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AccessSurgery | Print: Chapter 4. Diseases and Problems
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– CT/MRI abdomen/pelvis: invasion of adjacent organs?
– PET or PET-CT: pelvic activity, evidence for extrapelvic disease?
Verification of recurrence: biopsy (endoscopic, CT- or ultrasound-guided) vs time-dependent change on imaging and blood
work, consistent only with recurrence.
ADDITIONAL TESTS (OPTIONAL)
Contrast studies: assessment of residual colon length.
Urology/GYN consultation: if potential tumor invasion cannot be ruled out.
Assessment of general operability.
Classification
Location of the recurrence:
Central pelvis.
Lateral pelvic sidewall.
Presacral.
Nonoperative Treatment
Chemotherapy: indicated for all patients who tolerate it except for immediate perioperative period.
Radiation therapy:
– Tumors not previously radiated: first-line treatment option, potentially followed by a later resection.
– Previously radiated tumors: consideration for intraoperative radiation therapy (IORT), high-dose brachytherapy, or
IMRT if status post previous radiotherapy.
Best palliative care: if nonresectable tumor or nonoperable patient.
Operative Treatment
INDICATIONS
For aggressive resection:
– Resectable-appearing local recurrence without extrapelvic disease.
– Highly symptomatic, resectable-appearing local recurrence with extrapelvic disease.
For palliative surgery: highly symptomatic, but nonresectable recurrence.
OPERATIVE APPROACH
Re-resection of posterior compartment: redo-LAR with coloanal anastomosis, redo-APR (intraoperative ureteral
stents!), potential defect closure with myocutaneous flap.
Re-resection of posterior compartment (as above), with extension to middle/anterior compartments:
– Male: pelvic exenteration with/without fecal/urinary reconstruction.
– Female: vaginectomy/hysterectomy vs pelvic exenteration, with/without fecal/urinary reconstruction.
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Comment: No continence preservation (1) if the anal sphincter complex/pelvic floor muscles tumor-involved and have to be
resected, (2) if proximal bowel not reaching pelvic floor, (3) if preoperative fecal incontinence documented, or (4) if the
sphincters have been resected in previous procedure.
Major resection (as above), with inclusion of distal sacrum, etc: high morbidity!
Nonresective palliation: creation of colostomy/urostomy, stenting.
Outcome
Survival: estimated 30–35% long-term cure.
Symptom control: except for the first 3 months, surgical approach achieves better symptomatic relief than nonsurgical
options.
Follow-Up
Functional and cancer follow-up with repeated imaging at regular intervals.
Continuation of chemotherapy.
Cross-Reference
Positron emission tomography (PET/PET-CT) (Ch. 2)
Anorectal landmarks (Ch. 3)
Pelvic fascial structures and spaces (Ch. 3)
Carcinogenesis (Ch. 3)
Colorectal cancer—rectal cancer (154.1) (Ch. 4)
Colorectal cancer—liver metastases (197.7) (Ch. 4)
Colorectal cancer—lung metastases (197.3) (Ch. 4)
Low anterior resection (LAR)/total mesorectal excision (TME) (Ch. 5)
Transanal excision (polyp, cancer) (Ch. 5)
Transanal endoscopic microsurgery (TEM) (Ch. 5)
Chemotherapy protocols—curative intent (colorectal cancer) (Ch. 6)
Radiation therapy (Ch. 6)
Intensity-modulated radiation therapy (IMRT) (Ch. 6)
Follow-up for colorectal cancer (Ch. 6)
COLORECTAL CANCER—LIVER METASTASES (197.7)
Overview
Liver is the most common site for metastatic dissemination of GI malignancies (Figure 4–11). An aggressive workup and
management approach is justified in fit patients to improve short- and long-term survival. Systemic chemotherapy generally
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is indicated if tolerated. Hepatic arterial infusion chemotherapy offers no benefit. Surgical resection carries potential of cure
and long-term survival in 30–50% of selected patients (vs 1% 5-year survival if untreated).
Figure 4–11.
Extensive liver metastases.
Poor prognostic parameters:
Multiple metastases (> 4).
Bilateral liver metastases.
Short interval after primary resection.
Hilar lymph node metastases ("metastases from metastases").
Insufficient volume and quality of remnant liver tissue.
Ascites.
Timing of liver resection:
One-stage approach: simultaneous resection (with primary tumor) if both procedures relatively simple.
Two-stage approach: liver resection after 8–12 weeks of interval chemotherapy. Advantages: assessment of tumor
responsiveness to chemotherapy regimen, tumor downsizing with better resectability, elimination of rapidly progressive
disease patients.
Reversed two-stage approach (uncertain rational and benefit): liver resection first, resection of primary tumor after
interval chemotherapy.
Epidemiology
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Affects 40–50% of patients with colorectal cancer (CRC): 20% of CRC patients synchronous metastatic disease at time of
cancer diagnosis; 20–30% metachronous metastases after resection of the primary tumor.
Symptoms
Most commonly asymptomatic: detection intraoperatively during primary resection, or through imaging studies and CEA/liver
parameters during primary workup and/or follow-up.
Complications: liver failure (particularly after surgery) if > 50% of liver replaced by tumor; rarely: rupture of metastasis
intraperitoneal bleeding hemorrhagic shock, peritonitis, cancer seeding.
Differential Diagnosis
Nonmalignant liver lesions: cysts, hemangioma, Echinococcus, liver adenoma, focal nodular hyperplasia.
Hepatocellular carcinoma.
Metastases from noncolorectal primary tumor.
Pathology
Same as primary tumor.
Evaluation
REQUIRED MINIMAL STANDARD
History: patient's overall condition, performance status, comorbidities, operability.
Clinical examination: palpable tumor, jaundice, abdominal distention, cardiopulmonary assessment.
Blood work: anemia, liver function (albumin, PTT, PT), kidney function. Tumor marker: CEA.
Imaging studies for liver assessment and to rule out extrahepatic tumor disease:
– CT chest/abdomen/pelvis: hypodense focal lesions, 70–90% sensitivity.
– MRI (contrast-enhanced): 65–90% sensitivity.
– PET-CT: most accurate for staging and patient selection, > 90% sensitivity if performed before chemotherapy
administered.
ADDITIONAL TESTS (OPTIONAL)
Biopsy: tissue confirmation.
Biopsy of perifocal liver tissue: rule out liver cirrhosis.
Classification
Resectable liver metastases.
Potentially resectable metastases.
Nonresectable (based on number, distribution, proximity to vital structures).
Nonoperative Treatment
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Inoperable patients due to comorbidities and poor performance status.
Extrahepatic tumor manifestations (of uncurable extent).
Hepatic-only metastases that are:
Nonresectable.
Only potentially resectable.
palliative chemotherapy, reassessment for future resectability if chemoresponsive.
radiofrequency or cryoablation.
no benefit from: hepatic arterial infusion chemotherapy, ethanol injection, radiation, transplant.
Operative Treatment
INDICATIONS
Resectable metastases (unless prohibitive contraindications: comorbidities, extrahepatic tumor dissemination).
Resectability: R0 resection feasible, sparing of two adjacent liver segments possible, preservation of vascular in-
/outflow and biliary drainage possible, remnant liver of normal structure and > 20% of total liver volume.
OPERATIVE APPROACH
Intraoperative Management of Preoperatively Known Metastases
Diagnostic laparoscopy with laparoscopic intraoperative ultrasonography should be considered (particularly in absence
of top-quality preoperative imaging) as unnecessary laparotomy may be avoided in up to 25% of patients.
Intraoperative ultrasonography change or guidance of surgical procedure.
R0 resection: with goal of 1 cm safety margin, minimum margin of > 1 mm less ideal.
– Anatomic resection: hemihepatectomy, trisegmentectomy.
– Nonanatomic resection: metastasectomy, segmentectomy.
– Combinations: eg, hemihepatectomy + radiofrequency ablation of contralateral focus.
Intraoperative Management of Preoperatively Unknown Metastases
Resection if feasible (as above).
At least: Tru-cut biopsy for tissue confirmation.
Portal vein ligation: not commonly indicated.
Outcome
Five-year survival after resection of liver metastases: 30–50% (caveat: highly selected patients). Elective mortality of liver
resection: decreased from 20% to currently 1%.
Complications: bleeding, wound infections 5%, biliary leak, biloma, liver failure.
Follow-Up
Continuation of adjuvant/palliative chemotherapy indicated for:
All patients after metastasectomy.
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Patients ineligible for surgical resection as long as benefit > side effects.
Oncologic follow-up:
Colonoscopy: depending on overall prognosis and colonic condition.
Clinical examination and blood work (including CEA) as clinically indicated, at least every 3 months.
Imaging (CT chest/abdomen/pelvis, PET): depending on clinical course and treatment protocols.
Surgical reintervention:
Re-resection for recurrent liver metastases in highly selected candidates.
Resection of pulmonary metastases in selected patients.
Cross-Reference
Computed tomography (CT scan) (Ch. 2)
Positron emission tomography (PET/PET-CT) (Ch. 2)
Recurrent rectal cancer (789.3, 154.1) (Ch. 4)
Colorectal cancer—lung metastases (197.3) (Ch. 4)
Chemotherapy protocols—curative intent (colorectal cancer) (Ch. 6)
Chemotherapy—metastatic colorectal cancer (Ch. 6)
Follow-up for colorectal cancer (Ch. 6)
CEA monitoring for colorectal cancer (Ch. 6)
COLORECTAL CANCER—LUNG METASTASES (197.3)
Overview
Lung is the third most common site (after liver and peritoneal carcinomatosis) for metastatic dissemination of GI
malignancies including colorectal cancer. Pulmonary metastases most frequently represent surgically incurable tumor extent.
Systemic chemotherapy therefore is the principle treatment option and is indicated if tolerated.
Poor prognostic parameters:
Multiple metastases (> 4).
Bilateral lung metastases.
Short or no interval to primary tumor.
Insufficient residual lung function.
Surgical resection may play role in highly selected individuals who are fit and have a limited number of metastases with
relatively slow progression and no residual extrapulmonary tumor manifestations. Potential of cure and long-term survival
averages 38% (range 30–70%) of these highly selected patients (vs 5-year survival of < 1% in the whole group with lung
metastases).
Timing of lung resection: generally not indicated at the time of abdominal resection but rather 2-stage approach: lung
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resection after 12–16 weeks of interval chemotherapy. Advantages: assessment of tumor response to chemotherapy, tumor
downsizing with better resectability, elimination of nonbeneficial surgery in patients with unfavorable disease with rapid
extrapulmonary or intrapulmonary progression.
Epidemiology
Affects 10–15% of patients with colorectal cancer (CRC) metachronous metastases after resection of the primary tumor.
Symptoms
Most commonly asymptomatic detection during primary workup or follow-up.
Differential Diagnosis
Primary lung cancer.
Nonmalignant pulmonary nodule.
Pathology
Should be consistent with the primary tumor.
Evaluation
REQUIRED MINIMAL STANDARD
History: patient's overall condition, performance status, comorbidities (particularly cardiopulmonary, smoking, and other risk
factors for lung cancer), operability.
Clinical examination:
Abdominopelvic: rule locally recurrent tumor, jaundice, ascites, etc.
Cardiopulmonary assessment and pulmonary function tests: operability, decreased lung function, emphysema, COPD,
right ventricular congestion, etc.
Blood work: arterial blood gas analysis, anemia, liver function (albumin, PTT, PT), kidney function, tumor marker (CEA).
CT chest/abdomen/pelvis and/or PET-CT: rule out extrapulmonary tumor disease.
ADDITIONAL TESTS (OPTIONAL)
Bronchoscopy: if primary lung cancer more likely.
Biopsy (eg, CT-guided): tissue confirmation.
Classification
Resectable lung metastases.
Nonresectable (based on number, distribution, proximity to vital structures).
Nonoperative Treatment
Nonresectable pulmonary metastases (disseminated tumor disease, extrapulmonary tumor).
Inoperable patients due to comorbidities and poor performance status.
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Operative Treatment
INDICATIONS
Resectable metastases.
OPERATIVE APPROACH
Metastasectomy (rarely more extensive resection):
Video-assisted thoracoscopic surgery (VATS): faster recovery, higher miss rate.
Open resection: higher morbidity, lower miss rate (allows for palpation of lung tissue).
Outcome
Five-year survival after resection of lung metastases: 30–70% (caveat: highly selected patients).
Complications: bleeding, wound infections, air leak, respiratory failure.
Follow-Up
Continuation of adjuvant/palliative chemotherapy indicated for:
All patients after metastasectomy.
Patients ineligible for surgical resection as long as benefit > side effects.
Cross-Reference
Computed tomography (CT scan) (Ch. 2)
Positron emission tomography (PET/PET-CT) (Ch. 2)
Recurrent rectal cancer (789.3, 154.1) (Ch. 4)
Colorectal cancer—liver metastases (197.7) (Ch. 4)
Chemotherapy protocols—curative intent (colorectal cancer) (Ch. 6)
Chemotherapy—metastatic colorectal cancer (Ch. 6)
Follow-up for colorectal cancer (Ch. 6)
CEA monitoring for colorectal cancer (Ch. 6)
GASTROINTESTINAL STROMAL TUMOR (GIST; 171.9)
Overview
GIST is the most common mesenchymal tumor of the GI tracts and originates from the intestinal pacemaker cells, the
interstitial cells of Cajal. Distinguished from other mesenchymal tumors (eg, leiomyoma, schwannoma) through
immunohistochemistry identifying markers of GIST, which are equally expressed in interstitial cells of Cajal:
CD117 (KIT): gene product of stem cell factor receptor proto-oncogene c-kit.
CD34: hematopoietic progenitor cell antigen.
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PDGF receptor alpha (PDGFRA): alternative and mutually exclusive oncogenic mechanisms to c-kit pathway.
Malignant potential of GISTs is difficult to assess:
Favorable prognostic factors: gastric location, < 5 cm in diameter, mitotic index 5 mitoses per 10 HPFs, absence of
necrosis, low proliferating cell nuclear antigen, Ki-67 analogue index < 10%, confined tumor, lack of metastasis to
other sites.
Unfavorable prognostic factors: esophageal/colonic/rectal location, > 10 cm in diameter, mitotic index 10 mitoses
per 10 HPFs, presence of coagulative necrosis, invasion of adjacent organs, evidence of peritoneal seeding or distant
metastases.
GIST is highly resistant to conventional chemotherapy. Treatment is primarily surgical resection of localized GIST if tumor is
resectable without mutilation. Recurrent and locally advanced or metastatic tumors are increasingly treated with imatinib
(Gleevec) in palliative, adjuvant, or neoadjuvant setting. Tumor response imatinib (Gleevec, KIT kinase activity inhibitor)
correlates with expression of CD117.
Epidemiology
Constitutes 0.1–3% of all GI neoplasms. Estimated annual incidence: 10–15 new cases per 1 million people, ie, every year
approximately 3000–5000 new cases in the US. Median age: 50–60 years, no gender predominance.
GIST locations: stomach 50–60%, small intestine 25–30%, colon/rectum/rectovaginal septum 2–10%, esophagus 9%. Other
locations: retroperitoneal, pancreas, etc. Up to 50% of GISTs are metastatic/multifocal at the time of presentation.
Symptoms
Unspecific symptoms: pain, obstruction, bleeding, or visible/palpable mass.
Incidental finding on imaging or endoscopy studies.
Differential Diagnosis
Other benign mesenchymal tumors: leiomyoma, lipoma, neurofibroma (schwannoma).
Other malignant mesenchymal tumors: leiomyosarcoma, neurofibrosarcoma, etc.
Epithelial neoplasms.
Pathology
MACROSCOPIC
Solid or focally cystic mass of varying size: 1–20 cm.
Hemorrhage.
Focal necrosis.
Pattern of tumor dissemination: lymph node involvement comparably rare (< 25%); metastases to liver and
peritoneal cavity, less frequently to lung and bones.
MICROSCOPIC
Spindle cell, epithelioid, or pleomorphic (mixed) tumors ultrastructural and immunohistochemical distinction from
leiomyoma, leiomyosarcoma, or schwannoma.
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IMMUNOHISTOCHEMICAL POSITIVITY
CD117 (KIT): 75–80% (cytoplasm).
CD34 (hematopoietic progenitor cell antigen): 60–70%.
PDGFRA: 5–10%.
Conventional prognostic parameters: (1) tumor size, (2) mitotic rate (mitoses/number of HPFs). KIT positivity correlating
with imatinib response.
Evaluation
REQUIRED MINIMAL STANDARD
Full colonic evaluation (preferably colonoscopy) mandatory prior to elective surgery to rule out multifocal tumors,
synchronous cancers or polyps, rule out underlying colonic disease.
Imaging studies: CT, PET, MRI for assessment of local tumor extension and to rule out distant metastases.
ADDITIONAL TESTS (OPTIONAL)
Same as for CRC.
EGD.
Classification
Immunocharacterization: KIT-positive (GIST 85–95%) vs KIT-negative GIST (5–15%).
Tumor behavior: benign GIST vs malignant GIST.
Operable GIST vs inoperable or multifocal/metastatic GIST.
Nonoperative Treatment
Depending on tumor extent at presentation:
Potentially adjuvant treatment in all resected cases?
(Neo-)adjuvant therapy with imatinib (Gleevec, tyrosine kinase inhibitor) for borderline-resectable GIST.
Recurrent, locally advanced, and unresectable or metastatic GIST: palliative Gleevec treatment, results in 45–80%
initial response.
No defined role for radiation therapy.
Operative Treatment
INDICATIONS
Any localized GIST that allows complete resection without mutilation unless diffuse metastases or prohibitive
contraindications.
Multifocal GIST only in highly selected cases (after neoadjuvant imatinib treatment).
OPERATIVE APPROACH
En-bloc resection of tumor-involved organ with inclusion of intact pseudocapsule; radical lymphadenectomy not routinely
indicated.