Alberto A. Diaz-Arias

Primary small-cell carcinoma of the esophagus : report of 11 cases and review of the literature

Primary small-cell carcinoma of the esophagus is an uncommon esophageal malignancy. This report details the clinical and pathologic aspects of 11 cases seen at our institution over 20 years, as well as 123 other cases reported in the literature. Small-cell carcinomas of the esophagus show considerable histologic heterogeneity. Neurosecretory granules can be found in the majority of cases and some show evidence of multidifferentiation. Like primary small-cell cancers of the lung, those in the esophagus are highly aggressive, are usually associated with spread at the time of diagnosis, and have a dismal prognosis regardless of treatment. The possible origin of this interesting variety of esophageal neoplasm is also discussed.

En bloc endoscopic submucosal dissection of a 14-cm laterally spreading adenoma of the rectum with involvement to the anal canal: expanding the frontiers of endoscopic surgery (with video)

BACKGROUNDnEndoscopic submucosal dissection (ESD) was recently developed in Japan for en bloc removal of laterally spreading tumors (LSTs). Although initially used for gastric tumors, ESD has now been applied to lesions elsewhere in the gut. Recent reports from Japan included removal of colorectal lesions up to 10 cm.nnnOBJECTIVEnTo show the feasibility of ESD to remove en bloc, very large LSTs of the rectum, even when there is involvement to the dentate line.nnnDESIGNnCase report.nnnSETTINGnThe procedure was performed at an American GI unit. The patient was admitted to the hospital after the procedure for observation.nnnPATIENTSnA 53-year-old patient, with a 14-cm tubulovillous adenoma of the rectum, which, at its maximal extent, involved two thirds of the circumference of the rectum. The tumor extended distally to the dentate line.nnnINTERVENTIONSnEn bloc submucosal dissection with a conventional needle-knife to remove the neoplasm.nnnMAIN OUTCOME MEASUREMENTSnCompleteness of en bloc removal of the tumor and subsequent follow-up endoscopy that showed no residual neoplasm.nnnRESULTSnThe tumor was able to be removed en bloc by ESD. The distal margin included squamous mucosa. At a 2.5-week endoscopic follow-up, a 3-mm focus of residual polyp was seen and removed. At the time of the last follow-up, there was complete healing of the wound and no residual neoplasm.nnnLIMITATIONSnSingle case.nnnCONCLUSIONSnThis case demonstrated the feasibility of using ESD to remove large laterally spreading rectal tumors, including when there was involvement to the dentate line (and the dissection line must include squamous mucosa of the anal canal). ESD is a promising alternative to conventional surgical techniques; however, additional published experience is needed.

Achalasia Due to Diffuse Esophageal Leiomyomatosis and Inherited as an Autosomal Dominant Disorder: Report of a Family Study

Although achalasia is usually of idiopathic origin, it may be secondary to another disease process such as neoplasia. The first description of a familial achalasia syndrome that appears to be secondary to diffuse esophageal leiomyomatosis with entrapment of nerve ganglia is presented. Documented in four generations of a family, the disease followed an autosomal dominant mode of inheritance. Long lower esophageal sphincter pressure zones and a high incidence of epiphrenic diverticula were interesting accompaniments of achalasia in this family. Many achalasia-affected family members have also had associated intestinal leiomyomas or neurofibromas. Affected family members also had urticaria pigmentosa, and some had systemic mast cell disease as well.

Can pancreatitis be associated with amiodarone hepatotoxicity

We report a patient with classical features of amiodarone hepatotoxicity who died of progressive liver failure. Throughout the course of his illness, he had epigastric pain, nausea, vomiting, and persistent mild to moderate elevation of amylase and lipase in his serum and peritoneal fluid. Pancreatitis due to amiodarone has not been reported. We raise the question of whether or not the pancreas is yet another organ subject to amiodarone toxicity and speculate as to possible pathogenesis. We suggest that patients on amiodarone who develop abnormal liver enzymes, nausea, vomiting, or abdominal pain be evaluated not only for hepatotoxicity, but for pancreatitis as well.

Reactive hemophagocytic syndrome simulating acute hepatitis. A case due to hepatic peripheral T-cell lymphoma.

The reactive hemophagocytic syndrome may mimic acute hepatitis, as we report here. It should be considered when hepatitis is accompanied by high fever, progressive cytopenias, and weight loss, with or without adenopathy and striking lactate dehydrogenase elevation. Bone marrow, lymph node, or liver biopsy are necessary to confirm the diagnosis. Recognition is important, because the syndrome has multiple potential causes amenable to therapy. Preliminary reports suggest that high-dose corticosteroid therapy is useful in suppressing cytokine-stimulated fever and hemophagocytosis, whatever the underlying cause.

Osseous Metaplasia in an Inflammatory Polyp of the Rectum: A Case Report and Review of the Literature

Osseous metaplasia is a rare finding in colonic neoplasms. We report a case osseous metaplasia in a 74 year-old male who underwent surveillance colonoscopy and found to have a 7 mm rectal polyp. Histopathologic examination revealed an inflammatory polyp with osseous metaplasia.

Obstructive jaundice due to a rare periampullary tumor

Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes. A 92-year-old female presented with obstructive jaundice. On exam she had pale conjunctiva and icteric sclera. Abdominal examination revealed tenderness in the upper abdomen. Laboratory data was consistent with obstructive jaundice. Computed tomography of the abdomen revealed a dilated gall bladder and a common bile duct (CBD) with no evidence of liver lesions or pancreatic head mass. Endoscopic ultrasonography revealed a 1 cm isoechoic submucosal nodule at the periampullary area, dilated CBD (9 mm), a prominent pancreatic duct (4.1 mm) and a hydropic gall bladder with no stones. Endoscopic retrograde cholangiopancreaticography was performed to relieve obstruction and showed a 1 cm periampullary mass which underwent an en-bloc snare resection. Histopathology analyses with immunohistochemical stains were positive for cytokeratin, synaptophysin, S-100 protein, neuron specific enolase and negative for actin and desmin consistent with periampullary gangliocytic paraganglioma. Periampullary gangliocytic paraganglioma is a rare benign tumor of the small bowel. Common presentation includes abdominal pain and obstructive jaundice which should be included in differential diagnosis of obstructive jaundice. Endoscopic resection is a curative therapy in the absence of local invasion or distant metastasis.

T1622 Hepatic Steatosis and Increase Risk of Hepatocellular Carcinoma in Mice Heterozygous for Long Chain 3-Hydorxy Acyl-CoA Dehydrogenase (LCHAD)

Objective: To improve hepatic ischemia-reperfusion (IR) injury of experimental fatty livers after treatment of steatosis with omega-3 fatty acids. Background: Major liver resections are associated with relatively high morbidity and mortality. One of the emerging risk factors for liver resections in theWesternworld is non-alcoholic fatty liver disease and chemotherapyassociated steatohepatitis. Fatty livers show a high susceptibility to IR injury and less regenerative capacity after major liver resection, leading to an increased risk of postoperative liver failure. Omega-3 lowers hepatic omega6:3 fatty-acids ratio resulting in decreased synthesis and increased hepatic fatty-acid degradation. Experimental studies have investigated prevention, but not reversal of steatosis by omega-3 fatty acids and the subsequent effect on hepatic IR injury. Methods: Steatosis was induced in male Wistar rats by feeding a methionine/ choline-deficient diet for 3 weeks. Rats were daily administered omega-3 (Omegaven), isocaloric control fatty acids (Lipofundin), or 0.9% NaCl (n=10/group) per gavage during 2 weeks. After treatment, rats were anesthetized and underwent partial (70%) hepatic ischemia for 40min. The non-ischemic lobes (30%) were resected prior to the reperfusion period. Blood was drawn at 20min, 6h, and 24h reperfusion, and at 6 and 24h rats (n=5/ group) were sacrificed for liver tissue extraction. Liver damage parameters (ALT) and bilirubin levels were determined from blood plasma. The histopathological steatosis degree was determined in the non-ischemic lobes, and total fatty acids content was assayed by gas chromatography. The extent of necrosis, cytokine levels, and total anti-oxidant capacity were determined at 6 and 24h reperfusion. Results: Omega-3 fatty acids significantly reduced the histopathological macrovesicular steatosis degree (mean%±SD): 30±15% omega-3 vs. 81±16% control fatty acids (p<0.01) and 79±16% NaCl (p<0.01). The total hepatic fatty acids and omega-6:3 ratios were significantly (p<0.01) lower in the omega-3 group. At 20min, 6h, and 24h reperfusion ALT release was significantly reduced (p<0.05), and at 6 and 24 h reperfusion bilirubin levels were significantly decreased (p<0.05) in omega-3treated livers. Furthermore, these livers revealed less necrosis and a decreased IL-6 and TNF-α content (p<0.05) at 24h reperfusion. Total antioxidant capacity was improved in omega-3-treated livers at 6 and 24h reperfusion. Conclusion: Omega-3 fatty acids treatment significantly decreases the hepatic steatosis degree in an experimental steatosis model, resulting in significantly decreased IR injury.