False-positive somatostatin receptor scintigraphy due to an accessory spleen.

A patient with previous left caudal pancreatectomy and splenectomy presented with Zollinger-Ellison syndrome. Abdominal CT and endoscopic ultrasonography revealed a mass in the splenic area. Somatostatin receptor scintigraphy showed a nodular increase of the uptake corresponding to the lesion detected with conventional imaging. A second laparotomy was performed and the mass was resected. Histological analysis showed that the nodular lesion was an accessory spleen. Since physiologic uptake of 111In-pentetreotide is seen in the spleen, an accessory spleen mimicking a tumor, specially after previous splenectomy, may result in false-positive somatostatin receptor scintigraphy.

Steroid-responsive pulmonary hypertension in a patient with Langerhans' cell granulomatosis (histiocytosis X).

We report the case of a man with biopsy-proved pulmonary Langerhans' cell granulomatosis (histiocytosis X) and pulmonary precapillary hypertension. Pulmonary vascular resistances and mean pulmonary artery pressure decreased with corticosteroid therapy and dyspnea improved.

Helicobacter pylori infection in patients with Barrett's oesophagus: a prospective immunohistochemical study.

The prevalence of Helicobacter pylori infection in patients with Barrett's oesophagus was studied prospectively. A sensitive immunohistochemical staining of H pylori was performed in oesophageal and gastric biopsies of 73 patients from a surveillance group with this condition. H pylori was detected in 11 cases of Barrett's mucosa (15%) and in 26 gastric mucosa specimens (35.6%). All cases positive in Barrett's mucosa were also positive in the stomach. In Barrett's oesophagus, H pylori was never found on specialised epithelium. The percentage of Barrett's mucosa showing inflammatory changes was similar in specimens with and without H pylori, both for chronic (81% v 79%) and acute (9% v 10%) infiltrates. These results indicate that H pylori infection does not play an aetiological role in Barrett's oesophagus and that colonisation of the metaplastic mucosa by this bacteria is related with the presence of gastric type mucosa in the oesophagus and of H pylori infection in the stomach.

Successful resection of giant intrapericardial lipoma.

We report a case of giant extracavitary cardiac lipoma weighing 4,800 g. The mode of presentation, the preoperative evaluation, and the radiographic features are presented. The surgical management of this very rare cardiac pathology is discussed. This is one of the largest cardiac tumors ever reported.

Clinical, anatomical, and evolutive features of patients with the Zollinger-Ellison syndrome combined with type I multiple endocrine neoplasia.

The clinical evolution of type I multiple endocrine neoplasia (MEN I) was studied in 45 patients among a consecutive series of 172 with Zollinger-Ellison syndrome (ZES). These 172 patients were seen in our hospital between 1959 and 1989. Diarrhea was half as frequent in ZES-MEN I as in sporadic ZES cases. At diagnosis, mean basal acid output and serum gastrin levels in MEN I patients (28.8 +/- 6.6 mmol/h and 587 +/- 487 pg/ml, respectively) were not different from those observed in the others with sporadic ZES. Laparotomy was performed in all 36 patients with no diffuse liver involvement to attempt the removal of gastrinomas. Twenty-nine patients had adenomas, located in the pancreas in 21, in the duodenal wall in 3, and in both in 5. Adenomas were multiple in 23 cases (78%). No tumor was found in seven patients. Twenty-nine of the 36 operated patients were tumor-free after surgery; 7 died in the postoperative period between 1959 and 1970. Median follow-up of the 38 other patients was 95 months (range 17-278 months). Among the 24 patients without residual tumor at discharge (group I), biological and/or morphological evidence of a persistent or recurrent source of gastrin was obtained in 22. Among the 14 patients with residual tumor (group II), an increase in tumor size was seen in 5 after a median of 27 months (range 9-36 months), while no change occurred in 9 after 54 months (3-100 months). Actuarial survival curves were not different, either in group I versus group II patients (67 and 72% at 5-year follow-up, respectively) or in MEN I versus sporadic ZES patients. Apparently, complete resection of primary tumor did not reduce the incidence of subsequent liver metastases. In all, 21 of the 45 patients had malignant gastrinomas (47%), consisting of liver metastases in 14 (31%), metastatic lymph nodes in 11 (24%), and lung metastases in 2 (4%). Monitoring of fundic argyrophil cells disclosed hyperplasia in 13 of the 14 MEN I patients (92%), and 5 had invasive carcinoid tumors. Taken together, these results prompt us to recommend that in ZES-MEN I patients, surgery should be avoided and oxyntic mucosa regularly monitored.

Calcitonin-secreting tumors of the pancreas: about six cases.

Calcitonin release has rarely been reported in patients (pts) with neuroendocrine pancreatic tumors (NPT). The aim of this study was to describe the characteristics of calcitonin-secreting tumors (CST) of the pancreas. Serum calcitonin determination was part of the prospective evaluation of 66 pts with NPT referred to our institution over a 3-year period. Six pts (9%) had elevated calcitonin levels [at least twice the limit of the normal value (N)]. Abdominal ultrasonography, computed tomography scan, and endoscopic ultrasound were performed to identify the primary tumor(s) and metastases. Immunostaining using anticalcitonin and other antibodies was performed on the surgical resection specimen (four pts) or biopsy of liver metastases (two pts). Three of the six pts (four males, two females; median age, 51.5 years) had diarrhea. Serum calcitonin levels (median, range) were 17.5 N (6N-40N). Slight elevations in serum somatostatin (1.2N-2.3N) were associated in three pts. Pancreatic tumors were single in five of six pts and evenly distributed in the head and in the tail. Five pts had metastases, mainly in the liver. Multiple endocrine neoplasia type I was present in one pt. Immunostaining using calcitonin and somatostatin antibodies was positive in four pts each, respectively, and areas that were positive for one peptide were negative for the other. Diarrhea disappeared in the two pts who responded to treatment of the tumor(s). Three of the four pts with liver metastases died from tumor progression after 2, 10, and 24 months, respectively. CST of the pancreas are often malignant and can be considered as functional in half of the cases, irrespective of the serum calcitonin levels. Somatostatin secretion is often associated. Although rare, calcitonin secretion should be investigated in NPT pts presenting with diarrhea that cannot be explained by an increase in other hormone levels or in patients with nonfunctioning NPT.

Quantitative analysis of the immune cells in the anal mucosa.

The prevalence of anoperineal diseases, i.e. sexual transmitted infections, is increasing particularly in AIDS, a fact which is likely due to the alteration of mucosal immunity. However, no data were available on normal anal status. In order to study anal immunity in man, we characterized lymphocytes subtypes and Langerhans' cells (LC) using quantitative morphometric analysis and immunohistochemistry. Anal normal mucosal samples obtained from surgical specimens of 45 patients (30 suffering from hemorrhoids and 15 from fissurations) were analyzed. Immunohistochemistry was performed on frozen sections with antibodies recognizing CD1a (LC), CD3 (T lymphocytes), CD4 (T4), CD8 (T8) and CD22 (B-lymphocytes). Immunostained cells were counted per square millimeter of mucosal epithelium. The surface of CD1a cells was measured using a computerized software program and a percentage of CD1a immunostained area was calculated in comparison to the whole mucosal surface. LC and T-Lymphocytes were found in the squamous epithelium in all analyzed samples. The mean values of LC number were 84.13 +/- 9.6 and 64.77 +/- 9.8 in hemorroid- and fissure-patients, respectively. The mean values of LC area (% of CD1a stained area over total mucosal surface) were 3.89 +/- 0.44 and 4.84 +/- 0.64, respectively. In the two groups, the number of intraepithelial CD8 lymphocytes was higher than that of CD4 lymphocytes. These data suggest for the first time that anal mucosa could be considered as a part of MALT system.

Primary leiomyosarcoma of the liver MR findings.

The magnetic resonance (MR) features of a 67-year-old woman with a surgically and pathologically proved primary leiomyosarcoma of the liver studied at 1.0 T, using T1- (TR/TE = 450/15), and T2- weighted (TR/TE = 2200/45 to 90) spin-echo (SE) images, are described. On T1-weighted SE images, the tumor was well defined, was slightly heterogeneous, and displayed hypointensity to the adjacent hepatic parenchyma, with an area displaying hyperintensity. On T2-weighted SE images, the tumor was encapsulated, was heterogeneous, and displayed marked hyperintensity.

[Anatomoclinical study of aortic insufficiency in atrophic polychondritis. Apropos of a case]. / Etude anatomo-clinique de l'insuffisance aortique au cours d'une polychondrite atrophiante. A propos d'une observation.

The authors report the anatomo-clinical features of aortic insufficiency complicating atrophic polychondritis, a rare inflammatory disease affecting mainly cartilaginous tissues. This case illustrates the inflammatory changes of the aortic wall, particularly progressive during this disease, responsible for aortic insufficiency and aneurysmal dilatation of the ascending aorta which required aortic valve replacement and prosthetic replacement of the ascending aorta. Histological analysis showed inflammatory lesions of the aortic wall comparable to the cartilaginous lesions described in this condition and suggesting a common physiopathogenic mechanism.

[Carcinoid heart disease]. / Cardiopathie carcinoïde.

Carcinoid cardiac disease is a common complication of metastatic carcinoid tumours. It is characterized by tricuspid regurgitation and pulmonary stenosis. A 68 years old woman with a metastatic carcinoid tumour was admitted to hospital for congestive cardiac failure secondary to severe tricuspid regurgitation. Typical carcinoid lesions of the tricuspid and pulmonary valves were observed at echocardiography. A double valve replacement was performed with a favourable outcome. Postoperative echocardiography showed a significant improvement in right ventricular function. Surgical management of carcinoid valvular heart disease of NYHA Stage III patients is associated with an improved 2 years survival (from 8 to 40%) despite a high operative mortality (about 27%). Cardiac surgery remains the only hope of long-term survival with a spectacular improvement in symptoms.

[Obstructive jaundice caused by granulomatous stenosis of the extrahepatic bile ducts in sarcoidosis]. / Ictère obstructif par sténose granulomateuse des voies biliaires extra-hépatiques au cours d'une sarcoïdose.

A 37-year-old black West Indian woman with sarcoidosis developed obstructive jaundice due to stenosis involving the entire length of the common hepatic bile duct associated with stenosis of the cystic duct. Neither gallstones nor extrahepatic biliary tract lymph node involvement were found. Stenosis was ascribed to biliary involvement of sarcoidosis because of the presence of noncaseating granulomas in the cystic duct and the gallbladder neck. There was no hepatic involvement. Cholecystectomy and left hepaticojejunostomy were performed. Postoperative recovery was unremarkable. Jaundice disappeared and liver tests returned to normal values. This case report underlines the importance of verifying the patency of the extrahepatic biliary tract before severe cholestasis can be ascribed to intrahepatic involvement of sarcoidosis.

[Is ileorectal anastomosis still a valid therapeutic alternative in the surgical treatment of hemorrhagic rectocolitis? Analysis of the functional, endoscopic and histologic result of 74 cases]. / L'anastomose iléo-rectale est-elle encore une alternative thérapeutique valable dans le traitement chirurgical de la rectocolite hémorragique? Analyse du résultat fonctionnel, endoscopique et histologique de 74 observations.

The objectives of this study were to determine the functional results of ileorectal anastomosis (IRA) and the endoscopical and histological outcome of the rectal stump in 74 patients operated on for ulcerative colitis. Median follow-up was 7.5 years (range 1 to 35). Functional results, as defined by the usual accepted criteria, were good in 80% of patients. The mean daily number of bowel movements was 4.2 +/- 1.6 (SD). Long term endoscopical and histological features improved in most of cases and only 13.3% of rectal biopsies showed the persistence of acute inflammatory changes; 28.4% of the patients had one or more episodes of acute proctitis during the follow-up period, but none of the patients required proctectomy. One case of severe dysplasia was disclosed, leading to proctectomy. A second case of proctectomy was performed in a patient with microrectum. Biopsies performed preoperatively in this case showed the only case of moderate dysplasia found in our population. Provided that the usual contraindications to this surgical procedure are observed, IRA is still a valid option in the surgical treatment of ulcerative colitis.

[Prognostic value of x-ray computed tomography in acute pancreatitis: contribution of the injection of intravenous contrast media]. / Valeur pronostique de la tomodensitométrie dans les pancréatites aiguës: apport de l'injection de produit de contraste intraveineux.

Thirty seven patients suffering from acute pancreatitis were explored using contrast-enhanced computed tomography (CT). The authors found a good concordance between the extension of the lesions on the initial CT examination, prognostic criteria on admission and the course of the disease. In addition, a new prognostic CT sign was reported in the early phase of parenchymal contrast perfusion, i.e. the lack of contrast-enhancement in a limited portion of the pancreas. This sign was present in 8 patients, all with severe clinical symptoms. These 8 patients showed the highest morbidity rate in the series with abscess-type complications occurring in all, compared with 24 p. 100 among the 29 other patients who did not exhibit this CT sign. Moreover, these 8 patients showed the highest mortality rate (25 p. 100 compared with 3.5 p. 100 among the other patients). An histological analysis of partial pancreatectomy specimens was performed in 7 out of the 8 patients. Devitalized pancreatic tissue, at the site of the parenchymal abnormalities on CT scan, was found in all cases. One false negative case was reported. Contrast-enhanced CT scan seems to be the most reliable method for diagnosing pancreatic necrosis during acute severe pancreatitis. It appears to be an useful prognostic predictor of morbidity. It can improve the outcome of the disease by depicting and guiding needle aspiration of a fluid collection, and/or surgery in case of clinical findings suggesting abscess formation.

[Proctitis of diversion on an excluded rectum in patients with hemorrhagic rectocolitis]. / Proctite de diversion sur rectum exclu chez des patients atteints de rectocolite hémorragique.

OBJECTIVES: Diversion colitis is characterized by inflammatory lesions affecting colon or rectum excluded from the fecal stream for varied etiologies. These lesions may mimic both ulcerative colitis and Crohn's disease. The aim of our study was to examine the excluded rectum in patients with ulcerative colitis, and to study the evolution of the pathological lesions after ileo-rectal anastomosis. METHODS: Eighteen patients with ulcerative colitis treated by total colectomy before ileo-rectal anastomosis were studied. The pathological features i.e. glandular alteration, inflammatory infiltrate and mucosal ulceration or fissure, were studied during 3 periods: initial colectomy, excluded rectum at surgery for anastomosis and rectal biopsies after anastomosis. RESULTS: We observed on the excluded rectum a follicular lymphoid hyperplasia (18 cases), granulomas with giant cells (9 cases), mucosal fissures (9 cases). The inflammation extended to the submucosa in all cases and was occasionaly transmural. These lesions disappeared after the anastomosis and then seemed to be connected with the rectal diversion. CONCLUSIONS: The pathological changes of diversion proctitis that includes mucosal fissures, granulomas with giants cells or transmural inflammation, may lead to an erroneous diagnosis of Crohn's disease. The review of the previous colectomy is then mandatory to confirm the initial diagnosis of ulcerative colitis. These lesions disappear after anastomosis.

[Effect of prolonged treatment with proton pump inhibitors on serum gastrin levels and the fundus mucosa. Preliminary results]. / Influence des traitements prolongés par inhibiteurs de la pompe à protons sur la gastrinémie et la muqueuse fundique. Résultats préliminaires.

OBJECTIVES: To evaluate the evolution of fundic argyrophil cell density and hyperplasia grading, fundic chronic gastritis grading and serum gastrin levels in patients treated with proton pump inhibitors. METHODS: Thirty-two patients treated with proton pump inhibitors for gastroesophageal reflux and/or duodenal ulcer were studied. No patient had a gastric ulcer. The studied parameters were serum gastrin levels, fundic argyrophil cell density, the degree of fundic argyrophil cell hyperplasia, the grade of fundic atrophic gastritis and the presence of Helicobacter pylori. The first point of the study was 7 months (range: 0-42 months) and the last point 33 months (range: 7-72 months) after the beginning of the treatment. RESULTS: Serum gastrin levels significantly increased with treatment. Fundic argyrophil cell density did not change significantly. In 3 patients (9%), serum gastrin levels were twice the normal upper limit. The highest serum gastrin levels (249 and 665 pg/mL) were noted in the 2 patients treated with the highest doses of proton pump inhibitors. Micronodular hyperplasia of the fundic argyrophil cells was observed in 2 patients treated with omeprazole 20 mg/d for 4 years and lansoprazole 90 mg/d for 6 years, respectively. Non active superficial chronic gastritis was noted in 2 patients. Serum gastrin levels were significantly correlated with cell densities. CONCLUSION: There were minor modifications of fundic argyrophil cell population and of gastrinaemia during the study period. They were not related to chronic atrophic gastritis. However, survey is mandatory in patients treated with high dose proton pump inhibitors, in those in whom gastrinaemia is elevated and when treatment duration is longer than 5 years.

[Sensitivity of fine-needle puncture-biopsy in the histologic diagnosis of hepatic and pancreatic malignant tumors]. / Sensibilité de la ponction-biopsie à l'aiguille fine dans le diagnostic histologique des tumeurs malignes hépatiques et pancréatiques.

Aspiration-biopsies under ultrasound guidance, using a fine needle (external diameter inferior to 1 mm) have been performed in 61 malignant hepatic tumors (group I) and 25 pancreatic carcinomas (group II). Malignancy was diagnosed in 83 p. 100 cases; in group I, the sensitivity is 87 p. 100; the difference between hepatocellular carcinoma and liver metastases could be made in 88 p. 100 cases and in case of metastases, the site of the primitive carcinoma could be suggested in 77 p. 100 cases. In group II, the sensitivity is 72 p. 100. No complications were noted.

[Multifocal cryptogenetic stenosing enteritis: an autonomous entity?]. / L'entérite sténosante multifocale cryptogénétique: une entité autonome?

Multifocal idiopathic stenosing enteritis has been described as a relapsing disease affecting the small bowel. The main anatomical characteristics are multiple non-specific inflammatory strictures. Six cases (4 men, 2 women) presenting this disorder were followed up for 3 to 22 years. Five patients were young. Iterative obstruction or chronic diarrhea and loss of weight were the outstanding clinical features. Hyposideremic anemia and protein-losing enteropathy were common. The anatomical substrate was numerous (2-19) short annular strictures situated in the ileum (75 p. 100). These strictures presented shallow ulcerations and underlying fibrosis which never extended beyond the submucosa. The small bowel wall was otherwise normal and did not show mucosal or vascular abnormalities at microscopic examination. In case of relapse, strictures developed distal to or in close contact with previous suture lines. Cultures for pathogenic bacteria were constantly negative. The natural history of these 6 cases and of 7 other previously published cases suggests that this disease is an autonomous disorder which must be differentiated from other inflammatory bowel disease entities such as Crohn's disease or tuberculosis.

[Overexpression of protein p53 and Barrett esophagus. A frequent and early event in the course of carcinogenesis]. / Surexpression de la protéine p53 et endobrachyoesophage. Un événement fréquent et précoce au cours de la carcinogenèse.

OBJECTIVES AND METHODS: In Barrett's oesophagus, the risk of malignancy is evaluated histologically with the presence of dysplasia. The abnormal expression of p53 protein could represent a useful new marker. The aim of this study was evaluate the abnormal expression of p53 protein in a series of 52 oesophagectomy specimens with Barrett's oesophagus, either non-dysplastic (n = 3), dysplastic (n = 8), or malignant (n = 41). The immunohistochemical study was made on deparaffinized sections with the monoclonal anti-p53 antibody DO7. RESULTS: The 3 non-dysplastic cases were p53 negative; 1 case of low-grade dysplasia in 5 was positive, as were the 3 cases of high grade dysplasia and 33 of 41 cancers (80%), including 13 superficial cancers in 14 (93%) and 20 invasive cancers in 27 (74%). A common feature was the presence of rare p53 positive crypts in low grade dysplastic areas and non-dysplastic specialized mucosa that surrounded high grade dysplasia and cancers. CONCLUSIONS: Our results confirm the high frequency of the abnormal expression of p53 protein in cancer developed in Barrett's oesophagus. This expression is a consequence of alterations of the TP53 gene, and has an important role in the carcinogenesis of Barrett's mucosa; it is likely to represent an early event. Prospective studies are needed to evaluate its interest in the surveillance of patients with Barrett's oesophagus.

[Echotomographic, scanographic and anatomic study of a case of cystic dilatation of the common hepatic duct]. / Etude échotomographique, scanographique et anatomique d'un cas de dilatation kystique du canal hépatique commun.

The authors describe the case of a 16-year-old African woman presenting with a cystic dilatation of the common bile duct associated with a dilatation of the left intrahepatic bile duct, hepatic fibrosis and portal hypertension. The disease was revealed by a non-infectious cholestatic syndrome. The diagnosis was made before the intervention by abdominal ultrasonography and computed tomography. A choledococyst-jejunostomy was performed which led to progressive normalisation of liver function. This report emphasizes the possibility of simultaneous lesions at different levels of the biliary tree in patients with choledocal cysts. The prognosis depends upon the state of the liver. A liver biopsy is therefore mandatory when an operation is performed. In the present case, the follow-up is too short to assess the regression of the biliary cirrhosis as described in the literature.

[Papillary fibroelastoma: a rare but treatable cause of cerebral infarction]. / Le fibroélastome papillaire: une cause rare mais curable d'infarctus cérébral.

INTRODUCTION: Papillary fibroelastoma is a benign cardiac tumor which can be associated with serious embolic complications. EXEGESIS: We report on a 42-year-old man admitted for an ischemic stroke in the left middle cerebral artery region. Transesophageal echocardiography revealed a mitral valve tumor. Surgical excision and histological examination showed a papillary fibroelastoma. Clinical course was uneventful. CONCLUSION: We consider the high embolic potential of this tumor, which represents a surgically treatable cause of ischemic stroke.