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should have surgery delayed, if possible, to allow for adequate phys­ical, social, and intellectual maturity. For patients with classic FAP, surgery should be pursued well before cancer develops and typically occurs around 16 to 20 years of age.
Surgical options include an open or minimally invasive total proctocolectomy (TPC) with creation of an end or continent ileos­tomy, total abdominal colectomy (TAC) with creation of an ileorectal anastomosis (IRA), and a TPC with creation of an IPAA.
Proctocolectomy with End Ileostomy
A proctocolectomy with end Brooke ileostomy has a low rate of complications but leaves the patient with an incontinent stoma. Indications for this approach are patient preference, low rectal cancer requiring an abdominoperineal resection, rectal cancer requiring postoperative pelvic radiation, inability to create an IPAA (inade­quate mesenteric length), and poor sphincter function.
The procedure is carried out in an oncologic approach secondary to the risk of a preoperatively unrecognized cancer. A perineal inter­sphincteric dissection is carried out preserving the external sphincter and levator ani muscles. The perineum is closed in layers, and the greater omentum, if present, is mobilized and placed in the pelvis to prevent future bowel obstructions. After closure of the abdomen, the ileostomy is matured in a standard evaginated Brooke fashion, with an attempted ideal projection of 2.5 cm.
Proctocolectomy with Continent Ileostomy
Initially described by Nils Kock in 1969, the continent ileostomy still remains a viable alternative for motivated patients who are not candidates for an IPAA. Modifications and revisions to the original Kock continent ileostomy have been described (Barnett continent ileostomy reservoir and T-pouch) though without evidence to sug­gest they are better than the Kock pouch. Contraindications to con­struction of a continent ileostomy include Crohn’s disease, obesity, marginal small bowel length, and anyone with a psychological or physical disability that would preclude understanding or being able to perform daily stomal intubation.
Total Abdominal Colectomy with Ileorectal Anastomosis
Colectomy with ileorectostomy should only be considered in cases of attenuated or mild polyposis (<20 rectal, <1000 colonic adenomas), rectal polyps less than 3 cm in size, no colorectal dysplasia or cancer, a distensible and compliant rectum, and in patients with an intact sphincter mechanism who are willing to adhere to strict follow-up. Ileoproctostomy is an appealing alternative in younger patients of reproductive age to decrease the risk of impotence and reduced fecundity. Strict rectal surveillance (every 6–12 months) must be adhered to due to the increased risk of future neoplastic changes. The risk of rectal carcinoma can reach up to 40% by 30 years, though this is based on literature from the pre-IPAA era. In patients who require a completion proctectomy, an end ileostomy, restorative IPAA, or continent ileostomy are all options.
of construction and excellent functional outcomes, has become the most common choice for surgeons.
A total colectomy is performed in an oncologic fashion, and the ileum is transected flush with the cecum (Fig. 1). To provide ade­quate perfusion to the pouch, it is imperative to preserve the ileal branches of the ileocolic and distal mesenteric arteries. Evaluation for adequacy of reach of the small bowel to the deep pelvis should be undertaken before creation of the pouch. The proposed point of the pouch-anal anastomosis can be pulled down to the pubis, and if this point can be easily advanced 3 to 4 cm below the inferior edge of the pubis, one can feel confident of successful reach for anastomosis. Strategies to decrease tension at the anastomosis include complete mobilization of the small bowel mesentery to the root of the superior mesenteric artery cephalad to the head of the pancreas (Fig. 2), prox­imal division of the ileocolic artery (Fig. 3), and relaxing incisions of the mesentery over tension points along the superior mesenteric artery (Fig. 4). Rectal dissection is completed in the TME plane, and transection of the rectum with a 30- to 40-mm transverse stapler
FIG. 1 Transection of ileum flush with cecum. (From Kelley SR, Dozois EJ.
Ulcerative colitis. In A Companion to Specialist Surgical Practice: Colorectal Surgery. 5th ed. Edinburgh: Elsevier; 2014:129.)
Restorative Proctocolectomy/Ileal Pouch Anal Anastomosis
Initially described in 1978 by Parks and Nicholls, the restorative proctocolectomy has become the most common continence-preserv­ing procedure performed in patients who are appropriate candidates. Indications include severe polyposis (>20 rectal, >1000 colonic ade­nomas), rectal polyps larger than 3 cm in size, colonic dysplasia or cancer, dysplastic rectal polyps, and patients with an intact sphincter mechanism willing to adhere to strict follow-up. The restorative pouch can be fashioned in two limbs (J), three limbs (S), four limbs (W), or isoperistaltic (H) configurations. The J-pouch, due to its ease
FIG. 2 Mobilization of the small bowel mesentery to the root of the superior
mesenteric artery. (From Kelley SR, Dozois EJ. Ulcerative colitis. In A Companion to Specialist Surgical Practice: Colorectal Surgery. 5th ed. Edinburgh: Elsevier; 2014:131.)
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FIG. 3 Division of the ileocolic artery. (From Mayo Foundation for Medical
Education and Research.)
FIG. 4 Mesenteric relaxing incisions. (From Mayo Foundation for Medical
Education and Research.)
should occur 2 to 3 cm above the dentate line in the anal transition zone (ATZ) (Fig. 5). After reach has been verified, a J-configuration is fashioned with each limb measuring between 12 to 15 cm in length. The limbs are paired in an antimesenteric fashion and held in orientation with interrupted stay sutures. For those without evidence of adenomas in the ATZ or dysplasia in the lower rectum, a dou­ble-stapled IPAA can be fashioned, otherwise an anal mucosectomy and handsewn IPAA is recommended. Following creation of the IPAA, an air insufflation leak test is performed and, if necessary, a protective loop ileostomy fashioned, which should be created as close
FIG. 5 Rectal transection. (From Kelley SR, Dozois EJ. Ulcerative Colitis. In
A Companion to Specialist Surgical Practice: Colorectal Surgery. 5th ed. Edinburgh: Elsevier; 2014:132.)
to the pouch as possible to decrease issues with high output and in the unlikely case that a redo-IPAA would need to be performed. In selected patients, the operation can be completed with good results without the creation of a diverting loop ileostomy.
Double-Stapled Technique
An enterotomy is made in the antimesenteric apex of the pouch, and a linear cutting stapler is used to divide the walls of the two limbs creating a common channel (Fig. 6). A purse-string suture is then fashioned around the enterotomy, and the anvil from a circular stapler is placed inside the pouch where it is held in place by tightening the purse string (Fig. 7). The circular stapler is then placed transanally. After appropriate orientation, the circular stapler cartridge spike is advanced either above or below the transverse rectal staple line and attached to the anvil. The stapler is then closed, approximating the pouch and anus (Fig. 8).
Handsewn Technique
An anal canal mucosectomy is performed starting at the dentate line. Raising the mucosa with a submucosal injection (Fig. 9) of dilute saline and epinephrine (1:200,000) facilitates dissection of the mucosa away from the internal sphincter muscle (Fig. 10), which can be completed sharply or with electrocautery. After the mucosa and proximal rectum have been removed circumferentially, the pouch is gently brought down to the level of the dentate line. An enterotomy is made in the apex of the pouch, if not already created, and it is anchored in position by placing a suture in each of the four quadrants incorporating a full thickness bite of the pouch, internal sphincter muscle, and mucosa. Sutures are placed between the anchoring stitches to complete the anastomosis (Fig. 11).
Postoperative Surveillance
After the creation of an IPAA or IRA, lifelong interval lower endoscopic surveillance is required for adenomas, dysplasia, and
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FIG. 8 Stapled ileal pouch anal anastomosis. (From Mayo Foundation for
Medical Education and Research.)
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FIG. 6 J-pouch creation. (From Mayo Foundation for Medical Education and Research.)
FIG. 9 Submucosal injection. (From Mayo Foundation for Medical Education
and Research.)
a completion proctectomy in those with an IRA. Following TPC with end ileostomy, yearly stoma site surveillance by a stoma nurse or experienced practitioner is recommended due to the rare develop­ment of ileal adenocarcinoma, most commonly near the mucocuta­neous junction in the setting of long-standing ileostomies.
FIG. 7 Anvil in J-pouch. (From Mayo Foundation for Medical Education and Research.)
carcinomas at 1- to 2-year or 6-month to 1-year intervals, respec­tively. Histologic evaluation of random biopsies and polyps should be performed to exclude dysplasia and cancer. More frequent surveillance is performed for increased numbers or size of polyps.
OTHER POLYPOSIS SYNDROMES
Peutz-Jeghers Syndrome
Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disease resulting most commonly from a mutation in the LKB1 (STK11) tumor suppressor gene located on chromosome 19p13. Anywhere from 30% to 40% will occur de novo. Hamartomatous polyps are found throughout the GI tract, though most commonly in the small intestine. Extraintestinal manifestations are common
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Colonoscopy and EGD should be initiated at 8 years of age. If polyps are detected, endoscopic evaluation should continue every 2 to 3 years. If no polyps are found, repeat endoscopy and small bowel follow-through or capsule enteroscopy should be initiated by 20 years of age and repeated every 2 to 3 years. Other surveillance rec­ommendations with low levels of evidence include an annual clinical exam, annual testicular ultrasound starting at age 10, monthly breast exam and annual breast MRI starting at 18 years of age, cervical smear starting at age 25, and magnetic resonance cholangiopancrea­tography (MRCP) of the pancreas starting at 25 years of age.
GI surgery is reserved for symptomatic disease or cancer. Any polyp larger than 1.5 cm should be removed, if possible, at the time of surgery. Intraoperative on-table endoscopy can be utilized to eval­uate the entire GI tract.
Juvenile Polyposis Syndrome
Juvenile polyposis syndrome (JPS) is an autosomal dominant inher­ited disease resulting most commonly from mutations in the SMAD4 and BMPR1A genes, which are respectively located on chromosomes
FIG. 10 Anal mucosectomy. (From Kelley SR, Dozois EJ. Ulcerative Colitis.
In A Companion to Specialist Surgical Practice: Colorectal Surgery. 5th ed. Edinburgh: Elsevier; 2014:134.)
18q21 and 10q22. Polyps can be found throughout the GI system with the colon being affected 100% of the time. Extraintestinal manifestations occur around 15% of the time and can include cleft lip and palate, polydactyly, genitourinary anomalies, intestinal mal­rotation, hydrocephalus, and congenital heart disease. Hereditary hemorrhagic telangiectasia and bleeding arteriovenous malforma­tions (AVMs) are associated with some SMAD4 mutations and are found in the GI tract, lungs, brain, and mediastinum. Diagnosis of JPS is confirmed when five or more juvenile polyps are found in the colon or rectum, multiple polyps are appreciated in other regions of the GI tract, or after identification of polyps with a positive family history. Presenting symptoms can include hematochezia and mel­anotic stools, anemia, intussusception, obstruction, and passage of autoamputated or prolapsed polyps. Colorectal cancer is the most common associated malignancy with lifetime rates as high as 39%. Other malignancies include gastric, duodenal, and pancreatic.
Asymptomatic patients should begin with screening colonoscopy by 12 to 15 years of age and earlier for those with symptoms. If no polyps are detected, evaluation can be repeated every 2 to 3 years, otherwise annually. EGD is recommended by age 15. Those with a SMAD4 mutation should have periodic screening for AVMs.
Colorectal surgery is reserved for symptomatic disease, dysplasia, cancer, or significant polyp burden (>100 polyps). For those with a relatively spared rectum, a TAC with IRA can be pursued, and if the rectum is significantly involved, a TPC with IPAA is advisable. Sur­geries in the remaining GI tract may also be warranted.
FIG. 11 Hand sewn ileal pouch anal anastomosis. (From Mayo Foundation
for Medical Education and Research.)
with the hallmark phenotypic feature in adolescence being mucocu­taneous hyperpigmentation that can affect the perioral and buccal region, eyes, nostrils, perianal region, fingers and toes, and hands and feet. Hyperpigmentation dissipates as one ages. Hamartomatous polyps and mucocutaneous pigmentation confirms a diagnosis of PJS. Presenting symptoms can include abdominal pain, alteration in bowel habits, weight loss, bowel intussusception, anemia, hemato­chezia and melanotic stools, and small bowel obstruction. The risk of malignancy increases with age (13-fold higher than the general population) with the most common cancers being colorectal, breast, pancreatic, and genitourinary.
Cowden’s Syndrome
Cowden’s syndrome (CS) is an autosomal dominant disorder resulting from a mutation in the PTEN tumor suppressor gene located on chro­mosome 10q23. Polyps typically occur in the colon and stomach, and colonic polyps can include hamartomas, fibromas, adenomas, lipomas, and neurofibromas. Extraintestinal manifestations include pathogno­monic trichilemmomas, macrocephaly, and a wide variety of tumors and hamartomas of various organ systems (breast, thyroid, uterus).
The increased risk of colorectal cancer is estimated to be two or three times the general population (9%–16%). National Comprehen­sive Cancer Network (NCCN) guidelines recommend a screening colonoscopy starting at the age of 35. Thyroid screening with physical exam and ultrasound should be performed annually and begin at age
15. Annual mammography, with MRI for suspicious findings, should begin at the age of 30. Treatment is based on symptoms and pathology.
Bannayan-Riley-Ruvalcaba Syndrome
Bannayan-Riley-Ruvalcaba syndrome (BRRS) is an autosomal dom­inant disorder resulting from a mutation in the PTEN tumor
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suppressor gene located on chromosome 10q23. Common findings associated with BRRS include pigmented penile macules, macro­cephaly, hamartomas, hemangiomas, and mental retardation in upward of 50%.
The risk of developing colon and rectal cancer is thought to be no greater than the general population. Treatment is based on symptoms.
Cronkhite-Canada Syndrome
Cronkhite-Canada syndrome (CCS) is a noninherited disorder resulting from a mutation in the PTEN tumor suppressor gene located on chromosome 10q23. Hamartomatous GI polyps in addi­tion to alopecia, macrocephaly, onycholysis, and cutaneous pigmen­tation are common findings. Diffuse GI inflammation resulting in malabsorption, diarrhea, and protein-losing enteropathy can occur.
The risk of developing colon and rectal cancer is thought to be greater than the general population. Treatment is based on symptoms and pathology.
Hereditary Mixed Polyposis Syndrome
Hereditary mixed polyposis syndrome (HMPS) is thought to be an autosomal dominant inherited syndrome, though a specific mutation has yet to be identified. HMPS presents with multiple different colon and rectal polyps (adenomatous, hamartomatous, and hyperplastic).
The risk of developing colon and rectal cancer is thought to be greater than the general population, although this is unsubstantiated at this time. Screening colonoscopy should begin between the ages of 25 to 30. Treatment is based on symptoms and pathology.
Serrated Polyposis Syndrome
Serrated polyposis syndrome (SPS) is a disorder characterized by multiple polyps (hyperplastic or serrated) throughout the colon. A heritable pattern and genetic cause has not been identified. The World Health Organization has proposed two criteria for diagnos­ing SPS, of which diagnosis is made upon fulfillment of either of the criteria. The criteria include (1) at least five serrated polyps, all
greater than 5 mm in size and proximal to the rectum, two of which are greater than 10 mm in diameter, or (2) more than 20 serrated polyps of any size distributed throughout the large intestine with five being proximal to the rectum. These criteria distinguish the two SPS phenotypes; large serrated polyp proximal disease and distal small serrated polyp distal disease.
The risk of developing colon and rectal cancer is increased with rates of up to 25% to 70% documented in those with multiple polyps. The average age for developing colon and rectal cancer is 50 to 60. Treatment is based on polyp burden and dysplastic or neoplastic changes.
Strict surveillance with colonoscopy every 1 to 2 years is advis­able. First-degree relatives are at an increased risk of SPS and devel­oping colon and rectal cancer (5-fold), and should be offered the same surveillance starting at 40 years of age or 10 years younger than the index case.
S u g g e S t e d R e a d i n g S
Beggs AD, Latchford AR, Vasen HF, etal. Peutz-Jeghers syndrome: a systematic
review and recommendations for management. Gut. 2010;59(7):975–986. Dinarvand P, Davaro EP, Doan JV, etal. Familial Adenomatous Polyposis
Syndrome: An Update and Review of Extraintestinal Manifestations. Arch
Pathol Lab Med. 2019;143(11):1382–1398. Herzig D, Hardiman K, Weiser M, et al. The American Society of Colon
and Rectal Surgeons Clinical Practice Guidelines for the Management of
Inherited Polyposis Syndromes. Dis Colon Rectum. 2017;60(9):881–894. Kalady FM, Heald B. Diagnostic approach to hereditary colorectal cancer
syndromes. Clin Colon Rectal Surg. 2015;28(4):205–214. Latchford AR, Sturt NJ, Neale K, etal. A 10-year review of surgery for des-
moid disease associated with familial adenomatous polyposis. Br J Surg.
2006;93(10):1258–1264. Serrano PE, Grant RC, Berk TC, et al. Progression and Management of
Duodenal Neoplasia in Familial Adenomatous Polyposis: A Cohort Study.
Ann Surg. 2015;261(6):1138–1144. Syngal S, Brand RE, Church JM, etal. ACG clinical guideline: Genetic test-
ing and management of hereditary gastrointestinal cancer syndromes. J
Gastroenterol. 2015;110(2):223–262. Yang J, Gurudu SR, Koptiuch C, etal. American Society for Gastrointestinal
Endoscopy guideline on the role of endoscopy in familial adenomatous
polyposis syndromes. Gastrointest Endosc. 2020;91(5):963–982.
Surgical Managementof Colon Cancer
Mohamad A. Abdulhai, MD, and Michael A. Choti, MD
olon cancer is the third most common cancer and cause of cancer death in the United States. An estimated 4.5% of the US
C
population will be diagnosed with colon cancer in their lifetime. Fortunately, the mortality from colon cancer has been declining in recent decades, in part as a result of improvements in screening, surgical management, and chemotherapeutic options. The incidence of colon cancer in older adult patients has been steadily declining; however, we have been seeing an increase in frequency in patients younger than 50 years of age. More than two-thirds of colorectal can­cers arise in the colon, defined as the intraabdominal portion of the large bowel that extends from the cecum to the peritoneal reflection. Although the biologic distinction between the colon and rectum is
somewhat arbitrary, the oncologic behavior and cancer management differs considerably. Yet, similar to rectal malignancies, colon cancer is also best managed using a multidisciplinary team approach to optimize outcome. This chapter reviews the preoperative evaluation, clinical staging, and management of patients with colon cancer, high­lighting the importance of a multidisciplinary approach.
CLINICAL PRESENTATION AND
SCREENING
Colon cancer patients can present with a variety of gastrointestinal symptoms, depending on the size and location of the tumor. This can include abdominal pain, hematochezia, weight loss, anemia, constipa­tion, diarrhea, or change in the caliber of stools. Classically, right-sided tumors tend to present with anemia, whereas, left-sided cancers are more likely to present with obstructive symptoms. However, many patients with colon cancer are asymptomatic at the time of diagnosis and identified by screening. This highlights the importance of screening to detect colon cancer before it becomes symptomatic. Implementation of increased screening, including colonoscopy, has contributed to the decline in colon cancer-related mortality seen in the past 20 years.
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Screening for average-risk adults is now recommended to begin at age 45. There are several options for screening, including colo­noscopy every 10 years, multitarget stool DNA testing every 3 years, annual fecal immunochemical testing, annual fecal occult blood testing with flexible sigmoidoscopy every 5 years, or double-contrast enema and CT colonography every 5 years. Those at higher risk for the development of colorectal cancer may require more frequent screening. In some conditions such as familial polyposis syndromes or hereditary nonpolyposis (Lynch syndrome), prophylactic colec­tomy may be indicated. Options for screening for colorectal cancer may be changing in the future, particularly with the application of newer technologies for the detection of minute fragments of circulat­ing tumor DNA (ctDNA) in the bloodstream. Studies are underway evaluating the role of ctDNA for cancer screening.
PREOPERATIVE EVALUATION AND
STAGING
The initial evaluation may depend on the type and severity of symptoms the patient is experiencing, in some cases necessitating more urgent intervention. In most cases, colon cancer presents more electively, affording the opportunity for careful evaluation and staging. Adequate preoperative colonoscopy is important to assess the details of the colon tumor, including the size, location, and extent. Biopsy provides his­tologic confirmation and characterization of pathologic features such as tumor grade, microsatellite status, and in some cases molecular sequencing. However, in some cases of invasive cancer, the biopsy may miss the invasive component. In such cases, one must be careful not to assume no malignancy if the biopsy is negative, particularly with a presentation or appearance consistent with cancer. During colonoscopy, it is important to ensure that the entire colon is examined when possible as 5% of patients have a synchronous cancer and 10% to 20% have a synchronous advanced polyp, which might alter the surgical plan. In some cases, an endoscopically obstructing distal tumor may prevent the ability to evaluate the proximal colon. In such cases, one can evaluate the proximal colon intraoperatively or with a colonoscopy 3 to 6 months after resection. During colonoscopy, tattooing should be considered in some cases to facilitate intraoperative detection.
Routine blood analysis should be obtained, including a complete blood count and metabolic profile. In addition, a preoperative carci­noembryonic antigen (CEA) level should be measured. It is import­ant to obtain a baseline CEA level to compare with postoperative levels on surveillance and to alert to the increased probability of advanced disease. Cross-sectional imaging, most commonly CT, is also important before surgical intervention to determine the extent of local disease and assess for metastatic disease. Fluorodeoxyglu­cose positron emission tomography (FDG-PET) is not routinely recommended for the initial evaluation and should be used only selectively when the imaging is inconclusive or preoperative CEA is high (>20 ng/mL).
SURGICAL MANAGEMENT
Preoperative Preparation
The management of colon cancer depends on the stage at presenta­tion. The majority of patients with stage I to III colon cancer can be managed with initial surgical resection with curative intent. The goal of surgical resection is removal of the primary tumor in its entirety with negative margins in addition to performing a complete lymph­adenectomy of the draining lymph nodes. In cases in which the tumor involves adjacent organs, en bloc resection of those structures should also be considered. In cases of more advanced disease (stage IV), surgical resection of the primary tumor is done selectively, either palliatively for those with significant symptoms or as part of a cura­tive-intent approach combined with metastatectomy.
The patient should undergo medical optimization before sur­gery to ensure safety of proceeding with an abdominal operation under general anesthesia. Both mechanical and oral antibiotic bowel
preparations should be performed to decrease the risk of infectious complications. This includes a clear liquid diet the day before surgery and consumption of a purging solution such as polyethylene glycol (GoLYTELY or MiraLAX). In addition, patients are given oral anti­biotics (e.g., neomycin and metronidazole) concurrently with the mechanical bowel preparation.
As with other complex operative procedures, the perioperative management of patients undergoing elective colorectal surgery should be guided by enhanced recovery protocols to improve out­comes, standardize care, and lower healthcare costs. Such pathways focus on improved pain management and minimizing narcotics by using a multimodal pain regimen. In addition, early mobilization, early feeding, and limiting fluid administration is important to has­ten the return of bowel function and improve recovery.
The patient should receive prophylactic intravenous antibiotics before skin incision to reduce the risk of surgical site infection. In addition, patients should receive subcutaneous heparin and a sequential compression device placed before induction of anesthesia for venous thromboembolism prophylaxis. An indwelling urinary catheter is typically used. The patient is positioned supine for right­sided lesions and in the modified lithotomy position for left-sided lesions to allow access to the perineum for use of the end-to-end stapler and intraoperative endoscopy.
Operative Approach
The choice of operative method for colon resection can vary based on surgeon experience and patient/tumor factors. Several large mul­ticenter randomized controlled trials have shown equivalent onco­logic outcomes (e.g., nodal harvest, recurrence, survival) between open and laparoscopic colectomy. In most cases, a minimally inva­sive approach results in less pain, shorter hospital stay, and faster recovery, and it is recommended whenever the appropriate expertise is available. More recently, the use of robot-assisted surgery has been advocated as an alternative minimally invasive approach. Although few randomized trials have been conducted comparing robotic versus laparoscopic colectomy, improved visualization, facilitated intracorporeal anastomosis, and lower conversion rates may promote the robotic technique, albeit at a higher cost. In cases of large and locally advanced tumors requiring multivisceral resection, an open colectomy remains the preferred approach in most cases.
Colon Resection
Regardless of whether a minimally invasive or open approach is used, the technical and oncologic principles during surgery are the same. A thorough exploration of the abdomen should be initially performed to assess for metastatic disease. This includes visual inspection and, when possible, palpation of the peritoneal cavity along with the abdominal and pelvic organs, including the liver, omentum, and peritoneal surfaces. A curative resection of colon cancer entails removal of the involved segment of colon along with its mesentery at the origin of the feeding vessel. A 5- to 7-cm proximal and distal margin is typically recommended to ensure adequate removal of the pericolic lymph nodes. Histologic evaluation of a minimum of 12 lymph nodes is recommended to accurately determine nodal stage. Following extirpation, bowel continuity is restored using a tension-free, well-vascularized anastomosis. The alignment of the mesentery should be verified to avoid any torsion or internal her­nia. Anastomotic technique can differ based on surgeon preference. Randomized studies have shown no difference in outcomes between stapled and handsewn techniques. Ileocolonic anastomoses are usually performed in a side-to-side functional end-to-end fashion. Colocolonic and colorectal anastomoses can be performed using either an end-to-end or side-to-end technique.
The extent of colonic resection depends on the location of the primary tumor and its draining lymphatic basin (Fig. 1). Tumors of the cecum, ascending colon, and hepatic flexure are managed with
MCA MCA
AI
AL
AB
CD
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RCA RCA
LC
SA SA
SHA
ICA
MCA
IMA
RCA
LCA
SA
SHA
ICA
ICA
RCA
ICA
MCA
SHA
SHA
MA
CA
IMA
LCA
SA
FIG. 1 Extent of resection for colon carcinoma. (A) Cecal or ascending colon
cancer. (B) Transverse colon cancer. (C) Splenic flexure colon cancer. (D) Sigmoid colon cancer. ICA, Ileocolic artery; IMA, inferior mesenteric artery; LCA, left colic artery; MCA, middle colic artery; RCA, right colic artery; SA, sigmoidal arteries; SHA, superior hemorrhoidal artery. (From Ruo L, Guillem JG. Cancer of
the colon. In: Bland KI, Daly JM, Karakousis CP, eds. Surgical Oncology: Contemporary Principles and Practice. New York: McGraw-Hill; 2001.)
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SPECIAL SCENARIOS
Malignant Polyp
A malignant polyp is defined as an adenoma containing a focus of adenocarcinoma invading the muscularis mucosa into the submucosa (T1). About 5% of all endoscopically resected adenomas will contain invasive adenocarcinoma. Management of these polyps relies on detailed histopathologic examination of the specimen to estimate the risk of residual luminal cancer and the risk of lymph node metastasis. This in turn will determine whether endoscopic resection is suffi­cient or an oncologic segmental resection is warranted. If a negative histologic margin cannot be assessed, repeat endoscopy or segmental resection may be required. High-risk features for lymph node involve­ment include poor differentiation, deep submucosal invasion, pres­ence of lymphovascular or perineural invasion, high tumor budding score, and positive endoscopic resection margin. Sessile polyps in general confer a higher risk of lymph node metastasis compared with pedunculated polyps. A 2-mm negative resection margin is considered adequate. The depth of invasion into the submucosa is also predictive of occult lymph node metastasis, and several classification systems have been developed to estimate the risk. The first is the Haggit clas­sification for pedunculated polyps. In the absence of other high-risk features, the risk of lymph node metastasis in Haggit level 1, 2, and 3 lesions is less than 1%, but it can be up to 30% in Haggit level 4 polyps. The other commonly used classification system is the Kikuchi system, which evaluates the depth of invasion into the submucosa. The submucosa is divided into thirds: upper (Sm1), middle (Sm2), and lower (Sm3). The risk of lymph node metastasis for Sm1 tumors is 1% to 2%, Sm2 is <10%, and Sm3 is 20% to 25%. The application of the Kikuchi classification following endoscopic resection can be prob­lematic as the muscularis propria layer is usually not included in the specimen. Therefore, alternatively the absolute depth of invasion into the submucosa is used with depth of invasion less than or equal to 1 mm conferring a low risk of lymph node metastasis.
Patients who undergo complete adequate endoscopic resection and are considered to have a very low risk of occult lymph node metastasis must be closely monitored for recurrence. There is cur­rently no established standard for surveillance after endoscopic resection of malignant polyps in patients who do not undergo sur­gery. However, most experts recommend surveillance colonoscopy in 3 to 6 months. It is crucial to ensure that the site of the polypec­tomy is marked with a tattoo at the time of polypectomy or soon after (within 2 weeks) to allow for identification of the polypectomy scar on subsequent surveillance colonoscopies.
a right hemicolectomy. This entails high ligation of the ileocolic and right colic vascular pedicles and preservation of the middle colic vessels. This is followed by removal of the terminal ileum, cecum, ascending colon, and hepatic flexure and construction of an ileotransverse anastomosis. Transverse colon cancers are managed depending on their location. Tumors of the mid or distal transverse colon are managed with an extended right hemicol­ectomy, which involves ligation of the ileocolic, right colic, and middle colic pedicles and removal of the cecum, ascending colon, transverse colon, and construction with an ileodescending anas­tomosis. Tumors of the splenic flexure or descending colon can be managed with either a left hemicolectomy or an extended left hemicolectomy. The former entails takedown of the splenic flex­ure with ligation of the left colic artery along with the left branch of the middle colic artery and construction of a transverse-to-sig­moid anastomosis. The latter involves ligation of the inferior mesenteric artery with anastomosis of the distal transverse colon to the upper rectum. The oncologic outcomes for either approach are considered equivalent. Sigmoid tumors are managed with anterior resection of the sigmoid colon performed by high liga­tion of the inferior mesenteric pedicle with anastomosis of the descending colon to the upper rectum.
Locally Advanced Cancer
Locally advanced colon cancers with invasion of surrounding structures (T4) are seen in 5% to 10% of cases. Some of the most commonly involved organs include the small bowel, bladder, spleen, duodenum, and stomach. Detailed evaluation using cross-sectional imaging (CT scan or MRI) is important preoperatively to recognize multivisceral involvement. When possible, an en bloc multivisceral resection should be done to achieve an R0 resection and offer the best chance for cure. In such cases, neoadjuvant chemotherapy and sometimes radiation therapy can be used to improve the ability to achieve complete resection. This is reported in the FOxTROT international randomized controlled trial, which showed a 59% evidence of histologic downstaging and halving the rate of incom­plete resections when using neoadjuvant chemotherapy. In addition, neoadjuvant chemotherapy was well tolerated and did not increase perioperative morbidity. The long-term oncologic outcomes using this approach are yet to be determined.
Stage IV Cancer
About 20% to 25% of patients will present with synchronous meta­static disease. Colon cancer most commonly metastasizes to the liver,
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followed by the lung and peritoneal cavity. The management of stage IV colon cancer can be complex. First, it is important to identify if the metastatic disease burden is considered resectable to determine if the patient falls under a curative intent paradigm of treatment. Second, one should evaluate if the primary tumor is symptomatic, resulting in significant bleeding or obstruction. Recent evidence suggests less need for early palliative colectomy than in the past. Only if the primary cancer is appreciably symptomatic should the patient have this addressed with resection, diversion, or stenting before considering systemic therapy. Otherwise, early surgery is not indi­cated, and systemic chemotherapy is initiated. Even in symptomatic patients, it is not uncommon for the symptoms to improve following response to chemotherapy. The choice of first-line chemotherapy for advanced colon cancer typically includes a combination regimen of fluoropyrimidine, oxaliplatin, and/or irinotecan. In some cases, a biologic therapy such as bevacizumab may be included. Caution must be used, however, when administering bevacizumab with a bleeding or partially obstructing colon cancer in situ as this agent may increase the risk of wound complications or bleeding.
If curative resection of the oligometastatic disease is being con­sidered, surgical resection both of the primary tumor and metastases should be done, either in a staged or synchronous approach. The decision to proceed with which approach should be tailored to the individual patient and surgical availability, considering the patient’s physical condition and the extent and magnitude of the primary resection and proposed metastatectomy. In general, one can perform a limited metastatectomy (e.g., minor hepatectomy) with primary resection in a single stage. More complex surgery such as a major hepatectomy can be combined with a simple primary resection, but extensive metastatectomy along with complex primary resection is best managed with a staged approach.
TUMOR-RELATED EMERGENCIES
Perforation
The management of perforated colon cancers can be quite challeng­ing as the surgeon must address both the sepsis and contamination associated with the perforated colon while adhering to the oncologic principles of complete cancer resection when possible. Moreover, perforated tumors are associated with a higher risk of recurrence. Because emergent colectomy for perforation with primary anasto­mosis is associated with a higher risk of anastomotic leak, consid­eration of a proximal diversion (loop ileostomy) or resection with end colostomy/ileostomy with a Hartmann pouch may be prudent. When making this decision, one must consider the clinical condition and stability of the patient along with the quality of the bowel and degree of abdominal contamination.
Bowel Obstruction
Large bowel obstruction can be seen in up to one-third of newly diagnosed colon cancers. Left-sided tumors are more likely to cause obstruction compared with right-sided lesions. The management of obstructing colon cancer varies by location, degree of obstruction, and the clinical condition of the patient. The first step is to assess the acuity and degree of obstruction, determined by clinical exam­ination and imaging. It is important to assess the degree of proximal colonic dilation on imaging and look for signs of impending perfo­ration (significant dilation of the cecum to >10 cm or evidence of pneumatosis). One must also evaluate whether the ileocecal valve is competent as this can result in a closed-loop large bowel obstruction and a higher risk of perforation.
In general, obstructing right-sided lesions can be managed with a right or extended right hemicolectomy, often with primary anastomosis. In patients who are hemodynamically unstable, frail, or malnourished, end ileostomy or proximal diversion should be considered. The management of left-sided obstructing lesions can be more challenging. One must consider whether the tumor is
resectable as well as the presence and extent of metastatic disease. When performing urgent surgery for an obstructing left-sided can­cer, options for resection with anastomosis and diverting ileostomy, resection and end colostomy, simply diverting proximal colostomy without resection should be entertained.
Endoscopic stenting can also have a role in the management of obstructing left-sided tumors. In selected cases, stenting can allow for temporary decompression and bowel preparation and is followed by elective single-stage colectomy. In situations with more advanced disease, palliative stenting followed by systemic chemotherapy can be considered. Stenting should only be performed by experienced endoscopists to mitigate the risks of perforation, occlusion, and migration. Stenting therefore can be considered either as a bridge to curative intent surgical resection or in palliative cases in which patients have incurable disease, limited life expectancy, and poor surgical candidacy.
ADJUVANT THERAPY
The goal of adjuvant systemic chemotherapy following curative-in­tent resection of colon cancer is eradicating micrometastatic dis­ease and increasing the probability for cure. The decision to offer adjuvant chemotherapy is largely based on nodal status. However, the final shared decision should be individualized, considering other risk factors for recurrence and the patient’s general physical condition. In general, systemic adjuvant chemotherapy is indicated in stage III disease. Several large randomized clinical trials have demonstrated improvement in survival, with a 30% reduction in recurrence risk and mortality. In some cases, adjuvant chemo­therapy can also be considered for patients with high-risk stage II colon cancer as their recurrence risk is similar to that of stage IIIA disease. The high-risk features include T4 tumors, poorly differ­entiated histology, <12 lymph node harvest, presence of lympho­vascular or perineural invasion, obstructed or perforated tumors, and positive margins. A regimen including a fluoropyrimidine and oxaliplatin (FOLFOX or CapeOX) is typically recommended for adjuvant therapy following colectomy, most commonly for a 6-month duration. Recent randomized trials have found com­parable benefit with shorter duration (3 months) in select cases. Studies are ongoing to evaluate the emerging role of ctDNA to detect minimal residual disease following resection of colon cancer. This technology may help more clearly identify which patients will derive benefit from chemotherapy and can reduce its use in those with a higher probability of surgical cure.
SURVEILLANCE
Postoperative surveillance is important to allow for early identifi­cation of recurrent disease. Based on guidelines from the National Comprehensive Cancer Network, patients with stage II or III colon cancer should undergo serial serum CEA testing every 3 to 6 months for the first 2 years, then every 6 months up to 5 years. CT scanning is recommended every 6 to 12 months for 5 years. Surveillance colo­noscopy is recommended 1 year after resection and again in 3 years if no polyps are found.
CONCLUSION
Improvement in early detection, surgical techniques, and multidis­ciplinary management have led to improved outcomes for patients with colon cancer. Although operative approaches to colon resection can vary, the principles of oncologically adequate surgery remain standard. Importantly, adherence to evidence-based perioperative management can contribute to safer surgery, more rapid postoper­ative recovery, and improved long-term outcome. Beyond surgical technique, future discoveries in molecular genetics, screening, improved detection, risk determination, and systemic therapies will likely continue to improve outcomes in the next decade.
LARGE BOWEL
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PET Scanning in the Management of Colorectal Cancer
Joy Zhou Done, MD, and Sandy Hwang Fang, MD
INTRODUCTION
Accurate staging of disease is crucial to the management of colorectal cancers. Widely disseminated disease is a contraindication to surgi­cal resection of a primary tumor, and thus the detection of metastatic disease provides important information to guide therapeutic goals. According to National Comprehensive Cancer Network (NCCN) guidelines, computed tomography (CT) and magnetic resonance imaging (MRI) play a significant role in the initial diagnosis, staging,
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term outcomes in robot-assisted compared to laparoscopic colon cancer resec­tions: a systematic review and meta-analysis. Surg Endosc. 2022;36(1):32–46.
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Vogel JD, Felder SI, Bhama AR, etal. The American Society of Colon and
Rectal Surgeons Clinical Practice Guidelines for the Management of Colon Cancer. Dis Colon Rectum. 2022;65(2):148–177.
and management of colorectal cancer (CRC); however, these imaging techniques provide only anatomic information about the tumor. The use of positron emission tomography (PET) as a hybrid to tomo­graphic imaging, such as CT and MRI, adds functional data to these imaging modalities (Figs. 1 to 3; Table 1). The combined acquisition of PET CT provides more accurate information than either study alone by capturing quantitative data from regions of interest (ROI) to provide information about the entire cancer phenotype or microen­vironment. Parametric analysis (PA) allows the extraction of numer­ical data contained in the voxels of each image to determine tumor biology of a specified ROI. PA can characterize tumor perfusion, heterogeneity, cellularity and fibrosis, oxygenation, and glucose con­sumption within an ROI using quantitative data on spatial complex­ity, density, signal intensity, activity, and distributions. This chapter describes the functional utility of PET scanning in the management of CRC, which gives information beyond what is delineated through visual inspection by CT or MRI alone.
A
C
B
FIG. 1 Multimodal imaging of extramesorectal metas-
tasis of the right internal iliac lymph nodes, likely met­astatic as seen on FDG-PET CT (A), MRI (B), and CT
(C).
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A B
FIG. 2 Multiple FDG-avid hepatic lesions representing metastatic disease as seen on FDG-PET scan (A); ill-defined hypodense hepatic mass as seen
on CT (B).
A
C
B
FIG. 3 Osseous metastasis within the T5 ver-
tebral body as demonstrated by FDG-avidity on FDG-PET CT (A), T2-weighted hyperinten­sity and enhancement MRI (B), and irregularity within the vertebral body on CT (C).
TABLE 1 Sensitivity and Specificity of Imaging Modalities Used in Detection of Hepatic Metastases in CRC
Sensitivity (%) Specificity (%)
Contrast-enhanced CT 84 95 MRI 88 93 FDG-PET/CT 97 97
From Niekel MC, Bipat S, Stoker J. Diagnostic imaging of colorectal liver metastases with CT, MR imaging, FDG-PET, and/or FDG-PET/CT: a meta-analysis of prospective studies including patients who have not previously undergone treatment. Radiology. 2010;257(3):674–684.