an exact therapeutic effect of middle segment pancreatectomy for benign tumors of the amphi-neck of the pancreas. The treatment has little function damage to patients' endocrine and external secretion. The incidence rate of pancreatic fistula in middle segment pancreatectomy is higher than that in pancreaticoduodenectomy. As long as the drainage is kept unobstructed, most of the pancreatic fistula can be self cured.

Clinical applications of laparoscopic pancreatectomy

To summarize the experiences of laparoscopic pancreatectomy and to discuss its clinical indications and operative techniques. From March 2003 to February 2010, 49 patients with pancreatic diseases, including 18 males and 31 females with a mean age of (42 ± 14) years old (range: 21 - 77), underwent laparoscopy at our department. The preoperative diagnoses included insulinoma (n = 38), cystadenoma (n = 4), cystadenocarcinoma (n = 1), nonfunctional islet cell tumor (n = 5) and solid-pseudopapillary tumor (n = 1). Thirty-nine cases underwent total laparoscopic surgery and 8 cases converted to open surgery. The operative duration was (245 ± 146) minutes (range: 90 - 960). The mean intraoperative blood loss was (191 ± 306) ml (range: 20 - 1500). Secondary operations were needed in 3 cases and pancreatic fistula occurred in 7 cases. The postoperative hospital stay was (10 ± 7) days (range: 4 - 31). Laparoscopic pancreatectomy is safe and feasible in the treatment of most benign pancreatic diseases and some malignant tumors.

Endocrine Tumor of the Pancreas With Intraductal Spreading

A 68-year-old woman visited our hospital because of jaundice. CT showed an enhanced area measuring 4cm in diameter in the head of pancreas. The tumor had prolapsed from the orifice of the papilla of Vater. Endoscopic biopsy was performed and the tumor was pathologically diagnosed as a non-functioning islet cell tumor. Pancreaticoduodenectomy was performed. The main pancreatic duct was occupied with the tumor that had prolapsed from the orifice of the papilla of Vater in the resected specimen. The final diagnosis was a non-functioning islet cell tumor. Usually, endocrine tumors show expanding growth. Although some cases of endocrine tumors showing intraductal growth have been reported, tumors prolapsed from the orifice of the papilla of Vater seem to be very rare. We present a case of a non-functioning islet cell tumor uniquely growing within the lumen of the main pancreatic duct.

Pancreatic islet cell tumor mimicking obstructive chronic pancreatitis: a case report

Context: Pancreatic islet cell tumors are uncommon lesions, and nonfunctional islet cell tumors presenting with pancreatitis are extremely rare. Many patients with pancreatitis and islet cell tumors may initially present with acute attacks, which sometimes recur and the diagnosis of the tumors are usually delayed. Case report: We report a case of 44-year-old man who presented with a clinical impression of “calcifying chronic pancreatitis and pseudocyst” for 1 1⁄2-year before nonfunctional islet cell tumor was diagnosed. He had a distal pancreatectomy, splenectomy and partial gastrectomy after failure of conservative management. Grossly a 1.7 cm ill-defined tumor was identified which was compressing the pancreatic duct. Histology showed nests and trabeculae of uniform endocrine cells positive for chromogranin and negative for glucagon, insulin, somatostatin and gastrin. A lymph node was positive for metastatic tumor. Conclusions: Due to this rare clinical presentation and to prevent delayed detection and complications, islet cell tumor should be considered in patients with non-remitting, persistent calcifying chronic pancreatitis. Careful pathological examination of such pancreatic resection specimen is also recommended as very small tumors can cause obstructive pancreatitis.

Central pancreatectomy for benign tumors of the neck and body of the pancreas: Report of eight cases

To discuss the advantage of central pancreatectomy in the patients with benign tumors of the neck and body of the pancreas. Eight patients from January 1990 to December 2007 were retrospectively analyzed in our hospital. The operation is carried out by exposition of the pancreatic neck and body involved by the lesion. Thereafter, the lesion is dissected from the spleen artery and porto-mesenteric vein. The cephalic stump is sutured, and the distal stump is anastomosed by an end-to-end invaginated pancreaticojejunostomy with double-layer suture. Central pancreatectomy was done in eight patients including five mucinous cystadenomas, one serious cystadenoma, one insulinoma and one nonfunctional islet cell tumor. No mortality rate developed and morbidity rate was 37.5%. Pancreatic fistula occurred in three cases and was treated conservatively. Central pancreatectomy is a safe technique for benign tumors of the pancreatic neck and body, especially when the enucleation is very difficult.

Diagnosis and surgical treatment for non-functional islet cell tumor: a retrospective analysis of 44 cases

To evaluate the methods of diagnosis and surgical treatment for nonfunctional islet cell tumor (NICT). Forty-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. pancreatic 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. No 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.

Conservative resection for benign tumors of the proximal pancreas

To evaluate the safety and long-term prognosis of conservative resection (CR) for benign or borderline tumor of the proximal pancreas. We retrospectively analyzed 20 patients who underwent CR at the Second Affiliated Hospital of Zhejiang University School of Medicine between April 2000 and October 2008. For pancreaticojejunostomy, a modified invagination method, continuous circular invaginated pancreaticojejunostomy (CCI-PJ) was used. Modified continuous closed lavage (MCCL) was performed for patients with pancreatic fistula. The indications were: serous cystadenomas in eight patients, insulinomas in six, non-functional islet cell tumors in three and solid pseudopapillary tumors in three. Perioperative mortality was zero and morbidity was 25%. Overall, pancreatic fistula was present in 25% of patients. At a mean follow up of 42.7 mo, all patients were alive with no recurrence and no new-onset diabetes mellitus or exocrine dysfunction. CR is a safe and effective procedure for patients with benign tumors in the proximal pancreas, with careful CCI-PJ and postoperative MCCL.