Primary Signet Ring Cell Carcinoma of the Ileal Pouch.

A 55-year-old man with a history of ulcerative colitis status post ileal pouch-anal anastomosis underwent evaluation for pouchitis. He was found to have signet ring cell cancer within the pouch. Primary cancers of the ileal pouch are rare. Signet ring cell carcinoma is a rare form of adenocarcinoma that can involve the gastrointestinal tract and other organs. We present a case of a signet ring cell carcinoma arising from an ileal pouch.

Beyond Squamous Cell Carcinoma: Basaloid Squamous Cell Carcinoma of the Esophagus.

Basaloid squamous cell carcinoma (BSCC) is a poorly differentiated variant of squamous cell carcinoma (SCC) with distinct morphologic characteristics. Yet, there are no clearly defined guidelines established for management. BSCC in the esophagus is a very rare entity, with the proportion of esophageal BSCC ranging from 0.068% to 11%. This wide range is thought to be secondary to difficulty making the diagnosis on small biopsy specimens and the lack of a universally defined proportion of BSCC components necessary to make the diagnosis. We present the case of a 57-year-old African American female, who underwent esophagogastroduodenoscopy (EGD) after an abnormal barium swallow in the setting of two months history of dysphagia and weight loss and was diagnosed with BSCC of the esophagus on histopathology.

Serrated Polyposis Syndrome: Increasing Awareness and Importance.

Serrated polyposis syndrome (SPS) was formerly considered a rare condition. In the past decade, it has gained increasing recognition due to its close association with colorectal cancer (CRC). Diagnosis is made based on the updated World Health Organization (WHO) criteria of having serrated polyps (SPs) proximal to the rectum, all being ≥5 mm in size, with at least two being ≥10 mm in size (criterion I), and a more distal phenotype that presents with greater than 20 SPs of any size throughout the large bowel with five being proximal to the rectum (criterion II). There are three subtypes of SP: hyperplastic polyp (HP), sessile serrated lesion (SSL), and traditional serrated adenoma (TSA). We present a 61-year-old Caucasian male who was referred for surveillance colonoscopy due to a history of colon polyps. A total of 28 polyps were completely removed, 21 of which were found to be SPs, three of which were >10 mm in size, meeting the WHO criteria for SPS. A follow-up colonoscopy was recommended in one year. It is now recognized that SPS are significant contributors to the development of CRC. The United States Multi-Society Preventive Task Force recently updated their consensus statement in 2020 with specific guidance for surveillance of SPs. It is important to emphasize that the diagnostic criteria apply to cumulative polyp count over the individual's lifetime. The optimal surveillance for SPS remains unclear.

Anorectal Disorders: An Update.

Anorectal disorders encompass structural, neuromuscular, and functional disorders. They are common, often distressing, and in some cases debilitating, and significantly add to the health care burden. They present with multiple, overlapping symptoms that can often obscure the underlying pathology and can pose significant diagnostic and management dilemmas. A meticulous history and comprehensive digital rectal examination can provide clarity on the diagnosis, appropriate testing, and management of these conditions. Today, with the development of sophisticated diagnostic tools such as high-resolution and high-definition (3-D) anorectal manometry, 3-D anal ultrasonography, magnetic resonance defecography and imaging, and neurophysiological tests such as translumbosacral anorectal magnetic stimulation, it is possible to more accurately define and characterize the underlying structural and functional abnormalities. In this review, we present a succinct update on the latest knowledge with regards to the pathophysiology, diagnosis and management of anal fissure, hemorrhoids, rectal prolapse, intussusception, rectocele, solitary rectal ulcer syndrome, levator ani syndrome, dyssynergic defecation and fecal incontinence.

Racial Disparities in Clinical Presentation and Survival Times Among Young-Onset Colorectal Adenocarcinoma.

BACKGROUND: Recently published data indicate increasing incidence of colorectal adenocarcinoma (CRC) in young-onset (<50 years) patients. AIMS: This study examines racial disparities in presentation and survival times among non-Hispanic Blacks (NHB) and Hispanics compared with non-Hispanic Whites (NHW). METHODS: A retrospective single-center cohort study was conducted from 2004 through 2014 using 96 patient medical charts with a diagnosis of young-onset CRC. Age, gender, primary site, and histological stage at the time of diagnosis were assessed for survival probabilities by racial group over a minimum follow-up period of 5 years. RESULTS: Among subjects with CRC diagnosis before 50 years of age, the majority of subjects were between 40 and 50 years, with CRC presentation occurring among this age group for 51 (79.7%) of NHW, 18 (81.8%) of NHB, and 5 (50.0%) of Hispanics. The majority of all patients presented with advanced stages of CRC (31.3% with stage III and 27.1% with stage IV). NHB exhibited statistically significantly worse survival compared to NHW (adjusted hazard ratio for death = 2.09; 95% confidence interval 1.14-3.84; P = 0.02). A possible trend of worse survival was identified for Hispanics compared to NHW, but this group was low in numbers and results were not statistically significant. CONCLUSION: Disparities between racial groups among young-onset CRC cases were identified in overall survival and reflect growing concern in rising incidence and differentiated care management.

Mirrizi Syndrome and Markedly Elevated Levels of Carbohydrate Antigen 19-9 in the Absence of Malignant Disease.

Elevated carbohydrate antigen 19-9 (CA19-9) beyond 1000 U/L occurs in nonneoplastic conditions which is causing questioning of the use of CA19-9 as a marker for screening. We report a case where a 51-year-old male with Mirrizi Syndrome (MS) presented with markedly increased CA19-9 level (4,618 U/mL). MS is a rare complication characterized by compression of the common bile or hepatic duct caused by an impacted gallstone in the cystic duct or neck of the gallbladder. Biliary epithelial cells secrete CA19-9: it is hypothesized that increased proliferation of such cells caused by inflammation leads to increased secretion. CA19-9 should not be used as a diagnostic tool, but rather for surveillance.

Klippel-Trenaunay Syndrome of the Rectosigmoid Colon Presenting as Severe Anemia.

A 23-year-old female with Klippel-Trenaunay syndrome presented with abdominal pain and severe anemia. Colonoscopy revealed diffuse venous congestion extending circumferentially from the midsigmoid to the rectum, with multiple large varicosities. This case emphasizes that Klippel-Trenaunay syndrome may have visceral manifestations beyond the classic presentation, which can be a significant source of morbidity and mortality.

Ulcerative Colitis in Colonic Interposition for Esophageal Atresia.

A 38-year-old male with a history of colonic interposition for esophageal atresia as an infant presented with dysphagia and abdominal pain. On the basis of endoscopy findings, pathology, and response to therapy, he was found to have ulcerative colitis of the colonic conduit.

Racial Disparities in Colorectal Carcinoma Incidence, Severity and Survival Times Over 10 Years: A Retrospective Single Center Study.

BACKGROUND: Colorectal cancer (CRC) is the third leading cause of cancer-related deaths in the United States. Although studies have been performed on malignancy behavior in African Americans and Caucasians, scant data are present on other minority racial groups. METHODS: A retrospective single center study was performed where 1,860 patient charts with a diagnosis of CRC from January 1, 2004 to December 31, 2014 were reviewed. Data collected on each patient included age, gender, ethnicity, primary site and histological stage at the time of diagnosis. Survival time over the course of 5 years was documented for patients from January 1, 2004 to December 31, 2009. Comparisons were made amongst different racial groups for the above mentioned factors. RESULTS: Study population consisted of 27.09% African Americans, 65.61% Caucasians, 3.86% Hispanics, 0.54% South Asians, 1.03% Arabs, 0.54% Asians and 0.22% American Indians. Mean age of CRC presentation was found to be significantly different (P < 0.05) between the three largest racial groups: 71 years for Caucasians, 69 years for African Americans, and 61 years for Hispanics. African Americans (27.09%) and Hispanics (28.79%) presented predominantly at stage IV in comparison to other racial groups. Caucasians presented predominantly at stage III (24.84%). The rectum was the most common site of CRC across all racial groups with the exception of Asians, where sigmoid colon was the predominant site (30%). Adenocarcinoma remained the predominant cancer type in all groups. Hispanics had relatively higher incidence rate of carcinoid tumor (12.68%). Survival time analysis showed that Caucasians tend to have better survival probability over 5 years after initial diagnosis as compared to African Americans and Hispanic (P < 0.05). CONCLUSION: There is lack of studies performed on minority racial groups in North America. Our study highlighted some important clinical differences of CRC presentation in different racial groups which are not well studied and can be used to formulate future multi-center studies to assess disease behavior.

Markedly Elevated Liver Enzymes in Choledocholithiasis in the absence of Hepatocellular Disease: Case Series and Literature Review.

Liver enzyme levels are commonly obtained in the evaluation of many conditions. Elevated alanine transaminase and aspartate transaminase have traditionally been considered a "hepatocellular" pattern concerning for ischemic, viral, or toxic hepatitis. Elevations in these levels pose a diagnostic dilemma in patients without a clinical picture consistent with liver disease. On the other hand, elevated alkaline phosphatase historically represents a "cholestatic" pattern concerning for gallbladder and biliary tract disease. Often, patients present with a "mixed" picture of elevation in all 3 liver enzymes, further confounding the clinical scenario. We present 4 cases of women with severe upper abdominal pain and markedly elevated transaminases. Three of the patients had accompanying jaundice. A higher rise in enzyme levels was seen in those who had greater bile duct dilation. All patients saw a rapid decrease in transaminases after biliary decompression, along with a fall in alkaline phosphatase and total bilirubin levels. No evidence of liver disease was found, nor were there any signs of hepatocellular disease on imaging. The patients were ultimately found to have choledocholithiasis on endoscopic retrograde cholangiopancreatography with no hepatocellular disease. Furthermore, our cases show that severe abdominal pain in the setting of elevated liver enzymes is likely associated with biliary disease rather than a primary hepatic process. Recognition of this rare pattern of markedly elevated transaminases in isolated biliary disease can aid in avoiding unnecessary evaluation of primary hepatic disease and invasive surgical interventions such as liver biopsy.