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James Church

Publications and source records attributed to James Church.

At least 19 recordsLinked to original sources
PubMed2006

ASCO/SSO review of current role of risk-reducing surgery in common hereditary cancer syndromes.

Although the etiology of solid cancers is multifactorial, with environmental and genetic factors playing a variable role, a significant portion of the burden of cancer is accounted for by a heritable component. Increasingly, the heritable component of cancer predispositions has been linked to mutations in specific genes, and clinical interventions have been formulated for mutation carriers within affected families. The primary interventions for mutations carriers for highly penetrant syndromes such as multiple endocrine neoplasias, familial adenomatous polyposis, hereditary nonpolyposis colon cancer, and hereditary breast and ovarian cancer syndromes are primarily surgical. For that reason, the American Society of Clinical Oncology (ASCO) and the Society of Surgical Oncology (SSO) have undertaken an educational effort within the oncology community. A joint ASCO/SSO Task Force was charged with presenting an educational symposium on the surgical management of hereditary cancer syndromes at the annual ASCO and SSO meetings, resulting in an educational position article on this topic. Both the content of the symposium and the article were developed as a consensus statement by the Task Force, with the intent of summarizing the current standard of care. This article is divided into four sections addressing breast, colorectal, ovarian and endometrial cancers, and multiple endocrine neoplasia. For each, a brief introduction on the genetics and natural history of the disease is provided, followed by a detailed description of modern surgical approaches, including a description of the clinical and genetic indications and timing of prophylactic surgery, and the efficacy of prophylactic surgery when known. Although a number of recent reviews have addressed the role of genetic testing for cancer susceptibility, including the richly illustrated Cancer Genetics and Cancer Predisposition Testing curriculum by the ASCO Cancer Genetics Working Group (available through http://www.asco.org), this article focuses on the issues surrounding the why, how, and when of surgical prophylaxis for inherited forms of cancer. This is a complex process, which requires a clear understanding of the natural history of the disease and variance of penetrance, a realistic appreciation of the potential benefit and risk of a risk-reducing procedure in a potentially otherwise healthy individual, the long-term sequelae of such surgical intervention, as well as the individual patient and family's perception of surgical risk and anticipated benefit.

Female↗
PubMed2006

A comparison of adverse events and functional outcomes after restorative proctocolectomy for familial adenomatous polyposis and ulcerative colitis.

PURPOSE: Restorative proctocolectomy is the procedure of choice for patients undergoing proctocolectomy for familial adenomatous polyposis or ulcerative colitis. This meta-analysis was designed to identify differences in adverse events and functional outcomes between these two groups. METHODS: Studies published between 1986 and 2003 that compared outcomes between patients with familial adenomatous polyposis and ulcerative colitis were included. Meta-analytical techniques using random effect models were used to compare short-term and long-term adverse events as well as functional outcomes between the groups. RESULTS: Nineteen studies comprising 5,199 patients (familial adenomatous polyposis, 782; ulcerative colitis, 4,417) were analyzed. There were no significant differences in immediate postoperative adverse events between the two groups. Pouch-related fistulation was significantly increased in the ulcerative colitis group (10.5 percent vs. familial adenomatous polyposis 4.8 percent; odds ratio 2.31; P < 0.001). There was no significant difference in pouch failure between the two groups (ulcerative colitis 5.8 percent vs. familial adenomatous polyposis 4.5 percent; odds ratio 1.22; P = 0.43). The incidence of pouchitis was significantly greater in the ulcerative colitis group (30.1 vs. 5.5 percent; odds ratio 6.44; P < 0.001). Patients with familial adenomatous polyposis had a significant advantage in stool frequency with one less motion per 24 hours (95 percent confidence interval, 0.21-1.76; P = 0.01). CONCLUSIONS: In contrast to studies reporting similar outcomes for patients undergoing restorative proctocolectomy for familial adenomatous polyposis or ulcerative colitis, the present meta-analysis suggested that patients with ulcerative colitis are at greater risk of pouch-related fistulation and pouchitis. Although there was an increase in the 24-hour stool frequency in the ulcerative colitis group, this may be accounted for by the younger age at surgery in the familial adenomatous polyposis group.

Adenomatous Polyposis Coli↗
PubMed2006

ASCO/SSO review of current role of risk-reducing surgery in common hereditary cancer syndromes.

BACKGROUND: A significant portion of cancers are accounted for by a heritable component, which has increasingly been linked to mutations in specific genes. Clinical interventions have been formulated for mutation carriers within affected families. The primary interventions for mutation carriers of highly penetrant syndromes are surgical. METHODS: The American Society of Clinical Oncology and the Society of Surgical Oncology formed a task force charged with presenting an educational symposium on surgical management of hereditary cancer syndromes at annual society meetings, and this resulted in a position paper on this topic. The content of both the symposium and the position paper was developed as a consensus statement. RESULTS: This article addresses hereditary breast, colorectal, ovarian/endometrial, and multiple endocrine neoplasias. A brief introduction on the genetics and natural history of each disease is provided, followed by detailed descriptions of modern surgical approaches, clinical and genetic indications, timing of prophylactic surgery, and the efficacy of surgery (when known). Although several recent reviews have addressed the role of genetic testing for cancer susceptibility, this article focuses on the issues surrounding surgical technique, timing, and indications for surgical prophylaxis. CONCLUSIONS: Risk-reducing surgical treatment of hereditary cancer is a complex undertaking. It requires a clear understanding of the natural history of the disease, realistic appreciation of the potential benefits and risks of these procedures in potentially otherwise healthy individuals, and the long-term sequelae of such interventions, as well as the individual patient's and family's perceptions of surgical risk and anticipated benefit.

Female↗
PubMed2005

Practice parameters for the management of rectal cancer (revised).

The American Society of Colon and Rectal Surgeons is dedicated to assuring high-quality patient care by advancing the science, prevention, and management of disorders and diseases of the colon, rectum, and anus. The Standards Committee is composed of Society members who are chosen because they have demonstrated expertise in the specialty of colon and rectal surgery. This Committee was created to lead international efforts in defining quality care for conditions related to the colon, rectum, and anus. This is accompanied by developing Clinical Practice Guidelines based on the best available evidence. These guidelines are inclusive, and not prescriptive. Their purpose is to provide information on which decisions can be made, rather than dictate a specific form of treatment. These guidelines are intended for the use of all practitioners, health care workers, and patients who desire information about the management of the conditions addressed by the topics covered in these guidelines. It should be recognized that these guidelines should not be deemed inclusive of all proper methods of care or exclusive of methods of care reasonably directed to obtaining the same results. The ultimate judgment regarding the propriety of any specific procedure must be made by the physician in light of all of the circumstances presented by the individual patient.

Adenocarcinoma↗
PubMed2005

Sphincter defects are not associated with long-term incontinence following ileal pouch-anal anastomosis.

INTRODUCTION: Frequent loose stools test the integrity of sphincter function in patients undergoing ileal pouch-anal anastomosis. The authors hypothesized that women with anal sphincter defects were more likely to experience incontinence episodes than women with intact sphincter muscles following ileal pouch-anal anastomosis. METHODS: From 1996 to 1998, 42 women with a mean age of 42 (range, 22-63) years were prospectively evaluated by anorectal manometry and endoanal ultrasound before pouch surgery. Forty women underwent a stapled ileal pouch-anal anastomosis and two underwent a handsewn anastomosis. All patients considered themselves continent of stool before the procedure. A postoperative survey including the Cleveland Clinic Florida scale, Fecal Incontinence Severity Index, and Fecal Incontinence Quality of Life scale was sent to study participants. RESULTS: Nineteen women with an obstetrical history had significant sphincter defects associated with significant lower mean resting pressure, mean squeeze pressure, and shorter anal canal length (3 vs. 3.7 cm, P = 0.0007). Thirty-five women (83 percent) responded resulting in a mean follow-up of 62 (range, 49-72) months. Fourteen responders (mean age, 46 years) had sphincter defects but no significant difference was found in Cleveland Clinic Florida scale, Fecal Incontinence Severity Index, or Fecal Incontinence Quality of Life scale scores when compared with those without defects. CONCLUSION: Although almost all women reported episodes of seepage, marked sphincter defects associated with low anal pressures and shorter anal canal length did not affect anal function following pouch surgery. This study supports the findings that continent women with significant sphincter defects on ultrasound evaluation may be considered for restorative proctocolectomy.

Adult↗
PubMed2005

The quality of podium presentations at the american society of colon and rectal surgeons: does a decade make a difference?

PURPOSE: The technology of the seven-minute scientific presentation has changed greatly with the universal acceptance of computerized slides and graphics. We performed this study to see whether the quality of delivery has improved pari passu with technical advances in presentation of data. METHODS: In 1993 and in 2003 a sample of the podium presentations given at The American Society of Colon and Rectal Surgeons annual scientific meeting was analyzed. Number of slides, type of slides, time of presentation, and quality of presentation were recorded. Quality was scored by the presence of poor presentation techniques such as presenting too fast for the slide to be comprehended, presenting data that were different to what was on the slide, using fonts that were too small to be read, and reading the exact wording of the slide. One point was awarded for each transgression and for any talk lasting more than 7.9 minutes; the higher the score, the worse the presentation. RESULTS: Thirty-eight presentations were analyzed from the 1993 meeting in Orlando and 40 were analyzed from the 2003 meeting in New Orleans. There was no difference in mean time of the presentation (1993, 7.5 minutes +/- 1.5 standard deviation vs. 2003, 7.2 minutes +/- 0.7 standard deviation), in mean number of slides used per presentation (1993, 16.4 +/- 3.3 standard deviation vs. 2003, 17.1 +/- 5.4 standard deviation), or in slides per minute (1993, 2.3 +/- 0.8 standard deviation vs. 2003, 2.5 +/- 1.0 standard deviation). Presentation technique was much worse in 1993, however, with a mean quality score of 2.2 +/- 1.1 standard deviation vs. 0.8 +/- 0.9 standard deviation in 2003 (P < 0.001). The most common fault in both years was presenting a slide too rapidly, a fault more pervasive in 1993 than in 2003 (1993, 30/38 presentations vs. 2003, 16/40). Only ten presentations went over the allotted time in 2003, by an average of one minute. In 1993, 18 presentations were too long, by an average of 1.8 minutes each. CONCLUSIONS: The basic structure of the seven-minute podium presentation has changed little over the last ten years, despite major advances in technology. The most notable improvements have been in the technique of delivering the talk.

Audiovisual Aids↗
PubMed2005

Staging intra-abdominal desmoid tumors in familial adenomatous polyposis: a search for a uniform approach to a troubling disease.

INTRODUCTION: Desmoid tumors are a clinical problem in 12 to 15 percent of patients with familial adenomatous polyposis. There is no predictably effective treatment for intra-abdominal desmoid tumors, which sometimes cause significant complications by their effects on the ureters or bowel. The relative rarity and the clinical heterogeneity of intra-abdominal desmoid tumors make randomized studies difficult to do. In this article a staging system is proposed to make multi-institutional studies easier. METHODS: Intra-abdominal desmoid tumors can be staged according to their size, clinical presentation and growth pattern. CONCLUSION: A way of staging intra-abdominal desmoid tumors is proposed to facilitate stratification by disease severity during collaborative studies of various treatments.

Abdominal Neoplasms↗
PubMed2005

Ileoanal pouch neoplasia in familial adenomatous polyposis: an underestimated threat.

PURPOSE: Ileal pouch-anal anastomosis is one of the two main options available for the surgical treatment of patients with familial adenomatous polyposis. Its main advantage is the minimal risk of rectal cancer but a possible Achilles heel is the recurrence of epithelial neoplasia at the ileal pouch-anal anastomosis and within the ileal pouch. The significance of ileoanal anastomotic and ileal pouch adenomas is not yet fully appreciated, and there is a false sense of security about this operation. The consequences of worsening pouch polyposis are serious in that endoscopic treatment is unlikely to be an effective way of controlling it. This study has been done to alert those caring for patients with familial adenomatous polyposis to the looming danger of pouch polyposis and to suggest ways to deal with it. METHODS: Studies reporting ileoanal pouch adenomas, ileal pouch-anal anastomotic cancers, and ileal pouch cancers in patients with familial adenomatous polyposis were reviewed. Reports of adenomas in Kock pouches and in Brooke ileostomies in the setting of familial adenomatous polyposis were included. The primary end points of the study were the time between pouch construction and the diagnosis of neoplasia, the age of the patients at the diagnosis of neoplasia, and the severity of the neoplasia. RESULTS: There were 18 studies reporting pouch neoplasia, 15 with adenomas, and 3 with cancer. Ten were case reports, five were retrospective studies, and three were prospective studies. All three prospective studies showed that the incidence of pouch adenomas increases with time of follow-up and that the severity of the polyposis varies. The median time from pouch construction to diagnosis of pouch adenomas was 4.7 years and the range was 0.5 to 12 years. There were six studies reporting eight patients with cancer at the ileal pouch-anal anastomosis, diagnosed a median of 8 years after pouch construction (range, 3-20 years). One-half of the cancers were locally advanced (T4) and one-half were not (T1 or T2). One-half followed stapled anastomosis and one-half were after mucosectomy. There were eight case reports of cancer described in an ileostomy in patients with familial adenomatous polyposis. The median time from ileostomy construction to the ileostomy cancers was 25 (range, 9-40) years. DISCUSSION: The combination of fecal stasis, adenomatous epithelium, and a germline APC mutation is a potent recipe for epithelial neoplasia. There is increasing evidence that this happens in an ileostomy but that the process is much faster in an ileal pouch. Endoscopic treatment of ileal adenomas is likely to be difficult, reducing the options for their control to excising the entire pouch or chemoprevention.

Adenoma↗
PubMed2005

Pancreas-sparing duodenectomy is effective management for familial adenomatous polyposis.

Duodenal adenocarcinoma remains the leading cause of cancer death in familial adenomatous polyposis patients following colectomy. Stratification based on Spigelman's criteria provides a means for determining therapy. Spigelman stage IV patients have been selected for pancreas-sparing duodenectomy. Twenty-one patients underwent resection between 1992 and 2004, with a mean age of 58 +/- 11 years. The mean time from colectomy to duodenectomy was 27 +/- 13 years. Invasive cancer was found in the distal duodenum in one patient. Operative time averaged 327 +/- 61 minutes with a mean blood loss of 503 +/- 266 ml. There was no mortality, and eight patients (38%) had 14 complications: six (29%) with delayed gastric emptying, four (19%) with biliary/pancreatic anastomotic leak, one with pancreatitis, and one with wound infection. There were two reoperations: one for delayed gastric emptying and one for an early biliary leak. Mean length of stay was 15 +/- 10 days. Two late complications occurred: a stomal ulcer and an intestinal obstruction at 48 and 24 months, respectively. Mean follow-up was 79 months (range, 3-152 months). Two patients developed polyps in the advanced jejunal limb and were endoscopically treated. Pancreas-sparing duodenectomy represents a definitive treatment for advanced duodenal polyposis and can obviate the need for pancreaticoduodenectomy.

Adenomatous Polyposis Coli↗
PubMed2005

The utility of capsule endoscopy small bowel surveillance in patients with polyposis.

BACKGROUND: Small intestinal (SI) surveillance is recommended for polyposis patients. The utility and safety of capsule endoscopy (CE) for surveillance of SI neoplasia in patients with familial adenomatous polyposis (FAP) and Peutz-Jeghers syndrome (PJS) is unknown. METHODS: CE was offered to consecutive FAP and PJS patients due for routine upper endoscopic surveillance. The prevalence, location (jejunum, ileum), size (1-5 mm, 6-10 mm, >10 mm) and number (1-5, 6-12, >20) of polyps detected by CE was assessed. RESULTS: 19 subjects (15 FAP/4 PJS) with a mean age of 43 were included. All subjects had previous intestinal surgery. No complications occurred with CE. CE in FAP: 9/15 (60%) of subjects with FAP had SI polyps. The prevalence of SI polyps was related to the duodenal polyposis stage and subject age. The location, size and number of polyps progressed as duodenal polyposis stage advanced. CE in PJS: 3/4 (75%) of subjects with PJS had SI polyps. The polyps were diffuse in 2/4 and only in the ileum in one subject. CE findings led to laparotomy with intra-operative endoscopic polypectomy in two PJS patients. CONCLUSION: SI polyps are common in FAP but their importance is unknown. CE should be performed in FAP patients with stage III and IV duodenal disease. Clinically significant polyps are commonly detected by CE in PJS and lead to change in management in 50% of PJS subjects. CE should replace radiographic SI surveillance for PJS patients. CE is safe in polyposis patients who have undergone major intestinal surgery.

Adenomatous Polyposis Coli↗
PubMed2004

Inherited colorectal cancer registries in the United States.

PURPOSE: The prevalence of familial adenomatous polyposis-associated cancers in the United States has been conservatively estimated at 15,000, and that of hereditary nonpolyposis colorectal cancer (HNPCC)-associated cancers at 30,000. Every blood descendant of each of these patients is at risk of carrying a germline mutation predisposing them to early onset colorectal and other cancers. Optimal care of these high-risk families involves a center with expertise in the syndromes. This study was performed to see how many such centers exist in the United States and to learn something of how they work. METHODS: The mailing lists of three international societies concerned with inherited colorectal cancer were used to send surveys inquiring about the presence of a registry or center, and how that center worked. The Collaborative Group of the Americas, the Leeds Castle Polyposis Group, and the International Collaborative Group on hereditary nonpolyposis colorectal cancer were queried. RESULTS: There were 30 centers in the United States: 26 responded, representing 15 states. Eighteen centers that responded had registries for inherited colorectal cancer. There were 1,396 familial adenomatous polyposis families among the 18 registries, 2,058 hereditary nonpolyposis colorectal cancer families, 42 with juvenile polyposis, and 216 with Peutz-Jehger's syndrome. The 18 registries employed 29 genetic counselors or coordinators. Seven used Microsoft Access as a database, five used Progeny, three a SQL server-based system, one Filemaker Pro, one Microsoft Excel and one used Oracle. Cyrillic was the pedigree-drawing program in 6 registries, Progeny in 12, and Ped Draw in 1. Hereditary nonpolyposis colorectal cancer was defined using the Amsterdam I criteria by four registries, Amsterdam II by five, both criteria by six, Bethesda guidelines by one, and by genotype alone in two registries. CONCLUSIONS: The United States is underserved by registries for inherited colorectal cancer, having enrolled only a small proportion of the families theoretically available. Registries differ in fundamental aspects of function. More collaboration and more registries are needed.

Colorectal Neoplasms↗
PubMed2003

Patterns of surgery in patients belonging to amsterdam-positive families.

INTRODUCTION: The phenotype of hereditary nonpolyposis colorectal cancer includes an 80 percent lifetime risk of colorectal cancer, a predominance of lesions proximal to the splenic flexure, and a high incidence of synchronous and metachronous neoplasia. Although prophylactic colectomy is rarely advised for patients with a hereditary nonpolyposis colorectal cancer genotype and a normal colon, the presence of advanced neoplasia in the context of a qualifying family history or a hereditary nonpolyposis colorectal cancer genotype has led to such recommendations. We performed this study to document the patterns of colorectal surgery performed for cancer-bearing patients who are part of an Amsterdam criteria-positive family and to compare rates of metachronous cancers that follow index total or segmental colectomy. METHODS: Family trees fulfilling the classic Amsterdam criteria for hereditary nonpolyposis colorectal cancer were identified, and all patients for whom surgical and pathology records were available were included in the study. Type of surgery and the outcome of subsequent follow-up were abstracted. Patients were divided into those treated at the Cleveland Clinic and those treated elsewhere. RESULTS: There were 39 families with 93 affected patients. These patients had 127 colorectal cancers, 76 (60 percent) of which were right sided (proximal to the splenic flexure). Median age at diagnosis of the index cancer was 47 (range, 26-81) years. Sixteen patients (17 percent) had metachronous cancers and multiple surgeries, whereas four (4 percent) had synchronous cancers. Median follow-up for patients who underwent surgery at the Cleveland Clinic was 13 (range, 4-49) years, whereas that for those who underwent surgery elsewhere was 14 (range, 1-42) years. Sixteen (48 percent) of the 33 patients who underwent surgery at the Cleveland Clinic had a total colectomy vs. 7 (12 percent) of the 60 who had surgery elsewhere (Fisher's exact test, P < 0.001). Only one patient who had surgery at the Cleveland Clinic had a second operation for a metachronous cancer (1/17 patients having a segmental resection). Fifteen patients who underwent surgery elsewhere needed a second resection for metachronous cancer (15/53 patients having a segmental resection; Fisher's exact test, P = 0.094). CONCLUSION: We conclude that there is high risk of metachronous colorectal cancer if an index cancer in a hereditary nonpolyposis colorectal cancer patient (defined according to Amsterdam criteria) is treated by partial colectomy. However, this risk can be lowered by performing a total colectomy at the time of index surgery, or possibly by effective postoperative surveillance.

Adult↗