Familial rectal carcinoid: report of two first-degree relatives with rectal carcinoid and review of the literature.

Two brothers with familial carcinoid tumors of the rectum are presented. A few cases documenting the occurrence of carcinoid tumors in first-degree relatives in the absence of the multiple endocrine neoplasia (MEN) syndromes have been reported in the literature. Consistent with these previous reports, in this case both patients had gastrointestinal carcinoid tumors that are located in identical anatomic locations. The current literature on carcinoid tumors outside the setting of any known genetic syndrome is reviewed. Clinical relevance and screening recommendations are discussed.

Comparison of open and laparoscopic treatment of acute appendicitis.

BACKGROUND: Appendectomy can be performed using either a laparoscopic or an open technique. This study compares the outcome of patients treated for acute appendicitis by open appendectomy with the outcome of those undergoing laparoscopic appendectomy. METHODS: Patients undergoing appendectomy at The Mount Sinai Hospital between 1994 and 1998 were studied. Outcome of patients having open appendectomy was compared with that of patients having laparoscopic appendectomy. RESULTS: Seven hundred fifty-eight patients underwent appendectomy for acute appendicitis during the 5-year study period. Two hundred seventy-one (36%) had open appendectomy and 487 (64%) had laparoscopic appendectomy. Patients subsequently found to have a normal appendix had the highest rate of laparoscopic appendectomy, whereas those with gangrenous appendicitis were most likely to have open appendectomy (P <0.05). There was a significant decline in the postoperative length of stay for open cases during the length of the study. In the final year, the difference in length of stay between open and laparoscopic appendectomy was only 1 day. Patients with gangrenous appendicitis had a significantly longer length of stay than did patients with a normal appendix or suppurative appendicitis. The hospital cost of laparoscopic appendectomy was greater than that for open appendectomy but the extra expenditure in the operating room was offset by the longer length of stay of the patients having open surgery. CONCLUSIONS: Differences in outcome between open and laparoscopic appendectomy are minor. In this study, more difficult cases with gangrenous appendicitis were more likely to require open appendectomy whereas milder forms of appendicitis, especially in women, were more likely to be treated by laparoscopy. Savings from the slightly shorter hospital stay after laparoscopic appendectomy are offset by the higher surgical cost of the laparoscopic equipment.

Comparison of primary and reoperative surgery in patients with Crohns disease.

OBJECTIVE: This study was performed to determine the clinical results of patients with Crohns disease who require surgical resection. The outcome of patients undergoing initial surgery was compared with those having reoperation. METHODS: One hundred sixty-four patients undergoing intestinal resection for Crohns disease at The Mount Sinai Hospital from 1976 to 1989 were studied prospectively. The mean duration of follow-up was 72 months. RESULTS: Ninety patients (55%) underwent initial intestinal resection whereas 74 patients (45%) underwent reoperation for recurrent disease. Patients undergoing reoperation were older (33.4 vs. 38.7 years), had longer durations of disease (8.7 vs. 15.2 years), had shorter resections (60 vs. 46 cm), and were more likely to require ileostomy. Forty-seven percent of the patients with multiple previous resections required an ileostomy. This group also received a mean of 2.3 U blood in the perioperative period and showed a trend to increased symptomatic recurrence (49% vs. 71% at 5 years). CONCLUSIONS: Patients with Crohns disease undergoing first and second reoperation have outcomes similar to those in patients undergoing primary resection. Patients requiring multiple reoperations are more likely to require blood transfusions and permanent ileostomy and to show a greater trend to early symptomatic recurrence.

Colorectal cancer in familial adenomatous polyposis.

PURPOSE: This study was performed to determine the relationship among surgical treatment, colorectal cancer, and outcome in patients with familial adenomatous polyposis (FAP). METHODS: Records of 115 patients with FAP who underwent surgery at The Mount Sinai Medical Center between 1947 and 1994 were retrospectively reviewed. Patients without cancer were compared with those with colorectal cancer at initial surgery and with patients who developed rectal cancer following colectomy. RESULTS: Thirty-one patients (27 percent) had colorectal cancer at the time of initial surgery (colon = 24; rectal = 7). Another 11 patients (26 percent) developed rectal cancer after colectomy with ileorectal anastomosis (IRA). Mean age of patients with colorectal cancer at initial surgery was significantly higher than those without cancer (P < 0.01). Patients who developed rectal cancer after IRA were significantly older than patients with colorectal cancer at initial surgery (P < 0.01). All patients with rectal cancer after IRA had advanced disease with either nodal or distant metastases at the time of diagnosis. CONCLUSIONS: Colorectal cancer remains a major problem in the treatment of patients with FAP. Nearly one-fourth of these patients have colorectal cancer at initial operation, and one-fourth of patients with IRA develop rectal cancer after a mean follow-up of 13 years. Patients with rectal cancer following IRA are more likely to have advanced tumors than patients with colorectal cancer at initial operation. The high incidence and late stage of rectal cancer detected while under surveillance after IRA supports excision of the entire colorectal mucosa as the treatment of choice for most patients with FAP.

Colorectal adenocarcinoma in Crohn's disease.

OBJECTIVE: The authors' aim was to review the clinical features and estimate the long-term survival of patients with colorectal carcinoma complicating Crohn's disease. SUMMARY BACKGROUND DATA: Recent studies have demonstrated a significantly increased risk of colorectal carcinoma in patients with Crohns disease. METHODS: The authors reviewed retrospectively the medical records of 30 patients with Crohn's disease admitted to The Mount Sinai Hospital between 1960 and 1989 in whom colorectal adenocarcinoma developed. All patients were operated on and follow-up was complete for all patients to 10 years after operation, to the time of death, or to the closing date of the study in December 1989. RESULTS: The 30 patients in the series had 33 colorectal adenocarcinomas; three patients (10%) presented with two synchronous cancers. The patients were relatively young (mean age, 53 years) and had long-standing Crohn's disease (duration >20 years in 87%). The 5-year actuarial survival was 44% for the overall series: 100% for stage A, 86% for stage B, 60% for stage C. All five patients with excluded bowel tumor died of large bowel cancer within 2.4 years; by contrast, the actuarial 5-year survival for patients with in-continuity tumors was 56%. CONCLUSIONS: The incidence, characteristics, and prognosis of colorectal carcinoma complicating Crohn's disease are similar to the features of cancer in ulcerative colitis, including young age, multiple neoplasms, long duration of disease, and greater than a 50% 5-year survival rate (without excluded loops). These observations suggest the advisability of surveillance programs for Crohn's disease of the colon similar to those for ulcerative colitis of comparable duration and extent.

Prediction of early symptomatic recurrence after intestinal resection in Crohn's disease.

OBJECTIVE: This study was performed to identify clinical criteria that may help recognize patients with Crohn's disease who are at high risk for early symptomatic postoperative recurrence. SUMMARY BACKGROUND DATA: Currently, no reliable criteria are available to help recognize patients who are prone to experience early symptomatic recurrence. METHODS: One hundred sixty-four patients undergoing intestinal resection for Crohn's disease at the Mount Sinai Hospital between 1976 and 1989 were studied prospectively. Patients with symptomatic recurrent disease within 36 months were defined as having an early recurrence. RESULTS: Multivariate analysis revealed that the number of anastomoses was the most important prognostic indicator (p = 0.001), followed by inflammation at the resection margins (p < 0.05). Patients requiring an ileostomy had a significantly lower early recurrence rate than those having single or multiple anastomoses. There was no significant correlation between inflammation at the margins and early recurrence in patients requiring an ileostomy (n = 38), or a single anastomosis (n = 98). When the margins were examined in the 28 patients with 2 or more anastomoses, 10 of 11 patients (91%) with inflammation at either margin experienced early recurrence. Patients having multiple anastomoses with normal margins had the same recurrence rate as patients with single anastomosis (42%). CONCLUSIONS: Patients with extensive Crohn's disease requiring multiple resections with anastomosis, especially when microscopic inflammation is present at the margins, are at very high risk for symptomatic early recurrence. Ileostomy seems to be associated with a significantly lower early recurrence potential than anastomosis. Further study is needed to determine whether avoidance of multiple anastomosis and adjuvant medical treatment can alter the course of the disease after intestinal resection in patients at high risk for early symptomatic recurrence.

Surgical treatment of tumors of the distal rectum with sphincter preservation.

One hundred one patients with villous adenoma or invasive carcinoma of the distal rectum treated with local excision or coloanal anastomosis were studied. Twenty-three (45%) of the 51 patients with villous adenomas had transanal excision, another 23 (45%) had a posterior proctotomy, and five (10%) had a coloanal anastomosis. Only two patients with a villous adenoma developed a recurrence requiring repeat local excision. Fifteen (30%) of the 50 patients with invasive cancer were treated by transanal excision. All had tumors confined to the submucosa or superficial muscularis. Eighteen (85%) of 21 patients having posterior proctotomy also had tumors with similar depth of invasion. Six (43%) of the 14 patients having coloanal anastomosis had Dukes' B tumors, six (43%) were Dukes' C, and another two (14%) underwent palliative resection. The overall actuarial 5-year survival was 77%. Only four patients treated by transanal excision or posterior proctotomy died of metastatic disease. In the coloanal group, two of 12 patients undergoing curative resection died of recurrent cancer, and another has a pelvic recurrence. Villous adenomas of the distal rectum and selected carcinomas may be treated with local excision and coloanal anastomosis with preservation of sphincter function with good results.

Colorectal carcinoma associated with ulcerative colitis: a study of prognostic indicators.

Fifty-two patients with ulcerative colitis and colorectal cancer undergoing colectomy at the Mount Sinai Hospital between 1973 and 1988 were studied retrospectively to determine the correlation of age, sex, duration of colitis, tumor location, number of cancers, tumor differentiation, colloid content, presence of signet ring cells, Dukes' classification, and DNA ploidy with survival. The mean age was 45 years, with a mean duration of colitis of 21 years. Five patients (10%) had Dukes' A lesions, 17 (33%) had Dukes' B lesions, 17 (33%) had Dukes' C lesions, and 13 (25%) had distant metastases. Thirty patients (58%) had well- or moderately differentiated tumors, whereas tumors were poorly differentiated in 22 (42%). Twenty-eight patients (54%) had colloid tumors, and, in 14 (27%), signet ring cells were present. Thirty-one patients (60%) had nondiploid tumors. Actuarial analysis revealed that the 5-year survival rate was significantly worse for patients with nondiploid tumors (76% versus 32%). When stratified by stage, only patients with Dukes' C lesions showed a significant difference in survival for diploid versus nondiploid tumors. Multivariate analysis showed that the Dukes' classification was the best prognostic indicator, followed by tumor differentiation and DNA ploidy. Tumor location, colloid content, number of cancers, duration of disease, age, and sex did not correlate with the prognosis.

ras and c-myc protein expression in colorectal carcinoma. Study of cancer-prone patients.

This study was performed to determine the correlation of tumor ras and c-myc oncogene expression with clinical and prognostic variables in patients prone to develop colorectal cancer. One hundred eighteen patients with colorectal cancer were studied; mean age was 40 years. Fifty-three were young patients (age 40 or less), 49 had ulcerative colitis, and 16 had multiple polyposis coli. Immunoperoxidase stains of paraffin-embedded cancer sections were performed for the c-myc and ras proteins. ras staining was found to correlate with Dukes stage and prognosis. Patients with tumors negative for ras protein stain had an actuarial five-year survival of 61 percent versus 44 percent for those tumors with a positive stain (P less than 0.05). This correlation was not seen with the c-myc stain. Positive ras oncogene stain appears to be a useful indicator of advanced stage and poor prognosis in colorectal cancer occurring in cancer-prone patients.

DNA ploidy of colorectal cancer and synchronous polyps in polyposis coli.

Sixteen patients with polyposis coli and cancer were studied retrospectively to determine the incidence of DNA ploidy abnormalities in the tumors and synchronous polyps. Six patients (37 percent) had nondiploid tumors. Nondiploid tumors were more likely to be advanced and had a significantly worse prognosis (17 percent vs. 76 percent 5-year survival; P less than 0.01). Only 4 of 20 polyps studied were nondiploid. There was no association between tumor and polyp ploidy. All nondiploid polyps were found in patients with synchronous diploid cancers. Patients with nondiploid polyps were more likely to be older and have more advanced tumors than those with diploid polyps. DNA ploidy abnormalities seem to occur with the same frequency in polyposis coli as in the nonpolyposis population, and tumor ploidy correlates with prognosis.

Adenocarcinoma of the small intestine in Crohn's disease.

Nineteen patients admitted to The Mount Sinai Hospital with Crohn's disease between 1960 and 1989 had 20 adenocarcinomas of the small intestine. Sixteen patients had regional enteritis and three, ileocolitis. There were 15 males and four females. Carcinomas occurred in association with fistulas (four patients), fistulous tracts (three patients), excluded bowel (five patients/six cancers) and multiple strictures (three patients). None of the patients in our study had cancer develop in the first decade of Crohn's disease, and 11 had carcinoma in the third decade. As cancers occurred in three patients with multiple strictures admitted for strictureplasty, we recommend that all strictures be widely opened and carefully examined prior to strictureplasty, with frozen section biopsies of all suspicious areas. The possibility of small intestinal Crohn's carcinoma should be suspected in patients with long-standing disease, with or without excluded bowel, who present with sudden change in symptoms, especially after a lengthy quiescent period. Cancer should also be considered in patients in whom complete obstruction fails to resolve with adequate decompression and in those with multiple strictures.

Correlation of nuclear morphometry and DNA ploidy in rectal cancer.

Several investigators have used morphometric measurements to determine differences in the nuclear size and shape of normal and neoplastic colorectal tissue. Changes in nuclear morphometric parameters have also been shown to correlate with prognosis in a variety of noncolorectal cancers. The association of nuclear morphometry with prognostic indicators in rectal cancer has not been well studied. Measurements of the nuclear area, perimeter, longest cord, and circularity factor from 39 primary rectal adenocarcinomas were compared with DNA content, degree of tumor differentiation, Dukes' class, and patient survival. Nuclear circularity was found to correlate with DNA ploidy. Nondiploid tumors with a DNA index greater than 1.3 had significantly more circular nuclei than tumors with diploid or near-diploid DNA content. There was no correlation between nuclear morphometry and Dukes' class or patient survival. Significant increases in DNA content of rectal cancers appear to be reflected by measurable changes in nuclear shape. Nuclear morphometric measurements may provide useful information in the study of the progression of neoplastic changes in colorectal cancer.

Significance of DNA content abnormalities in small rectal cancers.

Several studies have shown that the presence of DNA content abnormalities, measured by flow cytometry, may correlate with a poor prognosis in a variety of cancers. The predictive value of DNA content in patients with small rectal cancers has not been well determined. Thirty-nine patients with primary rectal adenocarcinoma smaller than 3 cm were studied in a comparison of DNA content with established prognostic variables. The following covariates were evaluated for their prognostic value: sex, age, tumor size, location, distal margin, Dukes' classification, tumor differentiation, and DNA content. DNA content was assessed by flow cytometric analysis, and each tumor was categorized as diploid or nondiploid. Of the parameters studied, Dukes' classification and tumor DNA content were found to be independent prognostic indicators. Determination of DNA content seems to provide additional useful prognostic information in patients with small rectal tumors.

Ultrasonic mucosal proctectomy in patients with ulcerative colitis.

A technique for performing mucosal proctectomy in patients with ulcerative colitis using ultrasonic fragmentation is described. Twenty-eight patients undergoing colectomy and ileoanal anastomosis were studied. Removal of the mucosal layer of the distal rectum was performed using a titanium probe vibrating at 23 kHZ with an amplitude of 300 microns. This method produces complete mucosal destruction and the resulting debris and irrigating fluid is removed through the hollow central portion of the probe. Healing of the ileoanal anastomosis does not appear to be adversely affected by the use of this technique. Because ultrasonic fragmentation is not dependent on the integrity of the submucosal plane, it may be advantageous in those cases in which severe inflammation and submucosal scarring make manual dissection of the rectal mucosa difficult to perform.

Prognostic significance of DNA content abnormalities in young patients with colorectal cancer.

Several studies have shown that the presence of DNA ploidy abnormalities, measured by flow cytometry, may correlate with a poor prognosis in a variety of cancers. The predictive value of these DNA abnormalities in young patients with colorectal cancer has not been well studied. Fifty patients aged 40 years and younger with colorectal adenocarcinoma were studied to determine the correlation of tumor DNA abnormalities with survival. DNA content was determined by flow cytometric analysis and each tumor was categorized as diploid or nondiploid. Of the parameters studied, Dukes' classification and tumor DNA ploidy were found to be significant prognostic indicators. Determination of DNA content seems to provide additional useful prognostic information in young patients with colorectal cancer.

Clinical significance of rectal cancer in young patients.

Thirty-nine patients (age 40 years and younger) with rectal cancer treated at the Mount Sinai Hospital between 1967 and 1985 were studied. Their mean age was 34 years (range, 21 to 40). A positive family history for colorectal cancer was found in six patients (15 percent). Fifty percent of patients under age 30 had metastatic disease at diagnosis. Twenty-seven patients (69 percent) had potentially curative resections. Of these, 17 (63 percent) had lymph-node metastasis. This rate is twice as high as in a group of 315 patients with rectal cancer over age 40 (31 percent). The overall five-year survival for young patients having curative resection was 53 percent. Noncolorectal cancer occurred in three patients in this series and six patients also had first-degree relatives with noncolorectal cancer. Young patients with rectal cancer appear to belong to a high-risk cancer group which often seems to have a genetic pattern of predisposition.

Correlation of presence of granulomas with clinical and immunologic variables in Crohn's disease.

Approximately 50% of patients with Crohn's disease have epithelioid granulomas present in the diseased intestine. Some studies have associated the presence of granulomas with a good prognosis. In this prospective study, 44 patients with Crohn's disease requiring surgery were followed up for five years. Twenty-two patients (50%) had granulomas. Patients with granulomas were younger and had a shorter duration of disease. They also had more extensive disease and a greater degree of peripheral lymphopenia. Follow-up showed a trend toward greater recurrence rate in the patients with granulomas. It seems that patients with aggressive and extensive Crohn's disease are not protected from the development of symptomatic early recurrence by the presence of epithelioid granulomas.

Relationship of postoperative septic complications and blood transfusions in patients with Crohn's disease.

We prospectively studied 169 patients with Crohn's disease to determine if postoperative infectious complications could be related to perioperative blood transfusions. Postoperative septic complications developed in 18 of the 69 patients who received more than 1 unit of blood (26 percent) compared with 8 of the 100 patients (8 percent) who received 1 unit of blood or no blood (p = 0.0014). Previous operation, low body weight, and having an ostomy were also related to septic complications. Patients receiving more than 1 unit of blood were significantly more likely to have low preoperative serum albumin levels, to have undergone abdominoperineal or small bowel resection, and to have an ostomy. Postoperative septic complications were significantly related to perioperative blood transfusions after controlling for these potential confounding factors independently by subgrouping and simultaneously by using multiple logistic regression. Blood transfusion may be a more significant factor in postoperative immune suppression and susceptibility to infection than previously recognized.

Familial polyposis coli. Results of mucosal proctectomy with ileoanal anastomosis.

The outcome of mucosal proctectomy with ileoanal anastomosis in patients with polyposis coli has not been well studied. A series of 25 patients with polyposis treated at the Mount Sinai Hospital over a period of ten years is reported. The mean age of the patients was 23 years. Early postoperative complications were present in seven patients and consisted of thrombophlebitis (three), pelvic sepsis (three), and retraction of the anastomosis (one). Intestinal obstruction requiring laparotomy occurred in another five patients. Twenty-three patients were followed for a mean of 47 months after closure of the ileostomy. Ninety-one percent are satisfied with the operative results. The mean number of bowel movements per 24 hours is 6.0. All patients are continent, but eight have occasional episodes of rectal seepage at night. Nearly 50 percent require some antidiarrheal medication. New adenomatous polyps have developed just above the dentate line in four patients. Patients with polyposis coli seem to have fewer serious complications requiring excision of the ileoanal anastomosis than patients with ulcerative colitis. They also should have lifelong surveillance of the entire gastrointestinal tract even after total colectomy with ileoanal anastomosis.

Local recurrence following surgical treatment of rectal cancer. Comparison of anterior and abdominoperineal resection.

Three hundred twenty patients with rectal cancer were studied to determine factors that correlate with development of pelvic recurrence. The mean age was 65 years; anterior resection was performed in 202 (63 percent) and abdominoperineal resection in 118 (37 percent). Fifty-two patients (16 percent) developed pelvic recurrence. The mean duration of follow-up to development of pelvic recurrence was 22 months. Depth of tumor invasion, presence of lymph node metastasis, and colloid features were found to correlate with pelvic recurrence. The recurrence rate in patients having anterior resections was the same as that of patients undergoing abdominoperineal resections. Patients having anterior resection with distal margins of 1 cm or less had an extremely high recurrence rate (36 percent). Pelvic recurrent did not continue to improve when the distal margins were extended over 2 cm. Microscopic lateral tumor extension, which is not removed during operation, appears to be the major determinant of local recurrence in rectal cancer.