Pathological characteristics and clinical specifications in gastroenteropancreatic neuroendocrine tumors: a study of 68 cases.

INTRODUCTION: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) represent a group of tumors, having their origin in cells of diffuse endocrine system, with particular clinical course, diagnosis and treatment. PATIENTS AND METHODS: In our study, were included 68 patients with neuroendocrine digestive tumors admitted, diagnosed and treated in Fundeni Clinical Institute, Bucharest, in the last ten years--2000-2010 (retrospective study). RESULTS: Thirty-three (49%) patients were males, 35 (51%) females, and the main age was 58.9 years. In 62 (90.3%) cases was possible to find the primary tumor. The examined tumors had different localizations: pancreas--32 (47.04%) cases (head--17 (24.99%) cases, and body and tail--15 (22.05%) cases), stomach--7 (10.29%) cases, small intestine--7 (10.29%) cases, 6 (8.82%) cases--unknown primary site (diagnosis was established on metastases), right colon--6 (8.82%) cases, liver--6 (8.82%) cases, rectum--2 (2.94%) cases, and retroperitoneum--2 (2.94%) cases. Microscopic examination revealed 59 (86.8%) malignant tumors and 9 (13.2%) benign tumors. Using WHO 2000 Classification, 28 cases of malignant tumors were well-differentiated neuroendocrine carcinomas, and 31 cases were poor differentiated neuroendocrine carcinomas. From malignant cases, 25 (42.3%) have distant metastases and 15 (25.9%) lymph node metastases. CONCLUSIONS: Cases of gastroenteropancreatic neuroendocrine tumors included in our study had clinical and histopathological features in correspondence with data from literature--slight predominance in women, predominance in 5th and 6th decades of life, the most frequent localizations were at pancreatic level--both head and body and tail, but the rarest were in colon and retroperitoneum. Most of the cases studied, were malignant tumors, from these more than a half were poor differentiated, and a quarter of them having lymph node or distant metastases.

[Intestinal intussusception due to ileal gastrointestinal stromal tumor--a case report]. / Invaginatie intestinala prin tumora gastrointestinala stromala ileala--prezentare de caz.

Intestinal occlusion due to intussusception produced by intestinal tumors is a very rare condition. Gastrointestinal stromal tumors are also rare digestive neopasias, with an impredictable malignant behavior, which are usually growing outside the intestinal wall, being rarely the initiators of an intestinal intussusception. We present the case of a 59 years old female, admitted in our hospital to elucidate the etiology of her iron deficient anaemia, which developed an intestinal occlusion at the intestinal preparation for colonoscopy. The abdominal CT scan performed in emergency conditions highlighted occlusive intestinal tumor complicated with intestinal intussusception. We performed an emergency laparotomy that revealed intestinal occlusion due to ileo-ileal intussusception produced by an ileal tumor. The surgical intervention consisted in segmental ileal enterectomy including the tumor with latero-lateral entero-enteral anastomosis. The patient recovered without complications. The histopathological and immunohisto-chemical examinations established the diagnose of gastro-intestinal stromal tumor with high risk malignant behavior, therefore the patient was guided in the oncological department for specific treatment and oncological surveillance.

[Insulinoma of the pancreas: analysis of a clinical series of 30 cases]. / Insulinomul pancreatic--analiza unei serii clinice de 30 de cazuri.

UNLABELLED: Insulinoma is the most frequent neuroendocrine pancreatic tumor and is the main cause for hypoglicemia due to endogenous hyperinsulinism. We performed an analysis of a clinical series in order to study the clinical and biological spectrum of presentation, the preoperatory imagistic diagnosis and results of the surgical approach. Between 1986-2009, 30 patients with symptoms suggesting an insulinoma were hospitalized in our department. Preoperatory localization of insulinomas was possible in 16 patients. The most sensitive imagistic methods were ecoendoscopy and magnetic resonance. Intraoperatory ultrasound was performed in 16 patients and its sensitivity in detection of insulinomas was 93%; the combination between intraoperative ultrasound and manual exploration of pancreas by the surgeon reached a 100% sensitivity. Before the intraoperatory ultrasound was used the tumor excision was predominantly done by extensive pancreatic resection, while after this was available in our centre more conservative (enucleo-resection) procedures were chosen. In 1 patient the resection was done by laparoscopy, and in 1 patient by robotic surgery. The dimensions of the tumor were less than 2 cm in most of the patients; 2 had nesidioblastosis and 2 had multiple insulinomas; all 28 patients proved to have benign insulinomas at histological specimens. Following surgery, the symptoms disappear in all patients. The most common complication following extensive pancreatic resections was acute pancreatitis, while after enucleation pancreatic fistula occurred more frequently. CONCLUSIONS: Due to small dimensions, the preoperative diagnosis of insulinomas is usually difficult, ecoendoscopy being the most sensitive method. Intraoperative ultrasound is essential for insulinoma localization and for chosing the optimal type of excision. Enucleation is the resection method to be chosen whenever this it is technical possible. In benign insulinomas the prognosis is excellent, surgical resection being curative in all cases.

[Difficulties in diagnosis and surgical treatment of the angiodysplasia of the gastrointestinal tract]. / Dificultati de diagnostic si tratament chirurgical în angiodisplaziile tractului gastrointestinal.

BACKGROUND: Angiodysplasia (AD) of the gastrointestinal (GI) tract is a rare cause of surgical GI bleeding. It frequently poses difficult problems in diagnosis and treatment. The purpose of this study is to find answers to these problems for a better management of the AD patients. MATERIALS: From 1982 to 2006 a total of 75 patients suffering of AD of the GI tract were operated in our center. They represent about 3.6% of total patients operated for GI bleeding in the same period. The age of the patients was between 9 and 81 years old, with two peaks: one between 21 and 40 years old and the other between 51 and 70 years old. The localisation of the lesions was: righ colon +/- ileum 31 patients (41.33%), stomach 13 patients (17.33%), jejunum 6 patients (8%), descendent colon +/- sigmoid 5 patients (6.66%), rectum 4 patients (5.33%), pan-colonic 4 patients (5.33%), sigmoid colon 2 patients (2.66%), cecum + transverse colon 2 patients (2.66%), ileum 2 patients (2.66%), sigmoid colon + jejunum 1 patient (1.33%), cecum + sigmoid colon 1 patient (1.33%), cecum +/- sigmoid colon + jejunum 1 patient (1.33%), jejunum + ileum 1 patient (1.33%), pan-colonic + rectum 1 patient (1.33%). According to Moore classifications 29 patients were type 1 (38%) and 45 patients were type 2 (60%). In one patient AD was associated with Crohn disease (type 4 Fowler). RESULTS: The main symptom in AD was repetitive GI bleeding, of various amplitude, often obscure in origin, the patients having many hospital entries. The medical examination that give us the best help was selective angiography which was positive in 34 of 40 patients (85%). Upper and lower endoscopy were give to 50 surgical patients, being diagnostic in 32 (64%). Histopathologic examinations confirm the diagnosis of AD in all cases, without using injection techniques. All patients were operated for symptomatic AD. Other 11 patients non included in this study were find to have angiodysplastic lesions on operatory specimens for other diseases. The main indications for operative in AD were: continuing digestive hemorrhage of growing amplitude with detected source (54 patients = 72%), inefficient endoscopic and angiographic hemostasis (8 patients = 10.66%) and patients with massive bleeding without any preoperative evaluation (13 patients = 17%). Intraoperative exploration produced little information because of the mucosal and submucosal localisation of the lesions. Operative panendoscopy was the most rewarding investigation. Various types of resections were practiced depending on the site(s) known or presumed of the lesions. Perioperative morbidity was 23% (21 patients), rebleeding being in 4 patients (5.33%). Perioperative mortality was 12% (9 patients) a consequence of advanced age, comorbid conditions and frequent extreme emergency of the operations. CONCLUSIONS: Although rare as a cause of surgical digestive bleeding, AD poses often difficult problems of diagnosis and treatment. In patients with GI bleeding, without evident cause, multiple investigated, especially elderly but not always, we must think of an AD.

[Risk factors for malignant evolution of gastrointestinal stromal tumors]. / Factori de risc în evolutia maligna a tumorilor gastrointestinale stromale.

Gastrointestinal stromal tumors are the most frequent non-epithelial digestive tumors, being classified in the group of primitive mesenchymal tumors of the digestive tract. These tumors have a non predictable evolution and where stratified regarding the risk for malignant behavior in 4 categories: very low risk, low risk, intermediate risk and high risk. We performed a retrospective non randomised study including the patients with gastrointestinal stromal tumors treated in the Department of General Surgery and Liver Transplantation of Fundeni Clinical Institute in the period January 2002 - June 2007, to define the epidemiological, clinico-paraclinical, histological and especially evolutive features of the gastrointestinal stromal tumors from this group, with a special regard to the risk factors for their malignant behavior. The most important risk factors in gastrointestinal stromal tumors are the tumor size and the mitotic index, based on them being realised the classification of Fletcher in the 4 risk categories mentioned above. In our group all the local advanced or metastatic gastrointestinal stromal tumors, regardless of their location, were classified in the group of high risk for the malignant behavior. The gastric location and the epithelioid type were positive prognostic factors, and the complete resection of the tumor, an other important positive prognostic feature, was possible in about 80% of the cases, probably because the gastrointestinal stromal tumors in our study were diagnosed in less advanced evolutive situations, only about one third being metastatic and about 14% being locally advanced at the time of diagnose. The association with other neoplasias was in our cases insignificant, only 5% of the patients presenting concomitant malignant digestive tumors and 7.6% intraabdominal benign tumors. Gastrointestinal stromal tumors remain a challenge for the medical staff, regarding their diagnose and therapeutical management, the stratification of the risk for their malignant behavior being essential for the evolution of these patients.

[Severe upper gastrointestinal hemorrhage of a rare cause: aorto-esophageal fistula successfully treated]. / Hemoragie digestiva superioara cataclismica de cauza rara: fistula aortoesofagiana rezolvata cu succes.

Aorto-esophageal fistula is a rare entity, determining huge digestive bleeding with a very poor prognostic most often. We present the case of a patient with aorto-esophageal fistula of unknown origin, treated successfully in the Center of Surgery and Liver Transplantation from Fundeni Clinical Institute. A young man, 21 years old, was admitted in our clinic from another hospital for repetitive severe upper gastrointestinal bleeding, with hematemesis and melena, pale, asthenia, without any pain; the bleeding stopped when he was admitted. The upper digestive endoscopy and esophago-gastro-duodenal radioscopy with barium did not show any lesion. During the 7th day the patient presents sudden massive hematemesis with hemodynamic instability; during the surgery we found two subcardial ulcers (stress ulcers?) for which we have done excision and suture which temporary stopped the bleeding. After 14 days, the patient had another massive bleeding; the upper endoscopy shows 28 cm far from the dental arch a protrusive formation of 6-7 mm with a telangiectasis aspect and with a white spot (possibly a central ulceration); 1 ml pure alcohol was injected inside it with temporary bleeding ceasing. After 3 days the patient is bleeding again, with marked decrease in blood pressure; a Blakemore tube stopped the bleeding until surgery was performed. During the intervention an aorto-oesophageal fistula is detected; we made the excision of the fistula with simple suture of the aorta, subtotal esophagectomy, cervico-stoma and gastrostomy, with good postoperative evolution. After 4 months the digestive transit is restored by esophagoplasty with tubulized stomach placed behind the sternum. The diagnostic and treatment difficulties encountered in these kind of cases need to consider also an aorto-esophageal fistula diagnosis, especially for the cases with Chiari triad (mild thoracic pain, sentinel digestive bleeding and exsanguination). The Blakemore tube can save patient's life by ensuring temporary hemostasis and allowing the time for a definitive surgical intervention. The subtotal esophagectomy with cervicostoma and feeding gastrostomy, followed after few months by a esophageal reconstruction , is a solution for this extreme severe cases.