MALIGNANT NEOPLASMS

adenocarcinoma, squamous cell carcinoma, small cell carcinoma, leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma, liposarcoma, lymphomas

Carcinoma of the Esophagus

Squamous carcinoma accounts for the majority of esophageal carcinomas worldwide.

Squamous carcinoma
arise from the squamous mucosa that is native to the esophagus and is found in the upper and middle third of the esophagus 70% of the time. This type of cancer is due to exposure to environmental factors. Smoking and alcohol both increase the risk for foregut cancers by 5-fold..

Squamous carcinoma
Combined, the risk increases from 25- to 100-fold. Food additives, including nitrosamines found in pickled and smoked foods, long-term ingestion of hot liquids, and vitamin (vitamin A) and mineral deficiencies (zinc and molybdenum) have been implicated.

Squamous carcinoma
Other disorders that expose the esophagus to mucosal trauma including caustic ingestion, achalasia, bulimia, tylosis (an inherited autosomal dominant trait), Plummer-Vinson syndrome, externalbeam radiation, and esophageal diverticula all have known associations with squamous cell cancer

Sq.cell carcinoma
The environmental factors: -local foodstuffs (nitroso compounds in pickled vegetables and smoked meats) and mineral deficiencies (zinc and molybdenum) have been suggested. , - smoking and alcohol consumption achalasia, - lye strictures, tylosis (an autosomal dominant disorder characterized by hyperkeratosis of the palms and soles), and - humanpapillomavirus.

Squamous carcinoma
The 5-year survival rate varies but can be as good as 70% with polypoid lesions and as poor as 15% with advanced tumors

Adenocarcinoma
now accounts for more than 50% of esophageal cancer in most Western countries. Its prevalence is exploding, largely secondary to the well-established association between gastroesophageal reflux, BE, and esophageal adenocarcinoma

Adenocarcinoma
Intake of caffeine, fats, and acidic and spicy foods all lead to decreased tone in the LES and an increase in reflux. As an adaptive measure, the squamous-lined distal esophagus changes to become lined with metaplastic columnar epithelium (Barrett's esophagus). Progressive changes from metaplastic (Barrett's esophagus) to dysplastic cells may lead to the development of esophageal adenocarcinoma

Clinical Manifestations
Dysphagia,
With tumors of the cardia, anorexia and weight loss usually precede the onset of dysphagia Asymptomatic ( identified on surveillance endoscopy), . Stridor
.

(Extension into the tracheobronchial tree)

Clinical Manifestations
Coughing, choking, and aspiration pneumonia result (fistula) Severe bleeding (erosion into the aorta or . pulmonary vessels Vocal cord paralysis . Metastases are usually manifested by jaundice or bone pain.

Clinical Manifestations
Dysphagia usually presents late in the natural history of the disease, because the lack of a serosal layer on the esophagus allows the smooth muscle to dilate with ease. As a result, the dysphagia becomes severe enough for the patient to seek medical advice only when more than 60% of the esophageal circumference is infiltrated with cancer. Consequently, the disease is usually advanced if symptoms herald its presence. Tracheoesophageal fistula may be present in some patients on their first visit to the hospital, and more than 40% will have evidence of distant metastases..

Clinical Manifestations
The physical signs of esophageal tumors are Virchow,s L.Ns Distant metastases

Clinical Staging
Clinical factors that indicate an advanced stage of carcinoma and exclude surgery with curative intent are recurrent nerve paralysis, Horner's syndrome, persistent spinal pain, paralysis of the diaphragm, fistula formation, and malignant pleural effusion.

General Approach to Esophageal Cancer

Therapy of esophageal cancer is dictated by the stage of the cancer 1- confined to the esophagus, (T1T2, N0), 2- advanced (T13, N1), 3-or disseminated (any T, any N, M1). If cancer is confined to the esophagus, removal of the tumor with adjacent LNs may be curative.

Diagnosis
Diagnosed with endoscopic biopsy Staged with CT chest and abdomen, EUS, and PET scan for all patients with CT or EUS evidence of advanced disease (T2 or greater, N1 or NX).

Endoscopy
The diagnosis of esophageal cancer is made best from an endoscopic biopsy. During endoscopy, it is critical to document the following: -Location of the lesion (with respect to distance from the incisors -Nature of the lesion (friable, firm, polypoid -Proximal and distal extent of the lesion -Relationship of the lesion to the cricopharyngeus muscle, the GEJ, and the gastric cardia - Distensibility of the stomach

Esophagram
-Recommended for any patient presenting with dysphagia -It is able to differentiate intraluminal from intramural lesions and to discriminate between intrinsic (from a mass protruding into the lumen) and extrinsic (from compression of a structures outside the esophagus) compression. -The classic finding of an apple-core lesion in patients with esophageal cancer is recognized easily.

Barium contrast study showing an ulcerative but localized tumor of the mid-esophagus Corresponding surgical specimen

Computed Tomography
A CT scan of the chest and abdomen is important to assess the length of the tumor, thickness of the esophagus and stomach, regional lymph node status (including cervical, mediastinal, and celiac lymph nodes), and distant disease to the liver and lungs. It is also helpful in determining T4 lesions where the lesion is invading surrounding structures. It may identify a fistula or other anatomic variations such as a deviated trachea.

CT scan showing a locally advanced tumor with

.tracheal compression and aortic involvement

Endoscopic Ultrasound
EUS is the most critical component of esophageal cancer staging.
Can identify the depth of the tumor, the length of the tumor, the degree of luminal compromise, the status of regional lymph nodes, and involvement of adjacent structures.

EUS examination with a mini-probe showing a tumor

involving the full thickness of the esophageal wall

Positron Emission Tomography

A positron emission tomography (PET) scan evaluates the primary mass, regional and lymph nodes, and distant disease Itsspecificity slightly exceed those of CT; however, they remain low for definitive staging. The sensitivity and specificity of PET for evaluating metastatic disease are as high as 88% and 93%, respectively

PET scan showing a lower-third esophageal cancer with

metastasis to cervical lymph nodes

Bronchoscopy
Flexible bronchoscopy is performed to assess tumor involvement of the tracheobronchial tree, especially for tumors in the middle and upper esophagus.

Thoracoscopy and Laparoscopy

Thoracoscopic staging usually involves a rightsided approach, with opening of the mediastinal pleura from below the subclavian vessels to the inferior pulmonary vein for lymph node sampling.
Laparoscopic staging can include celiac lymph node biopsy and the use of laparoscopic ultrasound for detecting liver metastases

Clinical Approach to Carcinoma of the Esophagus and Cardia

The selection of a curative vs. a palliative operation is based on the 1-Location , 2-Age 3-Health, 4-E extent 5-Preoperative staging.

location
Cervical esophageal cancer is frequently unresectable because of early invasion of the larynx, great vessels, or trachea

location
Tumors that arise within the middle third of the esophagus are squamous carcinomas most commonly and are frequently associated with LN metastasis, which are usually in the thorax but may be in the neck or abdomen,

Location
Tumors of the lower esophagus and cardia are usually adenocarcinomas. Unless preoperative and intraoperative staging clearly demonstrate an incurable lesion, resection in continuity with a LN dissection should be performed

AGE
Resection for cure of carcinoma of the esophagus in a patient older than 80 years is rarely indicated, because of the additional operative risk and the shorter life expectancy

Cardiopulmonary Reserve
The respiratory function is best assessed with the forced expiratory volume in 1 second, which ideally should be 2 L or more

Nutritional Status
Profound weight loss, more than 20 lb, associated with hypoalbuminemia (albumin <3.5 g/dL) is associated with a much higher rate of complications and mortality than patients who enter curative surgery in better nutritional condition

Unfavorable parameters
Factors that make surgical cure unlikely include a tumor >8 cm in length, abnormal axis of the esophagus on a barium radiogram, more than four enlarged LNs on CT, a weight loss more than 20%, and loss of appetite.

Favorable parameters
Tumors <4 cm in length, The finding of a small tumor should encourage an aggressive approach

TNM Staging
T: Primary Tumor TxTumor cannot be assessed T0No evidence of tumorTisHigh-grade dysplasia T1Tumor invades the lamina propria, muscularis mucosae, or submucosa; does not breach the submucosa

TNM Staging
T2Tumor invades into but not beyond the muscularis propria T3Tumor invades the paraesophageal tissue but does not invade adjacent structures T4Tumor invades adjacent structures

TNM Staging
N: Regional Lymph Nodes NxRegional lymph nodes cannot be assessed N0No regional lymph node metastases N1Regional lymph nodes metastases

M: Distant Metastases

TNM Staging
Mx Distant metastases cannot be assessed M1a Upper thoracic esophageal lesion metastatic Midthoracic to cervical lymph nodes esophageal lesion metastatic to Lower thoracic mediastinal lymph nodes esophageal lesion metastatic to celiac lymph nodes

TNM Staging
M1b Upper thoracic esophageal lesion metastatic to mediastinal or celiac lymph Midthoracic esophageal lesion nodes metastatic to cervical or celiac lymph Lower thoracic esophageal lesion nodes metastatic to cervical or upper mediastinal lymph nodes

STAGE GROUPINGS
Stage 0 Stage I Stage II

T Tis T1
T2 T3

N N0 N0
N0 N0

M M0 M0
M0 M0

Stage III Stage IV

T3 Any T

N1 Any N

M0 M1

the following variables are considered

1. 2. 3. 4. Histology, location, and local extent (depth of invasion) of the primary tumor Status of the local and regional lymph nodes Presence of distant lymph nodes or systemic disease Overall condition of the patient (including nutritional status and ability to swallow) Intended goal of treatmentcurative or palliative

5.

Management

Treatment for High-Grade Dysplasia and Early Adenocarcinoma

Barrett's high-grade dysplasia, synonymous with intraepithelial cancer, is the last preinvasive stage in the metaplasia-dysplasia-cancer sequence. Options for treatment include: intensive surveillance, mucosal ablation, and

.esophagectomy

Surgical Resection for Esophageal Cancer

Cervical Esophageal Cancer For tumors confined to the proximal portion of the cervical esophagus with sufficient distal margin, free jejunal interposition graft or deltopectoral or pectoralis major myocutaneous flaps are options for reconstruction after resection.

Surgical Resection for Esophageal Cancer

Intrathoracic Esophageal Cancer For tumors in the upper thoracic esophagus, obtaining a sufficient proximal resection margin dictates an anastomosis placed in the neck. For this reason resection is best carried out by a three-phase esophagectomy or the McKeown approach

Surgical Resection for Esophageal Cancer

Abdominal Esophagus and Gastric Cardia Tumors Abdominalright thoracic approach as in a Lewis-Tanner esophagectomy is one option, with the proximal stomach also resected in order to gain an adequate distal resection margin.

Surgical Resection for Esophageal Cancer

A left thoracoabdominal incision through the seventh or eighth rib space also gives excellent exposure of the low mediastinum and upper abdomen Transhiatal esophagectomy

Combined Multimodal Treatment Strategies

Neoadjuvant Radiotherapy failed to show increased resection rate or improved survival compared with surgery Adjuvant Radiotherapy demonstrated improved local disease control.

Combined Multimodal Treatment Strategies

Neoadjuvant Chemotherapy A significant survival advantage for chemotherapy Adjuvant Chemotherapy The 5-year disease-free survival rate was significantly different, at 45% with surgery alone and 55% with surgery plus chemotherapy, respectively.

Palliation
Several palliative therapies are available for patients who have unresectable disease, have metastatic disease, or are medically unfit for surgery

Palliation
Percutaneous gastrostomy (PEG) tube 0pen gastrostomy or jejunostomy tube is required Intubatin and stentng Laser Fulguration Photodynamic Therapy

BENIGN NEOPLASMS
Benign neoplasms of the esophagus are rare. Leiomyoma is the most common benign esophageal neoplasm

Leiomyoma
Account for roughly two thirds of all benign
80% of leiomyomas are located originate in the muscularis propria.

slow-growing tumors

Evaluation
a barium swallow, endoscopy, endoscopic ultrasound (EUS), and a CT scan of the chest

Indications for resection

symptoms of dysphagia increase in tumor size, mucosal ulceration, and definitive establishment of a diagnosis ruling out a malignant process such as a .leiomyosarcoma

TTT
Leiomyomas less than 8 cm without annular characteristics are best treated by surgical extramucosal enucleation

Staging of Esophageal Cancer

Choosing the best therapy for an individual patient requires accurate staging. Staging starts with the history and physical. LN disease remote from the tumor, particularly in the cervical region, may be palpable on neck examination and generally indicates indicating that these ,M1a disease cancer dissemination. This is often referred to as patients should not be treated with therapy directed toward locally advanced cancer. Other metastatic LNs are rarely palpable but are equally ominous, especially the .umbilical LN in GEJ cancer Chest x-ray is the first step of staging, but is rarely informative. Computed tomographic (CT) scanning of the chest, abdomen, and pelvis provides information on local invasion of the primary cancer, LN involvement, or disseminated disease. The most common sites of esophageal cancer metastases are lung, liver, and peritoneal surfaces, including the omentum and small bowel mesentery. If masses are identified that are not characteristic for cancer or are in a location that precludes resection with the cancer specimen, positron emission tomography (PET) scanning may be able to tell whether the masses are metabolically active (likely to be cancer) or not. A PET active focus corresponding to a mass on CT scan outside of the field of esophageal resection should be biopsied before resection is performed.

Experience with esophageal resections in patients with early disease has identified characteristics of esophageal cancer that are associated with improved survival. A number of studies suggest that only metastasis to LNs and tumor penetration of the esophageal wall have a significant and independent influence on prognosis. Factors known to be important in the survival of patients with advanced disease, such as cell type, degree of cellular differentiation, or location of tumor in the esophagus, have no effect on survival of patients who have undergone resection for early disease. Studies also showed that patients having five or fewer LN metastases have a better outcome. Using these data, Skinner developed the wall penetration, LN, and distant