Malignant Disorders of the Esophagus

Saint Barnabas Medical Center Frank Nami, M.D.

Esophageal Cancer

Most esophageal tumors are malignant, fewer than 1% are benign 13,000 new patients in the United States each year, and almost matching that figure is the expected death rate of 12,000 patients

Esophageal Cancer

Most North American patients still present with locally advanced (stage T 3 and/or N 1 ) disease Within North America and Europe, the incidence of adenocarcinoma rose 100% in the 1990s, and it had a strong correlation with reflux, Barrett's metaplasia, and dietary factors (e.g., fat).

Esophageal Cancer

Squamous cell still persists 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.

Risk Factors

CONSUMPTION OF:
Tobacco, Alcohol

UNDER-CONSUMPTION OF:
Fruits, Fresh meat, Riboflavin. Beta-carotene, Vitamin C, Magnesium, Vegetables, Fresh fish, Niacin, Vitamin A, Vitamin B complex, Zinc

Risk Factors

PREDISPOSING CONDITIONS:
Caustic injury, Esophageal webs, Achalasia, Barrett's esophagus, Esophageal diverticula

OTHER EXPOSURE:
Asbestos, Ionizing radiation, Exceptionally hot beverages (tea), Location: Middle East, South Africa, northern China, southern Russia, India

Anatomy of Esophagus

Lymphatics of Esophagus

Squamous Cell Carcinoma

95% of esophageal cancer worldwide Commonly 7th decade of life, 1.5-3 times more common in men Thought to occur from prolonged exposure of esophageal mucosa to noxious stimuli in persons with a genetic predisposition to the disease.

Squamous Cell Carcinoma

Histologically, characterized by invasive sheets of cells that run together and are polygonal, oval, or spindle-shaped with a distinct or ragged stromal-epithelial interface. 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 Carcinoma

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; hemorrhagic, friable with surrounding induration

Squamous Cell Carcinoma

(3) infiltrating: a dense, firm, longitudinal and circumferential intramural growth pattern (4) polypoid: intraluminal polypoid growth with a smooth surface on a narrow stalk (fewer than 5% of cases) A 5-year survival of 70% is associated with the polypoid tumor compared with a less than 15% 5-year survival for all other types

Adenocarcinoma

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.

Adenocarcinoma

Whites are at four times greater risk than blacks Men have an eightfold higher risk than women. In the US and Europe, frequency of this tumor is increasing faster than any other cancer.

Adenocarcinoma

Esophageal adenocarcinoma may have one of three origins:

malignant degeneration of metaplastic columnar epithelium (Barrett's mucosa) heterotopic islands of columnar epithelium the esophageal submucosal glands.

Adenocarcinoma

Gastric adenocarcinoma may also involve the esophagus secondarily. Gastroesophageal junction tumors arise initially as flat or raised patches of mucosa. They may subsequently ulcerate and become large (up to 5 cm) nodular masses. Tumor size is related to prognosis. For tumors smaller than 5 cm, 40% are localized, 25% have spread beyond the esophagus, and 35% have metastasized or are unresectable. For tumors that are more than 5 cm in length, 10% are localized, 15% have invaded mediastinal structures, and 75% have metastasized.

Rare esophageal cancers

Anaplastic small cell (oat cell) carcinoma arise in the esophagus from same argyrophilic cells found in the lung. Adenoid cystic esophageal carcinoma Primary malignant melanoma of esophagus Carcinosarcoma, features of SSC and malignant spindle cell sarcoma.

Clinical Findings

Dysphagia in more than 90% of patients with esophageal cancer Nonspecific retrosternal discomfort Indigestion Weight loss Pain Regurgitation, resp symptoms, hoarseness

Clinical Findings

Symptom Dysphagia Weight loss Vomiting or regurgitation Pain Cough or hoarseness Dyspnea

Percent 87-95 42-71 29-45 20-46 7-26 5

Dysphagia

Barium swallow evaluation

Mucosal irregularity Tumor shelf

Endoscopic evaluation
Esophageal biopsy and brushings for cytology Establishes diagnosis in 95% of patients with malignant strictures

Clinical Findings

Careful examination of cervical and supraclavicular lymph nodes FNA or excisional biopsy for diagnosis Evaluate for abdominal masses and liver nodularity

Labwork, imaging studies

Imaging Studies

Barium swallow exam

Imaging Studies

Computed tomography (CT) of the chest and upper abdomen is the standard radiographic technique for staging esophageal cancer. Normal esophageal wall thickness 5mm Regional adenopathy Metastasis to lung, liver, adrenal, or distant nodes FNA biopsy for tissue diagnosis

Imaging Studies

Positron emission tomography (PET) Does not rely on anatomic or structural distortion for detecting malignancy PET is 88% sensitive, 93% specific, and 71 to 91% accurate for identifying distant metastasis

Imaging Studies

Cellular FDG uptake is not specific for tumors and that areas of inflammation often predispose to false-positive results MRI has a 56 to 74% accuracy in detecting lymph node metastases

Endoscopic Ultrasound

Method of choice to determine depth of tumor invasion and regional nodal disease and involvement of adjacent structures, with an overall accuracy to 92% A significant error associated with endoscopic ultrasound T staging is to overstage 7 to 11% of early disease

Endoscopic Ultrasound

Algorithm

TNM Staging

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

TNM Staging

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

Stage Grouping

Stage 0

T0N0 T is N 0 M0 T 1 N 0 M0 IIA T 2 N0 M 0 T 3 N 0 M0 IIB T 1 N 1 M0 T 2 N 1 M0

Stage I Stage II

Stage Grouping

Stage III

T 3 N 1 M0 T 4 any N M 0 any T any N M 1

Stage IV

5 Year Survival

Stage I Stage IIA Stage IIB Stage III Stage IV

50-55% 15-35% 15-27% 4-15% 0-2%

Treatment Options

Palliative Treatment for unresectable lesions include:

Dilatation Stenting Photodynamic therapy Radiation therapy Laser therapy Surgical palliation

Treatment Options

Curative resection? Mid esophagus approached from right Distal esophagus from left

Ivor-Lewis combined right thoracic and abdominal incisions for mid esophagus

Mid-Esophageal Tumor

Upper Esophageal Tumor

Stomach Mobilization

Esophageal Substitution

Esophageal Substitution