Introduction
Cancer of the cervical esophagus has long been known as one of the most difficult neoplasms to treat and cure surgically. It is relatively uncommon, representing less than 5% of all esophageal cancers, themselves representing 5% of all digestive system cancers. In 2010 in the United States there were an estimated 16,640 new cases of esophageal carcinoma, with 14,500 deaths, thus explaining the paucity of data with cervical esophageal carcinoma. Patients often present with advanced-stage cancer and several comorbidities. The aggressive clinical course of this disease is influenced by the high incidence of submucosal spread, ipsilateral or bilateral lymph node metastasis, and the high rate of a second primary cancer in the esophagus. Involvement of the larynx and trachea is often encountered, as a distant metastasis. The surgical defects are often quite significant and most of the time circumferential, necessitating challenging reconstructive planning. A multidisciplinary team is therefore required for the proper management of patients with cancer of the cervical esophagus, including a head and neck surgeon, thoracic surgeon, reconstructive surgeon, medical oncologist, radiation oncologist, speech language pathologist, nutritionist, and physiotherapist. As such, the patients’ treatment remains personalized and tailored to their needs, with the main goal being that of curing while attempting to allow for functional rehabilitation to maximize the patients’ quality of life.
Squamous cell carcinoma is the most frequent histologic type of cancer found in the cervical esophagus. Primary adenocarcinoma of the cervical esophagus is rare and may arise from Barrett esophagus. Less common are tumors that arise from heterotopic gastric mucosa without evidence of Barrett disease. Benign lesions occur infrequently, the most common being pedunculated hyperplastic or fibrovascular polyps. Benign lesions are most often removed by endoscopic excision.
Key Operative Learning Points
- •
Cancer of the cervical esophagus is a rare disease and may be a direct extension of a hypopharyngeal lesion.
- •
Early diagnosis is one of the most important factors for survival.
- •
Posttreatment quality of life, in association with functional outcomes, should be a key discussion point before choosing to move ahead with extensive surgery.
- •
Computed tomography (CT) is essential in the preoperative evaluation of a patient.
- •
Invasion of the prevertebral fascia must be ruled out before, or at the beginning of, any surgical intervention.
- •
Any level of invasion is a strict contraindication to surgery.
- •
- •
Encasement of the carotid artery is a strict contraindication to surgery.
- •
The presence of distal cervical esophageal or proximal thoracic esophageal cancer involvement is an indication for total esophagectomy.
Preoperative Period
History
- 1.
History of present illness
- a.
Patients with cervical esophageal cancer often have vague and nonlocalizing symptoms, while also being known as long-term smokers and drinkers, thus requiring a thorough head and neck oncologic questionnaire, including the following signs and symptoms:
- 1)
Sore throat
- 2)
Hoarseness
- 3)
Gastroesophageal reflux
- 4)
Heartburn/chest pain
- 5)
Difficulty swallowing: dysphagia, odynophagia, aspiration, globus sensation
- 6)
Hemoptysis
- 7)
Weight loss; often associated with advanced disease
- 8)
Dyspnea
- 9)
Referred otalgia
- 10)
Mass in the neck
- 1)
- b.
A clear timeline of the signs and symptoms is important to collect because it often provides a preliminary idea of the evolution and progression of the cancer.
- c.
More than one-third of patients with early-stage cancer will be asymptomatic at presentation.
- a.
- 2.
Past medical history and associated risk factors
- a.
Medical illnesses
- b.
Surgeries: oropharyngeal, laryngeal, or hypopharyngeal
- c.
Previous head and neck radiation
- d.
Family history of cancer
- e.
Risk factors: tobacco (cigarette or pipe smoking), alcohol abuse, Plummer-Vinson syndrome, lower socioeconomic class, and lower educational levels. Heavy alcohol and tobacco abuse increases the risk of cancer of the head and neck.
- a.
Physical Examination
- 1.
Appearance: Patients will often seem malnourished, dehydrated, and with poor oral hygiene.
- 2.
The oral cavity and oropharynx should always be examined to avoid overlooking any asymptomatic second primary cancer or direct extension from a large cancer.
- 3.
Mirror examination and flexible endoscopy
- a.
Evaluation of the base of the tongue with proper protrusion of the tongue
- b.
Vallecula
- c.
Hypopharynx: requires a thorough evaluation reviewing bilateral vocal cord mobility, any extension into the pyriform sinuses (using a puffed cheek maneuver), postcricoid region, and/or the posterior pharyngeal wall. Pooling of secretions is often encountered as a result of distal obstruction by the cancer.
- a.
- 4.
Palpation of the neck is performed to rule out regional metastasis. Paratracheal nodes are a common site of metastasis from cervical esophagus cancer.
Imaging
Imaging studies contribute to the diagnosis and staging while giving information about whether the cancer appears to be technically operable. Although flexible endoscopy provides a direct visualization of the mucosa, it is the imaging that will be most helpful in staging the cancer.
- 1.
Esophagography (barium esophagogram)
- a.
Used to determine the extent of mucosal involvement and to establish the presence of a second primary cancer in the more distal aspect of the esophagus ( Fig. 50.1A )
Fig. 50.1
A, Barium esophagogram demonstrating carcinoma of the cervical esophagus (arrow) . B, Computed tomography scan of same patient demonstrating the cancer with narrowing of the lumen of the cervical esophagus (arrow) .
- b.
The most valuable aspect of this imaging modality is the evaluation of invasion of the posterior wall of the esophagus.
- c.
Obliteration of the retropharyngeal and retroesophageal space or invasion of the prevertebral fascia can be estimated through dynamic fluoroscopic evaluation.
- d.
In the presence of aspiration, it would be prudent to carry out a diatrizoate meglumine (Gastrografin) esophagogram rather than a barium esophagogram because less reaction occurs if the patient aspirates Gastrografin into the lungs than if barium is aspirated.
- a.
- 2.
CT scan with intravenous contrast enhancement of the head, neck, and chest. All newly diagnosed cancers of the head and neck require at the least a CT of the chest before staging (see Fig. 50.1B ).
- a.
Most widely used imaging technique for cancer of the cervical esophagus
- b.
CT of the neck should be performed using maximum 3-mm slice thickness, whereas chest CT can be done with 5- to 7-mm slice thickness.
- c.
Goal: accurately evaluate the location and size of the primary cancer and determine whether there is direct extension of tumor into the neck, especially posteriorly into the retropharyngeal and esophageal space, prevertebral fascia, or vertebral column ( Fig. 50.2 )
Fig. 50.2
Squamous cell carcinoma of the cervical esophagus with extensive invasion of surrounding structures in the neck (arrow) .
- d.
Key points:
- 1)
An anteroposterior diameter of the cervical esophagus greater than 24 mm must be considered abnormal.
- 2)
The average thickness of the wall is 4.8 mm laterally and 3.8 mm posteriorly.
- 3)
Thickening of the esophageal wall and effacement of the adipose tissue plane are the two criteria with the best sensitivity.
- 4)
Presence of a circumferential mass surrounding the esophagus greater than 180 or 270 degrees has a reported sensitivity of 100%.
- 1)
- e.
Cancer of the cervical esophagus most often metastasizes to paratracheal, jugular chain, recurrent laryngeal nodes and superior mediastinal lymph nodes.
- a.
- 3.
Magnetic resonance imaging (MRI) may provide greater soft tissue delineation and insight toward prevertebral fascia invasion; however, it does not provide additional staging information than does a CT scan. Some surgeons may prefer this modality to CT scan for initial staging.
- 4.
Positron emission tomography (PET)-CT scan is an imaging tool that is now routinely used for staging cancer of the head and neck, as well as for evaluation of metastatic disease. It should be considered in the workup of cervical esophageal cancer, as well as in monitoring active response in treated patients. Having a trained radiologist or nuclear medicine specialist reviewing the PET-CT images is of utmost importance due to their complexity and the high rate of false-positive readings.
- 5.
Ultrasonography (US) may be used to provide complementary information to the aforementioned imaging techniques via a transesophageal evaluation of the tissues. A US assessment of cervical lymph node metastasis can be made coupled with fine-needle aspiration biopsy to provide valuable information in the staging process.
- 6.
Endobronchial US (EBUS) with fine-needle aspiration for more difficult to attain suspicious lymph nodes (paratracheal, subcarinal, paraesophageal) is often used in our center and allows for a more accurate staging of the disease.
Indications
- 1.
The cancer is resectable with only regional metastases.
- 2.
Patient fit for extensive surgical procedure with challenging and lengthy postoperative recovery period
Contraindications
- 1.
Severe medical comorbidities rendering the patient unfit for surgery (poor nutritional status, medically unstable)
- 2.
Encasement of the carotid artery visualized in the preoperative imaging is a definite contraindication to surgery. However, if the surgeon unexpectedly finds encasement of the carotid arteries intraoperatively, the extirpative part of the procedure should be completed with the understanding that it will be palliative rather than curative surgery.
- 3.
Involvement of the prevertebral fascia or vertebral column. This should be assessed clinically at the beginning of surgery by using finger dissection of the hypopharynx and esophagus from the prevertebral fascia. If the cervical esophagus cannot be separated easily from the prevertebral fascia, cure is unlikely.
- 4.
Distant metastases
Preoperative Preparation
- 1.
Complete medical preoperative evaluation, including an electrocardiogram, complete blood count, liver function, and renal function tests is required. As previously mentioned, patients often present with severe comorbidities, such as advanced chronic obstructive pulmonary disease secondary to long-term cigarette smoking or cirrhosis of the liver due to chronic alcoholism. Preoperative evaluation by the internal medicine team is essential.
- 2.
Before surgery, patients should be optimized accordingly:
- a.
Patients with low hemoglobin should undergo transfusion.
- b.
Nutritional status should be evaluated with a referral to a medical nutritionist, if deemed severe.
- a.
- 3.
A diagnostic endoscopy and biopsy should be performed under general anesthesia, although some of these patients may also have upper airway obstruction or an unstable airway because of bilateral vocal cord paralysis, in which case the endoscopy should be preceded by a tracheostomy under local anesthesia. Tracheoscopy is also performed to evaluate the integrity of the tracheal wall.
- 4.
Staging of the cancer should be performed after the physical examination, imaging, and biopsies are available. Staging for a cancer of the cervical esophagus does not resemble that of cancers of the hypopharynx and instead follows the latest National Comprehensive Cancer Network published clinical practice guidelines ( Box 50.1 ).
BOX 50.1
Tx: Unable to assess the primary tumor
T0: Unable to find the primary tumor
Tis (HGD): High-grade dysplasia (HGD) is abnormal cells that have not invaded deeper cell layers. This term has replaced the term carcinoma in situ for columnar mucosa in the gastrointestinal tract.
T1a: Tumor invades into the lamina propria or muscularis mucosa
T1b: Tumor invades the submucosa
T2: Tumor invades the muscularis propria
T3: Tumor invades the adventitia
T4a: Resectable tumor invading the pleura, pericardium, or diaphragm
T4b: Unresectable tumor that invades other structures, such as aorta, spine, trachea
Nx: Neck lymph nodes cannot be assessed.
N0: No evidence of any spread to nodes
N1: 1 or 2 cancerous nodes in the region
N2: 3 to 6 cancerous nodes in the region
N3: 7 or more cancerous nodes in the region
M0: No evidence of distant spread
M1: Evidence of spread outside of the head and neck (lungs, bone, brain)
Staging of Cervical Esophageal Cancer
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