Arteriovenous malformations of the bowel: an analysis of 22 cases and a review of the literature.

Twenty-two patients with colonic arterio-venous malformations (AVM) are reported and 218 other cases in the English literature are reviewed. Of our patients, 16 (73%) underwent right hemicolectomy because of repeated episodes of rectal bleeding or iron-deficiency anemia. Ten (63%) of these patients have remained in good health with no further evidence of intestinal bleeding after surgery. In six (37%) recurrent episodes of rectal bleeding or iron deficiency anemia have gone unexplained despite thorough work-up including repeat arteriography. Our experience and that in the literature suggests: 1. Most AVM's (78%) occur in the cecum or right colon, the jejunum being the next most common site. 2. Patients with AVM's tend to be elderly with a strong history of cardiovascular disease. A potential association between aortic stenosis, intestinal bleeding, and AVM's is suggested. However, prospective studies are needed to confirm this relationship. 3. In younger patients AVM's tend to occur at atypical sites such as the small bowel. 4. Surgical removal of an AVM cannot be expected to be uniformly curative. The causes of recurrent bleeding after surgery include: incomplete resection of the initial AVM, occult AVM's missed on arteriography and left behind at surgery, and the appearance of new AVM's postoperatively. The cause of bleeding in many patients with recurrence however, remains undiagnosed despite repeat angiography.

Focal lymphoid hyperplasia (pseudolymphoma) of the esophagus.

Focal lymphoid hyperplasia is an uncommon but ubiquitous lesion. It occurs most commonly in the gastrointestinal tract in association with chronic peptic ulcer disease of the stomach. We describe the hitherto unrecognized association of lymphoid hyperplasia in the esophagus with chronic stenosing ulcerating esophagitis and Barrett's mucosa. This association is considered to be analogous to the more prevalent coexistence of lymphoid hyperplasia and chronic peptic ulcer disease in the stomach.

Epithelioid hemangioendothelioma of liver. Clinicopathological correlation of 10 cases treated by orthotopic liver transplantation.

We present a review of the clinicopathologic features of a series of 10 patients with hepatic epithelioid hemangioendothelioma treated by orthotopic liver transplantation. This tumor has an unpredictable prognosis. Morphological features predictive of biological behavior were not identified in these patients. It appears that metastatic spread at the time of surgery is not a significant contraindication to surgery and has no correlation with survival. Nine patients are alive (5-134 months), five disease free and four with tumor. The etiology of this tumor is unknown. However, there is an association of oral contraceptive use with this tumor in four (66%) of the six female patients in this series and in 37% of such reported cases.

Transplantation of the small intestine: the pathologist's perspective.

Small-bowel transplantation is now ready for clinical trials. The surgical techniques and methods for immunosuppression and monitoring bowel status have been developed in animal models over the past 30 years. Several attempts at small-bowel transplantation in humans have already been reported. In the course of future trials, pathologists will be involved in the monitoring of the posttransplant course by mucosal biopsies and functional studies, including maltose and xylose absorption tests. The morphology of rejection has been studied in canine and rat models. Activated lymphocytes and plasma cells infiltrate the lamina propria and invade crypt epithelium, causing "cryptitis." Villous blunting ensues, resulting eventually in necrosis. Graft survival without immunosuppression is about 10 days. Under Cyclosporine immunosuppression, a lymphoplasmacytic infiltrate has been noted around nerves and vessels in the submucosa. The overlying mucosa may be relatively normal. End-stage bowel is characterized by a contracted, scarred mass. Due to the large amount of lymphoid tissue in the allograft, graft-versus-host disease is a significant problem in small-bowel transplantation.

Evaluation of gastric biopsies for neoplasia: differences between Japanese and Western pathologists.

Cited variations in the evaluation of gastric endoscopic biopsies for neoplasms between pathologists in Japan and those in the United States and Europe (the West) may have stemmed from several causes. The five-tiered group classification of the Japanese Research Society for Gastric Cancer (JRSGC) for interpretation of biopsies is not used in the West. Some differences may also exist in the morphologic criteria to reach a diagnosis of dysplasia or carcinoma. The goals of this study were to test the Western and Japanese classifications of gastric dysplasia and adenocarcinoma and to assess the differences between four Japanese and seven Western pathologists. One hundred biopsies, 20 from each of the five categories of the JRSGC scheme as determined by one observer, were collected. The Japanese observers used the JRSGC system, expressed in Roman numerals, whereas Western pathologists used a five- or six-tiered scheme expressed in diagnostic terms. Pairwise agreement was evaluated using k statistics within both groups. Consensus diagnosis on each biopsy was accepted as the opinion of the majority. The sensitivity and specificity of each reviewer for a certain diagnosis were also assessed. The intragroup agreements were moderate for both the Japanese (mean k = 0.663) and the Westerners (mean k = 0.652). The pairwise agreements between Japanese and Western observers were low (mean k = 0.542). Overall, the sensitivity was low for all Japanese observers for the diagnosis of dysplasia (38.7% vs 92.5%), and the sensitivity for the diagnosis of adenocarcinoma was high in both groups but higher among the Japanese observers (93.9% and 85.2%, respectively). Overall, the Japanese-Western interobserver agreement was moderate. The JRSCG scheme did not translate into higher interobserver agreement among Japanese observers. The sensitivity for the diagnosis of gastric adenocarcinoma was high for both groups, but the specificity was low among the Japanese. The cause seemed to be centered around the diagnosis of dysplasia in the Western system, which was a lesion frequently interpreted as carcinoma in Japan because of the different definitions of carcinoma in each system. Such a discrepancy might be important because it may explain some of the differences in the prevalence and prognosis of early gastric cancer between Japan and the West. An international effort is needed to harmonize morphologic criteria and analyze whether therapeutic consequences may stem from such discrepancies.

Fibrolamellar carcinoma of the liver: an immunohistochemical study of nineteen cases and a review of the literature.

Hepatocellular carcinoma (HCC) is a rapidly fatal neoplasm of high worldwide prevalence. Fibromellar carcinoma (FLC), a variant of HCC, lacks the dismal prognosis of "ordinary" HCC (O-HCC) and is characterized by a diagnostic histologic appearance. The current study analyzes the clinical characteristics, immunohistochemistry, and treatment of nineteen cases of FLC. These data, together with a detailed review of the literature, further characterize this unique variant. FLC affects younger patients and lacks the male predominance of O-HCC. Also, FLC lacks specific association with cirrhosis, hepatitis B virus infection, use of oral contraceptives, and alcohol abuse, all of which are implicated in other hepatic tumors. This, along with differences in serum tumor marker prevalence (AFP, B12 binding protein) suggests that its pathogenesis differs from that of O-HCC. Despite these differences, FLC shares a common differentiation with O-HCC. The increased amounts in FLC of stainable alpha-1-antitrypsin, fibrinogen, and C-reactive protein, all of which are acute phase reactants and normal hepatocyte products, implies better differentiation of FLC cells. Finally, the better prognosis of FLC is supported by this study, since only two of the 19 patients died because of tumor. This contrasts with the reported survival of patients with O-HCC, usually measured in weeks. Hepatic transplantation may hold promise for future patients with "surgically unresectable" FLC as procedure-related complications are overcome.

Colonic ganglioneuroma.

Ganglioneuromas are neuroectodermal tumors that are found in diverse anatomic sites, but they are very uncommon in the colon. We observed a patient with partial colonic obstruction whose roentgenogram had a napkin-ring construction that was thought to be carcinoma. On resection, pathologic examination showed a ganglioneuroma. Mesenchymal neoplasms of various histologic types occur thoughout the gastrointestinal tract. Neurogenic tumors are not uncommon in Von Recklinghausen's neurofibromatosis and multiple mucosal neuroma syndrome, but solitary lesions unassociated with these conditions are rare. To our knowledge, this complication in the colon is only the second such reported case. We believe that this case is noteworthy because of its clinical and roentgenographic resemblance to carcinoma.

Immunomagnetic T-lymphocyte depletion (ITLD) of rat bone marrow using OX-19 monoclonal antibody.

Graft versus host disease (GVHD) may be abrogated and host survival prolonged by in vitro depletion of T lymphocytes from bone marrow (BM) prior to allotransplantation. Using a mouse anti-rat pan T-lymphocyte monoclonal antibody (OX19) bound to monosized, magnetic, polymer beads, T lymphocytes were removed in vitro from normal bone marrow. The removal of the T lymphocytes was confirmed by flow cytometry. Injection of the T-lymphocyte-depleted bone marrow into fully allogeneic rats prevents the induction of GVHD and prolongs host survival.

Barrett's mucosa with multiple carcinomas of the esophagus and oral cavity.

A 62-year-old man with carcinoma of the tongue had two separate asymptomatic carcinomas of the esophagus, one squamous and the other adenocarcinoma arising from Barrett's mucosa. We review the multicentric origin and synchronous occurrence of tumors in the oropharyngoesophageal area with and without Barrett's mucosa to emphasize the importance of complete evaluation of the esophagus in patients with oropharyngeal malignancy.

Comparative histochemistry of gastrointestinal mucosubstances.

Two histochemical sequential staining techniques, Alcian blue-PAS (AB-PAS) and high-iron-diamine-Alcian blue (HID-AB), which differentiate neutral from sulfated and non-sulfated acid mucosubstances, were applied to sections from the gastrointestinal tract of eleven mammalian species, including man, to study patterns of distribution of gastrointestinal epithelial mucosubstances. The application of these techniques under uniform laboratory conditions enabled a detailed description of epithelial mucosubstances throughout the gastrointestinal tract relative to location in each species as well as a comparison of their distribution among various species. The results showed that all of the 11 species studied had a distinctly different distribution of epithelial mucosubstances throughout the entire gastrointestinal tract. These differences were most obvious in the stomach and duodenum. In each species, neutral mucosubstances were most evident in the stomach and acid mucosubstances in the intestines, with sulfomucins most prominent in the large bowel. Acid mucosubstances were noted in Paneth cell granules of rodent species only. These histochemical observations make available a uniform comparative basis for the normal distribution of mammalian gastrointestinal mucosubstances which should facilitate their study in diverse pathological conditions.

The role of the pathology department in a liver transplant program.

This article gives an overview of the role of the anatomic pathologist in a liver transplant program. The effect of such a program on the workload in each of the pathology subdisciplines is discussed. Differential diagnoses where the anatomic pathologist is of particular assistance, as well as diagnosis in which histopathology may be of little help, are described. Methods of tissue handling for optimal utilization of pathology services are outlined.

Variable expression of Marfan syndrome in monozygotic twins.

A pair of monzygotic twins with the Marfan syndrome with variable expression is presented. One of the twins, in addition to more severe musculoskeletal and ocular manifestations, had coarctation of the aorta as the cardiovascular manifestation of this symdrome. Analysis of red cell antigens, serum proteins and dermatoglyphic examination suggests a high probability of monozygosity. Accordingly, the variation in expression of this autosomal dominant disorder between the twins is most likely due to the modifying influences of environmental factors. Also noteworthy is the fact that the resected coarctation tissue demonstrated the histopathologic changes characteristic of cystic medial necrosis, and thus served as an additional piece of evidence supporting the diagnosis of the Marfan syndrome. This was of particular importance in view of the absence of any family history of this syndrome and the absence of ectopia lentis or the more typical cardiovascular manifestations in either twin.

Evaluation of esophageal tests in the diagnosis of reflux esophagitis.

The sensitivity and specificity of each of five esophageal tests used in the diagnosis of reflux esophagitis were compared with those of six combinations of two tests, one indicating esophagitis and the other indicating sphincter incompetence. The esophageal tests were performed in patients with reflux symptoms, chest pain, and esophagitis without reflux symptoms. Control data were obtained from normal subjects (negative control) and duodenal ulcer patients (positive control). The results indicate that the acid infusion test and esophageal biopsy combined with esophageal pH study after HC1 have similar sensitivity and greater specificity than any test alone. In normal subjects, the cumulative incidence of abnormalities with esophageal tests alone was 30%, but with combinations of two tests it was only 5%. The use of criteria (simultaneous esophagitis and sphincter incompetence) which establish the diagnosis of reflux esophagitis helps to resolve conflicting results obtained with single tests. The most sensitive and specific test combination for the diagnosis of reflux esophagitis appears to be esophageal biopsy with esophageal pH study after HC1.

Acute allograft rejection in liver transplant recipients: lack of correlation with loss of hepatic artery diastolic flow.

Eighty hepatic artery Doppler ultrasound (US) examinations performed in 49 patients after liver transplantation were retrospectively analyzed to determine if loss of diastolic flow correlated with pathologic evidence of acute allograft rejection. All 80 Doppler examinations were performed within 7 days of hepatic needle biopsy. Forty-three Doppler waveforms from 27 patients showed normal diastolic flow. Seventeen Doppler studies in 17 patients showed complete absence of diastolic flow. Review of biopsy results for each group showed no significant difference in the proportion of acute allograft rejection present (42% for the normal group and 46% for the group lacking diastolic flow). The data from 53 US and biopsy examinations performed 2 days apart in 37 patients confirmed the lack of correlation between absent hepatic artery diastolic blood flow and rejection. The authors conclude that the loss of hepatic artery diastolic flow has no apparent clinical application for the diagnosis of acute hepatic allograft rejection.

Eosinophilic gastroenteritis with esophageal involvement.

A patient with a lifelong history of asthma and hay fever was investigated because of symptoms of esophageal spasm. Esophageal biopsies revealed elongated papillae and basal zone hyperplasia of the epithelial layer with eosinophilic infiltration of the lamina propria and muscularis mucosae. There was no evidence of reflux. Small bowel biopsies revealed a flat mucosal pattern with absent or blunted villi, tall columar surface epithelium, and eosinophilic infiltration of the lamina propria. He did not respond to a gluten-free diet. This patient is thought to have eosinophilic gatroenteritis with esophageal involvement, the first such case reported.

Value of a single forceps biopsy of colonic polyps.

The accuracy of colonoscopic biopsies in predicting the histological diagnosis of colonic polyps removed at colonoscopy or at laparotomy was studied. Forty-two patients were colonoscoped before the removal of 50 polyps. A single fractional biopsy was obtained from each lesion with the standard endoscopic biopsy forceps and was compared to the final histological diagnosis of each excised lesion. Thirteen (26%) of the singular fractional biopsies did not demonstrate the significant histological features of the excised polyps. These tiny biopsies do not adequately represent the entire polyp. Moreover, the 2- to 3-mm size of the biopsy does not permit the study of the central submucosal area of the polyp stalk, the critical area for assessing invasive malignancy. Histological examination of a completely excised polyp is essential for accurate diagnosis and appropriate therapy.