Risk-reducing surgery in FAP: role for surgeons beyond the incision.

BACKGROUND: Although primary therapy in familial adenomatous polyposis (FAP) is surgical, little is known about patients' surgical decision-making experience. The objective was to explore the decision-making process surrounding risk-reducing surgery in FAP using qualitative methodology. METHODS: In-depth, semi-structured interviews with 14 FAP patients and 11 healthcare providers with experience caring for FAP patients were conducted. Using grounded theory, line-by-line content analysis identified categories from which themes describing patients' experiences emerged; analysis continued until data saturation. RESULTS: Median age at surgery was 23 (7-37) years; at interview 41 (19-74) years. Two patients underwent surgery secondary to cancer, the remainder for risk-reduction. Content experts included colorectal surgeons (3), geneticists (2), gastroenterologists (3), nurses (3).Three themes emerged: Information: Family was the primary information source, and patients' level of information varied. The importance of up-front information was emphasized. Influences on decision-making: Influential factors included family experiences, youth, emotional state, support, and decision-making role. Although patients often sought opinions, most (12/14) wanted an active/shared role in decision-making. Life after surgery: Patients described surgery as the "easy part," emphasizing the need for long-term relationships with care providers. CONCLUSIONS: Decisions surrounding risk-reducing surgery in FAP are unique. A decision support tool may facilitate decision-making, better preparing patients for life after surgery.

Young age influences treatment but not outcome of colon cancer.

BACKGROUND: Early age at onset is often considered a poor prognostic factor for colon cancer. The aim of this study was to determine the association between age, clinicopathologic features, adjuvant therapy, and outcomes following colon cancer resection. METHODS: A prospective database of 1,327 surgical stage I-III colon cancer patients operated on from 1990-2001 was evaluated, and patients grouped by age. RESULTS: Sixty-eight patients (5%) were diagnosed at age 40 (older). Younger patients were more likely to have left-sided tumors (66% vs 51%, P = .02), but no more likely to present with symptomatic lesions, more advanced tumors, or have worse pathologic features. Younger patients were noted to have more nodes retrieved in their surgical specimens than older patients (median 18 vs 14, P = .001), although the numbers of total colectomies were similar in both groups. Younger patients were also more likely to receive adjuvant chemotherapy, and this was most pronounced in the stage II cohort: 39% vs 14%, P = .003. With a median follow-up of 55 months, 5-year disease-specific survival (DSS) was similar in both study groups: 86% vs 87%, but 5-year overall survival (OS) was significantly higher in the younger patient cohort (84% vs 73%, P = .001). CONCLUSION: Younger patients undergoing complete resection of stage I-III colon cancer had DSS similar to older patients. However, younger patients had more nodes retrieved from their specimens and were more likely to receive adjuvant therapy, especially for node-negative disease. These factors may have contributed to their overall favorable outcome.

Neuroendocrine carcinomas of the colon and rectum.

PURPOSE: This study was designed to review experience with neuroendocrine carcinomas of the colon and rectum at a single institution, with emphasis on the pathology and clinical characteristics of this uncommon malignancy. METHODS: A study group of patients was identified from a prospective colorectal service database. Pathology was reviewed and neuroendocrine tumors were classified by a single pathologist. Medical records were retrospectively reviewed. RESULTS: From March 1975 to September 1998, 38 patients with neuroendocrine carcinomas were identified from the colorectal service database comprising 6495 patients (0.6 percent). These neuroendocrine carcinomas did not include carcinoid tumors. Average patient age was 57 years (range, 29-86 years). There were 17 males (44.7 percent) and 21 females (55.3 percent). Tumors were located as follows: 17 colon, 14 rectum, 6 anal canal, and 1 appendix. The diagnosis of neuroendocrine carcinoma was suggested preoperatively from tissue biopsy in 59.3 percent (16/27) of patients evaluable. Pathology was reviewed and tumors were categorized as small cell carcinoma (n = 22) or large cell neuroendocrine carcinoma (n = 16). Most tumors (20/25 evaluable, 80 percent) stained positive by means of immunohistochemistry for neuroendocrine markers, including chromogranin (18/19), synaptophysin (10/15), and/or neuron-specific enolase (14/15). Metastatic disease was detected at the time of diagnosis in 69.4 percent of the patients (25/36). Tumors were advanced at the time of diagnosis, with American Joint Committee on Cancer (AJCC) Stage I (n = 6), Stage III (n = 7), and Stage IV (n = 25) tumors. As a group, these tumors had a poor prognosis, with a median survival of 10.4 months. One-year, two-year, and three-year survival was 46 percent, 26 percent, and 13 percent, respectively. There was no significant difference in survival based on pathologic subtypes. Median follow-up time was 9.4 months (range, 0.6-263.7 months). CONCLUSIONS: Neuroendocrine carcinomas of the colon and rectum are uncommon, comprising less than 1 percent of colon and rectal cancers. Pathologically, these tumors are poorly differentiated carcinomas with distinctive cytoarchitectural features and are often immunoreactive for markers of neuroendocrine differentiation. The prognosis for high-grade neuroendocrine carcinomas is poor, as most patients have metastatic disease at the time of diagnosis.

Surgical resection and high dose rate intraoperative radiation therapy for locally recurrent rectal cancer.

For intra-pelvic recurrence of rectal cancer, surgical resection is technically difficult and must be aggressive to achieve a high rate of negative resection margins. Resection with clear margins can be curative, particularly for those patients with true anastomotic recurrence. HDR-IORT is a safe, feasible, versatile, logistically sound modality that is highly reliable in delivering radiation to at-risk surgical margins in the pelvis. Despite surgery and IORT, overall local failure rates in this population are 33 to 50 percent. The most important prognostic variable is the state of surgical resection margins. At our institution, in patients with negative and positive resection margins the 2-year actuarial local recurrence rates are 33 percent versus 73 percent and 5-year survival rates are 51 percent versus 16 percent, respectively. On subset analysis, the most favorable outcome was seen in patients with true anastomotic recurrences (78 percent 5-year survival).

Results after rectal cancer resection with in-continuity partial vaginectomy and total mesorectal excision.

BACKGROUND: Although sharp mesorectal excision reduces circumferential margin involvement and local recurrence, a concomitant partial vaginectomy may be required in women with locally advanced rectal cancer. METHODS: Sixty-four patients requiring a partial vaginectomy during resection of primary rectal cancer were identified. Survival was determined by the Kaplan-Meier method, and distributions were compared by the log-rank test. RESULTS: Locally advanced disease was reflected by presentation with malignant rectovaginal fistulae (n = 6) or cancers described as bulky or adherent/tethered to the rectovaginal septum (n = 32). Thirty-five patients received adjuvant radiation with or without chemotherapy. At a median follow-up of 22 months, 27 (42%) patients developed recurrent disease, with most of these occurring at distant sites. The 5-year overall survival was 46%, with a median survival of 44 months. The 2-year local recurrence-free survival was 84%. The crude local failure rate was 16% (10 of 64), and local recurrence was more common in patients with a positive as opposed to a negative microscopic margin (2 [50%] of 4 vs. 8 [13%] of 60, respectively). Positive nodal status had a significant effect on overall survival (P <.001). CONCLUSIONS: Partial vaginectomy is indicated for locally advanced rectal cancers involving the vagina. The results are most favorable in patients with negative surgical margins and node-negative disease.

Resection of locally recurrent colorectal cancer in the presence of distant metastases: can it be justified?

BACKGROUND: We aimed to determine the outcome of resections for local recurrence of colorectal carcinoma in the presence of distant (M1) disease. METHODS: Patients who underwent resection of local recurrence in the presence of potentially resectable M1 disease were identified from the colorectal database. Outcome was determined by chart review. RESULTS: Forty-two patients (23 men) of mean age 60 years (range, 34-88 years) underwent complete gross resection of their local recurrence in the presence of M1 disease. Thirteen of the 42 underwent synchronous M1 resections to render them free of gross disease (R0). Nine of the 29 patients who left with residual disease (R1) subsequently underwent staged M1 resection, so that 22 of 42 were rendered R0 by surgery. The median survival of all patients was 14.5 months (interquartile range, 6-30 months), and that of patients rendered R0 was 23 months (interquartile range, 10-37 months), in comparison with 7 months (interquartile range, 3-25 months) for those of R1 status (P =.006; log-rank method). Ability to achieve R0 status by synchronous or staged resection was the only factor predictive of survival. CONCLUSIONS: The presence of M1 disease per se should not preclude resection of local recurrence, although case selection is problematic.

Surgical debulking and intraperitoneal chemotherapy for established peritoneal metastases from colon and appendix cancer.

BACKGROUND: Aggressive treatment of peritoneal metastases from colon cancer by surgical cytoreduction and infusional intraperitoneal (IP) chemotherapy may benefit selected patients. We reviewed our institutional experience to assess patient selection, complications, and outcome. METHODS: Patients having surgical debulking and IP 5-fluoro-2'-deoxyuridine (FUDR) plus leucovorin (LV) for peritoneal metastases from 1987 to 1999 were evaluated retrospectively. RESULTS: There were 64 patients with a mean age of 50 years. Primary tumor sites were 47 in the colon and 17 in the appendix. Peritoneal metastases were synchronous in 48 patients and metachronous in 16 patients. Patients received IP FUDR (1000 mg/m2 daily for 3 days) and IP leucovorin (240 mg/m2) with a median cycle number of 4 (range, 1-28). The median number of complications was 1 (range, 0-5), with no treatment related mortality. Only six patients (9%) required termination of IP chemotherapy because of complications. The median follow-up was 17 months (range, 0-132 months). The median survival was 34 months (range, 2-132); 5-year survival was 28%. Lymph node status, tumor grade, and interval to peritoneal metastasis were not statistically significant prognostic factors for survival. Complete tumor resection was significant on multivariate analysis (P = .04), with a 5-year survival of 54% for complete (n = 19) and 16% for incomplete (n = 45) resection. CONCLUSIONS: Surgical debulking and IP FUDR for peritoneal metastases from colon cancer can be accomplished safely and has yielded an overall 5-year survival of 28%. Complete resection is associated with improved survival (54% at 5 years) and is the most important prognostic indicator.

Staging: what makes sense? Can the pathologist help?

The staging of colorectal cancer continues to be an interesting and evolving process. Accurate staging can predict overall prognosis and helps to select appropriate treatment options. This article addresses the clinical and pathologic staging of colon and rectal cancer. The use of endorectal ultrasound in the preoperative clinical staging of rectal cancer is reviewed. The importance of surgical resection margins, lymph node retrieval rates, and tumor markers is discussed.

Functional and quality-of-life outcomes in patients with rectal cancer after combined modality therapy, intraoperative radiation therapy, and sphincter preservation.

PURPOSE: Locally advanced primary and recurrent rectal cancers treated with external beam radiation therapy, intraoperative radiation therapy, and chemotherapy represent a complex group of patients in the setting of extensive pelvic surgery and sphincter preservation. We sought to define functional outcome and quality of life in this subset of patients. METHODS: We retrospectively reviewed our experience with locally advanced primary and recurrent rectal cancer patients who underwent intraoperative radiation therapy with either low anterior resection (n = 12) or coloanal anastomosis (n = 6) between 1991 and 1998. Current functional outcome and quality of life were evaluated by a detailed questionnaire. RESULTS: Median time from operation to assessment was 24 (range, 6-93) months. Using a standardized Sphincter Function Scale, incorporating the number of bowel movements per day and degree of incontinence, patients were graded as poor, fair, good, or excellent function. Of all patients, 56 percent reported unfavorable (poor or fair) function. Of the subset of patients with coloanal anastomosis or very low low anterior resection, 88 percent had unfavorable function as compared with 30 percent with standard low anterior resection. (P = 0.02; Fisher's exact probability test). A quality-of-life satisfaction score based on social, professional, and recreational restrictions demonstrated 56 percent of patients to be dissatisfied with their bowel function. CONCLUSIONS: The majority of patients with advanced rectal cancers who require external beam radiation therapy, extensive pelvic surgery, and intraoperative radiation therapy report unfavorable functional and quality-of-life outcomes after sphincter preservation. In this setting patients being considered for coloanal anastomosis or very low anterior resection may be better served by permanent diversion.

Practice parameters for the treatment of sigmoid diverticulitis--supporting documentation. The Standards Task Force. The American Society of Colon and Rectal Surgeons.

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.

Prokinetic effect of erythromycin after colorectal surgery: randomized, placebo-controlled, double-blind study.

PURPOSE: Nausea and vomiting three to seven days after an elective operation on the colon and rectum remain a persistent clinical problem. Erythromycin, a safe, inexpensive drug that stimulates intestinal motilin receptors, has previously been shown to accelerate gastric emptying significantly after upper gastrointestinal surgery. We aimed to evaluate the effect of postoperative intravenous erythromycin on postoperative ileus in patients undergoing elective surgery for primary colorectal cancer. METHODS: Between May 1998 and April 1999, 150 patients undergoing primary resection of colon or rectal cancer were enrolled in this prospective, randomized, placebo-controlled trial. One hundred thirty-four patients completed the study. Patients were excluded if they had extensive metastatic disease, were taking medications known to interact with erythromycin, or if they required an ileostomy. Patients received either 200 mg of intravenous erythromycin or placebo every six hours. Clinical endpoints were recorded and continuous end-points are presented as mean +/- standard deviation. RESULTS: There were no significant complications related to erythromycin. The erythromycin (n = 65) and placebo (n = 69) groups were comparable regarding demographic and operative factors. The erythromycin group had a slightly shorter length of time to passage of flatus (4.1 +/- 1.3 vs. 4.4 +/- 1.1 days; P = 0.03). There was no significant difference between erythromycin and placebo in time to first solid food (5.6 +/- 1.9 vs. 5.4 +/- 1.8 days), time to first bowel movement (5.2 +/- 1.9 vs. 5.4 +/- 1.3 days), or time to discharge from hospital (7.5 +/- 2.0 vs. 7.6 +/- 2.8 days). There was no difference in the rate of clinically significant nausea (26 vs. 26 percent; P = 0.99), vomiting (17 vs. 16 percent; P = 0.88), or nasogastric tube placement (9 vs. 7 percent; P = 0.68). CONCLUSIONS: Erythromycin does not seem to alter clinically important outcomes related to postoperative ileus in patients undergoing resection for colorectal cancer.

Standards for anal sphincter replacement.

PURPOSE: Anal sphincter replacement offers a new treatment option for patients with severe refractory fecal incontinence or for those who require abdominoperineal resection for localized malignancy. The purpose of this study was to review the current status of anal sphincter replacement, formulate a consensus statement regarding its current use, and outline suggestions for future development. METHODS: Four areas of interests were selected: indications for sphincter replacement, continence scoring and quality of life, choice of therapy, and dissemination of new technology. A questionnaire regarding these issues was developed and circulated to working party members; its results served as the basis for this consensus document. RESULTS: Both electrically stimulated skeletal muscle neosphincter and artificial anal sphincter are options for patients with end-stage fecal incontinence. Electrically stimulated skeletal muscle neosphincter is also appropriate for reconstruction after surgical excision of the anorectum in selected cases. Avoidance of complications requires strict attention to sterile technique, prophylactic antibiotics, and deep venous thrombus prophylaxis. A standardized scoring system is proposed that evaluates both continence and evacuation. Quality of life is a critical endpoint for assessing sphincter replacement, and use of The American Society of Colon and Rectal Surgeons incontinence-specific quality-of-life instrument is recommended. As the efficacy of sphincter replacement becomes proven, dissemination of the technique should occur in a controlled manner to ensure adequate surgeon training, minimization of complications, and optimization of results. CONCLUSIONS: Sphincter replacement by electrically stimulated skeletal muscle neosphincter and artificial anal sphincter provide a continent option for patients with end-stage fecal incontinence and those requiring abdominoperineal resection. The guidelines offered in this document are intended to facilitate the controlled and safe development and acceptance of these new techniques.

Role of endorectal ultrasound in the conservative management of rectal cancers.

Endorectal ultrasonography (ERUS) extends the ability of the clinician to define the clinical features assessed on routine physical examination, and remains the best modality for accurately staging depth of penetration and presumptive nodal status in rectal cancers. The success of conservative management of rectal cancers is predicated on proper patient selection. The preoperative selection of the ideal patient for local therapies can be difficult, and the decision-making process takes into account many critical factors. Careful assessment of the T and N stages is critical in determining the success of conservative therapies, and directing treatment algorithms. Local resections with curative intent are limited to patients with T1N0 rectal cancers, and select patients with T2N0 tumors with favorable pathological criteria. Conservative management may also be extended to patients identified with significant underlying comorbid conditions staged preoperatively with unfavorable T2/T3 lesions, often combined with adjuvant therapies in a palliative setting. In addition, ERUS may have a role in the selection of those patients with more advanced lesions to neoadjuvant chemoradiation, followed by radical resection. Though not clearly defined, ERUS is evolving in its role in the postoperative follow-up of patients treated conservatively for rectal cancers, and can lead to the early detection of local recurrences. The widespread use of ERUS remains limited due to high operator variability and errors in interpretation; however, the role of ERUS in the postoperative management of rectal cancers is evolving and requires further evaluation.

Effect of delivery on anal sphincter morphology and function.

PURPOSE: Anal sphincter injury is a serious complication of childbirth, which may result in persistent anal incontinence. Occult injuries, visualized with endoanal ultrasonography, have previously been reported in up to 35 percent of females in a British study. The aim of the present study was to study anal sphincter morphology and function before and after delivery in primiparous females in the United States. METHODS: Thirty-eight primiparous patients (mean age, 31 years) were evaluated with endoanal ultrasonography, anal manometry, and pudendal nerve terminal motor latency during pregnancy and after delivery. Bowel function before and after delivery was recorded according to set questionnaires. Cesarean section was performed in three patients. RESULTS: Clinical sphincter tears, requiring primary repair, occurred in 15 percent of the patients. After delivery endoanal ultrasonography revealed disruptions in the external anal sphincter in six patients, but no patient had disruption in the internal anal sphincter. One patient had slight scarring in the external sphincter. Of the seven patients with pathologic findings at endoanal ultrasonography, the left pudendal latency increased after delivery (P < 0.05), and manometric results were reduced. Three of these seven patients had a third-degree or fourth-degree tear during delivery. All investigations were normal in the three patients who underwent cesarean section. CONCLUSIONS: The present study demonstrates a significant frequency of sphincter injuries (20 percent) after vaginal delivery. Obstetricians should be aware of this risk and explicitly inquire about incontinence symptoms at follow-up after delivery.

Signet-ring cell carcinoma of the colon and rectum: a matched control study.

PURPOSE: There is little information comparing signet-ring cell carcinoma to common non-signet-ring cell colon and rectal cancers. The aim of this study was to better define the clinicopathologic differences between these two distinct entities. METHODS: Using a prospective database of 5,350 surgical patients with rectal cancers operated on at Memorial Sloan-Kettering Cancer Center between 1986 and 1997, 46 patients with signet-ring cell carcinoma were identified. Signet-ring cell carcinoma lesions were those in which signet-ring cells constituted more then 50 percent of the tumor. Six patients who presented with recurrent disease were excluded from the study. Control patients were matched for age, gender, TNM stage, primary site, procedure, and adjuvant therapy. Age, primary site of the tumor, stage at presentation, and survival times of patients with signet-ring cell carcinoma were also compared with 3,371 patients with primary non-signet-ring cell rectal cancers. Survival was calculated using Kaplan-Meier survival estimates. RESULTS: Mean age of the signet-ring cell carcinoma group was 59 +/- 12 years and median age was 61 (range, 20-91) years. Male-to-female ratio was 1.1:1. Lymphatic and peritoneal spread was more common among the signet-ring cell carcinoma group. Approximately one-third of signet-ring cell carcinoma patients presented with metastatic disease. Mean survival time of the signet-ring cell carcinoma group was 45.4 months (95 percent confidence interval, 26.9-63.8) compared with 78.5 months (95 percent confidence interval, 62.0-94.9) for the control patients group; P = 0.02 by the log-rank test. The cumulative survival curve of patients with signet-ring cell carcinoma resembles that of patients with poorly differentiated rectal cancers. CONCLUSIONS: Patients with signet-ring cell carcinoma of the colon and rectum have a worse prognosis compared with matched controls with the same stage of disease.