THE KURSK STATE MEDICAL UNIVERSITY

Department of surgical diseases № 1

ESOPHAGEAL TUMORS AND CYSTS

Information for self-training of English-speaking students

The chair of surgical diseases N 1 (Chair-head - prof. S.V.Ivanov)

By ass. professor M.V. Yakovleva

KURSK-2010

I INTRODUCTION

Benign tumors of the esophagus are rare, constituting only 0.5 to 0.8% of all esophageal neoplasms. Approximately 60% of benign esophageal neoplasms are leiomyomas, 20% are cysts, and 5% are polyps. Despite advances in surgery, critical care, radiotherapy, and chemotherapy, esophageal cancer afflicts some 13,000 new patients in the United States each year.

The disease represents 4% of newly diagnosed cancers. А major shift in the histologic type of tumors has occurred. Traditionally, esophageal cancer has been squamous cell in patients with the usual risk factors for other aerodigestive tract carcinomas, specifically smoking (5-fold) and alcohol (5-fold) abuse. Heavy smoking and heavy drinking combine to increase the risk 25- to 100-fold.
II GENERAL AIM OF THE LESSION
General aim of the lesion includes:
1.  Acquiring knowledge about etiology and clinical symptoms of the benign tumors and esophageal cancer.
2.  Acquiring the practical skills of the patients objective examination.
3.  Mastering of the main instrumental used in this pathology.
4.  Determining the indications for conservative, palliative and surgical curative treatment.
Tasks for self –training:
After individual studying of the material every student have to
A/know: 1) Etiology of the benign tumors and esophageal cancer.
2) Classification of the esophageal benign tumors and cancer.
3) Clinical picture of this pathology.
4) Instrumental methods for diagnostics, such as: X-ray examination,
esophagoscopy, CT-scaning, ultrasonography.
5) Different kinds of the palliative treatment of the esophageal cancer.
6) Surgical curative treatment of the tumors.
7) Indications for different surgical operations in depending on cancer stage.
B/be able: 1) to find out main complains and assess the present state of the patient;
2) to realize objective examination of the patient with these diseases;
3) to estimate received data of instrumental methods of examination;
4) to determine indications for palliative and surgical treatment.
IV BRIEF OUTLINE OF THE TOPIC ( OBLIGATORY MATERIAL FOR ACQUISITION )
Benign Esophageal Tumors
Classification of Benign Esophageal Tumors
I. Epithelial tumors
A. Papillomas
B. Polyps
C. Adenomas
D. Cysts
II. Nonepithelial tumors
A. Myomas
1. Leiomyomas
2. Fibromyomas
3. Lipomyomas
4. Fibromas
B. Vascular tumors
1. Hemangiomas
2. Lymphangiomas
C. Mesenchymal and other tumors
1. Reticuloendothelial tumors
2. Lipomas
3. Myxofibromas
4. Giant cell tumors
5. Neurofibromas
6. Osteochondromas
III. Heterotopic tumors

Leiomyomas are the most common benign tumors of the esophagus. These intramural tumors typically occur between 20 and 50 years of age, and are multiple in 3 to 10% of patients. More than 80% of these tumors occur in the middle and lower thirds of the esophagus, rarely in the cervical region. Histologically, the tumors consist of interlacing bundles of smooth muscle cells with or without calcification. These tumors do not infiltrate surrounding tissue, so the overlying mucosa is rarely, if ever, invaded. [144]

DIAGNOSIS

Symptoms of dysphagia and vague retrosternal pressure or pain are produced only by large tumors (larger than 5 cm). Most are found incidentally at autopsy and are asymptomatic. Esophageal symptoms prompt performance of a barium swallow and/or an endoscopic examination. The barium swallow appearance is distinctive because the well-localized mass has a smooth surface and distinct margins, and it is not circumferential. Most frequently, a leiomyoma is seen on a chest x-ray as a posterior mediastinal mass or is found unexpectedly during endoscopic examination. During endoscopy, the mucosa is intact, and the extrinsic mass narrows the lumen but can easily be displaced and passed with the esophagoscope.

TREATMENT.

As a general rule, excision of symptomatic leiomyomas or those larger than 5 cm is advised. Asymptomatic or smaller tumors discovered incidentally can be observed and followed.

Esophageal resection may be required for eigher giant leiomyomas of the cardia or for leiomyomatosis.The results of resection of leiomyomas are excellent, and recurrence has not been reported.

Polyps of the cervical esophagus (20% of benign tumors) are intraluminal lesions that may cause dysphagia or may even be regurgitated into the larynx with the potential for asphyxiation. They are composed of a fibroelastic core and usually are covered with normal epithelium. The preferred approach for resection is through a lateral cervical esophagomyotomy, thereby delivering the polyp and resecting the mucosal origin of the pedicle under direct vision. Esophageal polyps have also been removed endoscopically by electrocoagulating the pedicle. Lipomas, vascular tumors, and neurofibromas are extremely rare, but they must be removed to control symptoms or to exclude malignancy.

Esophageal Cancer

When resection is indicated, benign tumors of the middle third of the esophagus are approached through a right thoracotomy; those in the distal third are approached through a left thoracotomy.

Carcinoma of the esophagus now appears to affect younger, healthier patients. Nutritional factors and potential carcinogens have been incriminated, including alcohol, tobacco, zinc, nitrosamines, malnutrition, vitamin deficiencies, anemia, poor oral hygiene and dental caries, previous gastric surgery, and long-term ingestion of hot foods or beverages. Some esophageal lesions are premalignant, including achalasia, reflux esophagitis, Barrett's (columnar epithelial-lined) esophagus, [142] radiation esophagitis, [145] caustic burns, Plummer-Vinson syndrome, leukoplakia, esophageal diverticula, and ectopic gastric mucosa.

The extensive mediastinal lymphatic drainage, which communicates with cervical and abdominal collateral vessels, is responsible for the finding of mediastinal, supraclavicular, or celiac lymph node metastasis in at least 75% of patients with esophageal carcinoma. Cervical esophageal cancers drain to the deep cervical, paraesophageal, posterior mediastinal, and tracheobronchial lymph nodes. Lower esophageal tumors spread to paraesophageal, celiac, and splenic hilar lymph nodes. Distant spread to liver and lungs is common.

Histologically, approximately 95% of esophageal cancers worldwide are squamous cell carcinomas. Early forms of esophageal cancer have been variously termed carcinoma in situ, superficial spreading carcinoma, and intramucosal carcinoma. Endoscopically, carcinoma in situ most often presents as a slightly raised, granular, reddish, plaquelike lesion.

Squamous cell carcinoma arises from the mucosa of the esophagus. Located mainly in the thoracic esophagus, approximately 60% of these tumors are found in the middle third and about 30% in the distal third. Squamous cell neoplasms have four major gross pathologic presentations. (1) fungating: predominantly intraluminal growth with surface ulceration and extreme friability that frequently invades mediastinal structures; (2) ulcerating: flat-based ulcer with slightly raised edges; hemorragic, friable with surrounding induration; (3) infiltrating: a dense, firm, logitudinal and circumferential intramural growth pattern; and (4) polypoid: intraluminal polypoid growth with a smooth surface on a narrow stalk (fewer than 5% of cases).

Adenocarcinoma is now the most common cell type of esophageal cancer in the United States. Adenocarcinoma arises from the superficial and deep glands of the esophagus, mainly in the lower third of the esophagus, especially near the gastroesophageal junction. Men have an eightfold higher risk than women. Esophageal adenocarcinoma may have one of three origins: (1) malignant degeneration of metaplastic columnar epithelium (Barrett's mucosa), (2) heterotopic islands of columnar epithelium, or (3) the esophageal submucosal glands.

Symptoms of Esophageal Cancer /
Symptom /
Dysphagia
Weight loss
Vomiting or regurgitation
Pain
Cough or hoarseness
Dyspnea

The tumor may be advanced sufficiently to be identified on a chest x-ray as an abnormal azygoesophageal recess, widening of the mediastinum, or posterior tracheal indentation. A barium swallow will show the extent of the tumor and location, if the tumor distorts the esophageal lumen, and the presence of obstruction or fistulas. The CT or endoscopic ultrasound examination can determine the anatomic location and enlargement of the mediastinal, perigastric, or celiac lymph nodes. Esophagoscopy is required to diagnose and determine the extent of longitudinal intramural tumor spread. The entire esophagus is visualized, and brush cytology plus biopsy tissue samples may be obtained for histologic analysis.

Once the diagnosis of esophageal carcinoma has been histologically established after esophagoscopy and biopsy, staging of the tumor is the next critical step in determining which therapeutic option is appropriate. The stage of a tumor is classified most frequently by a TNM-based system. The "T" (tumor) indicates the progressive degree (1 to 4) of invasion of the tumor into the esophageal wall. "N" stands for nodal involvement, and "M" represents distant metastasis.

Lymph node involvement may be assessed by endoscopic ultrasound, CT, positron emission tomography (PET), or video-assisted thoracoscopy and laparoscopy. Endoscopic ultrasound can assess the size, shape, border, and internal echo characteristics of the lymph node. CT and endoscopic ultrasound imaging alone rely on the anatomic size of the node as a predictor of malignancy, but they cannot differentiate between hyperplastic nodes and nodes enlarged because of metastasis. Endoscopic ultrasound and CT can then be used for image-directed fine-needle aspiration of mediastinal or celiac nodes.

Stage Grouping of Esophageal Cancer
Stage 0 / T 0 N 0
T is N 0 M0
Stage I / T 1 N 0 M0
Stage II / IIA T 2 N0 M 0
T 3 N 0 M0
IIB T 1 N 1 M0
T 2 N 1 M0
Stage III / T 3 N 1 M0
T 4 any N M 0
Stage IV / any T any N M 1
T: PRIMARY TUMOR
T 0 No evidence of a primary tumor
T is Carcinoma in situ (high-grade dysplasia)
T 1 Tumor invading the lamina propria, muscularis mucosae, or submucosa but not breaching the boundary between submucosa and muscularis propria
T 2 Tumor invading muscularis propria but not breaching the boundary between muscularis propria and periesophageal tissue
T 3 Tumor invading periesophageal tissue but not adjacent structures
T 4 Tumor invading adjacent structures
N: REGIONAL LYMPH NODES
N 0 No regional lymph node metastasis
N 1 Regional lymph node metastasis
M: DISTANT METASTASIS
M 0 No distant metastasis
M 1 Distant metastasis
DISTANT METASTASIS (M STAGE)

Endoscopic ultrasound is especially suited to visualize lymph nodes around the celiac axis and the left liver lobe (both considered distant metastases). CT is specific for liver, lung, and pleural metastases larger than 2 cm in diameter, [165] but evaluation with fine-needle aspiration or transbronchial biopsy is necessary for the determination of malignancy. Bronchoscopy is required for patients with tumors of the upper and middle third of the esophagus to view the pharynx, larynx, and tracheobronchial tree for synchronous and metachronous malignancies. If a patient complains of bone pain, a bone scan should be performed. [57]

The plain chest x-ray is abnormal in only 50% of patients with esophageal cancer, with findings such as an air-fluid level in the obstructed esophagus in the posterior mediastinum, a dilated esophagus, abnormal mediastinal soft tissue representing adenopathy, a pleural effusion, or pulmonary metastasis being most common. The chest film, however, may be deceivingly normal even in patients with advanced disease.

Treatment

Curative efforts include surgery, chemotherapy, radiation, or a combination of these techniques; however, despite multitudes of clinical trials and retrospective reviews, no treatment modality alone has proved superior. Current trials have focused on radiation and chemotherapy with or without resection. Therapy for esophageal carcinoma is influenced by the knowledge that in most of these patients, local tumor invasion or distant metastatic disease precludes cure. In fact, 85 to 95% of patients have lymph node involvement at the time of surgical resection. Fewer than 10% of patients with lymph node involvement survive for 5 years. [146] In the past, palliative techniques were advocated because of the poor long-term survival rates of patients with esophageal carcinoma. Palliation affords the patient the ability to swallow (at least saliva) and perhaps to resume a normal life for 9 to 12 months. After the initial evaluation for staging, the physician can assess whether palliative or curative approaches are indicated.

PALLIATIVE TREATMENT

Palliation is appropriate when patients are too debilitated to undergo surgery or have a tumor that is unresectable because of extensive invasion of vital structures, recurrence of resected or irradiated tumor, and/or metastases. Most of these patients have complete or partial obstruction of the esophagus resulting from the tumor, and swallowing is painful or impossible. The goal of palliation is to use the most effective and least invasive means possible to relieve dysphagia and discomfort, to support nutrition, and to limit hospitalization. Palliation includes dilatation, intubation, photodynamic therapy, radiotherapy with or without chemotherapy, surgery, and/or laser therapy.

CURATIVE TREATMENT

] At best, only 50% of patients are eligible for a curative resection at presentation. [2] The lymphatic drainage of the esophagus is extensive, both within the esophageal wall and in the surrounding mediastinal tissues. As a result, longitudinal extension of the esophageal carcinoma may be extensive, and tumors may be multicentric. In 10% of patients, tumor recurs at the resection margin in patients who have had a 6- to 8-cm margin of normal esophagus removed. If an esophagectomy is indicated, three major technical approaches are available: (1) a transthoracic esophagectomy, (2) transhiatal esophagectomy without a thoracotomy, and (3) an en bloc radial esophagectomy. Although no consensus has been formed on the preferred technique, transthoracic esophagectomy is preferred by most thoracic surgeons.

Transthoracic esophagectomy is still preferred by most thoracic surgeons because it allows complete lymph node dissection under direct vision, complete resection of tumor mass and adjacent tissue, and complete staging of the tumor. . Great care is taken to avoid any damage to the recurrent laryngeal nerve, to avoid hoarseness.

The two major complications are similar to transhiatal and transthoracic esophagectomy: anastomotic leak and respiratory complications.

Because of the risks associated with the more radical transthoracic or en bloc esophagectomies and the overall low survival rate of patients with esophageal carcinomas, transhiatal esophagectomy without thoracotomy was proposed.

The advantages of this approach are as follows: (1) a thoracotomy is avoided, thus minimizing the physiologic insult of the operation; (2) an intrathoracic esophageal anatomosis is avoided, and if a cervical leak does occur, it is more easily managed and rarely causes mediastinitis or fatal complications; (3) no intra-abdominal or intrathoracic gastrointestinal suture lines are present; and (4) clinically significant gastroesophageal reflux seldom occurs after a cervical esophagogastric anastomosis.