Category Archives: esophagus

carcinoma, esophagus, malignant, primary cancer

Esophagus Carcinoma

Carcinoma

Definition

Esophageal cancer is a malignant growth disorder of esophageal mucosa. Historically squamous cell cancer was the predominant type; however, more recently, a surge in adenocarcinoma as the more common entity has evolved. Sqaumous cell carcinoma characteristically occurs in the thoracic esophagus while adenocarcinoma occurs at the GE junction.

Many different causative factors have been associated with this condition including GERD, Barrett’s esophagus, dietary factors, smoking and geographic location.

The disease results in an aggressive space occupying mass that progressively impinges on the lumen of the esophagus to cause dysphagia

It is most commonly diagnosed by endoscopy and biopsy, staged with CT and PET scan, and best treated with a combination of surgical and medical therapies.

The overall prognosis is quite poor, with 5 year survival estimated to be between 10-13% for advanced disease. With the increased diagnosis of Barrett’s esophagus, and the routine surveillance that follows, earlier detection of cancer allows for treatment and better survival rates. Survival rates are much improved with more superficial cancers that are found with increasing frequency during endoscopic surveillance.

Ulcerating Squamous Cell Carcinoma of the Esophagus

02425 02420 code esophagus + mass narrowing stricture ulceration heaped edges squamous cell carcinoma + grosspathology

Ulcerating Esophageal Carcinoma with Heaped Edges

01270c code esophagus + fx mass + dx carcinoma + barium swallow upper GI UGI imaging radiology contrast X-Ray mass neoplasm malignant primary carcinoma cancer tumor Courtesy Ashley Davidoff MD

Causes and Predisposing Factors

ie etiology and pathogenesis

Squamous cell carcinoma and adenocarcinoma each have their own predisposing conditions, and as such, will be described separately.

Causes Related to Squamous Cell Carcinoma:

Geographic location has been shown to be a risk factor for the development of squamous cell carcinoma of the esophagus. Asian countries have a higher incidence than that of the western part of the world.

Dietary risk factors also exist. Much like that of other gastrointestinal cancers, foods with n-nitroso compounds are thought to increase the risk. Within the Asian countries, the common practice of chewing of the Betel nut, has been associated with squamous cell carcinoma.

Other dietary risk factors include the consumption of alcohol. A correlation between the amount consumed and the risk of cancer exists.

Tobacco use has also been closely associated with squamous cell esophageal cancer.

Patients with underlying conditions that affect the esophagus are also at increased risk. A previous history of caustic ingestion, classically with lye, increases the risk.

Causes Related to Adenocarcinoma:

The most important risk factor for the development of adenocarcinoma of the esophagus is a history of Barrett’s esophagus. The progression of Barrett’s to carcinoma is well documented and reviewed at length in the section on Barrett’s.

Other risk factors for the development of adenocarcinoma of the esophagus include tobacco use, obesity, GERD, and other conditions that will increase acid exposure in the esophagus

Statistics

In the USA it is relatively uncommon with about 10,000 new cases per year or between 2 – 8 people per 100,000

There are certain areas in China with 100 new cases per 100,000

Race

Higher in the black population – could this show a genetic predisposition?

Sex

M:F range from
2:1 to 20:1.

Age

adult disease
usually over age 50

Other Statistics

1% of all cancers
10% of gastrointestinal malignancies

Result

Since the lymphatic network in the submucosa is so allows both circumferential and longitudinal spread and the disease is usually more extensive than the macrsosopic appearance and early lymphatic spread to areas remote from main mass is characteristic. As mentioned above, the 5 year survival rates for esophageal cancer are quite low. Early detection is important as if found prior to metastasis, the treatment options are more plentiful.

Gross Pathology

polypoid or fungating (common)
ulcerating form
infiltrating form
varicoid form

Ill Defined GE Junction Tumor – Adenocarcinoma

00567c03 distal esophagus GE junction gastroesophageal junction stomach GE junction tumor fx nodular appearance to the GE junction dx adenocarcinoma of the GE junction grosspathology malignant cancer Courtesy Ashley Davidoff MD

Histopath

squamous cell carcinoma (95%) Squamous cell carcinomas are moderately to well differentiatedsquamous cell carcinoma (common)
adenocarcinoma (4%)
Barrett 70%
GE junction 30%

other
adenoid cystic carcinoma
carcinosarcoma

Squamous cell carcinomas are moderately to well differentiatedsquamous cell carcinoma (common)

They pesent as;

well differentiated (common)
mderately differentiated (common)
poorly differentiated

Complications

The mass may cause local obstruction. Local extension into mediastinal structures is early and common. Tracheoesophageal fistula can occur in 5 -10%. Other complications include ulceration, bleeding airway compression, and invasion into the aorta. Systemic complications are characterized by metatastasis to nodes, lung, liver, adrenal gland.

Classification based on Staging

TNM system:

T, tumor <5 cm in length without circumferential involvement

T2 tumor >5 cm in length / circumferential or obstructive lesion

T3 extraesophageal spread

classification based on radiologic appearance

Diagnosis

Clinical

Clinically, patients present with a myriad of complaints, the most common of which is dysphagia. As the lumen narrows for the growth of the lesion, food is unable to pass smoothly thru the esophagus. Patients can present with upper gastrointestinal bleeding, weight loss, halitosis, in addition to many non-specific complaints.

The onset of the dysphagia is insidious. The esophagus is a pliable and mobile organ and is therefore forgiving and can accomodate the advancing mass. Thus clinical presentation and the onset of dysphagia is late.The delay in time to clinical presentation implies advanced disease at presentation and this affects outcome.

Imaging

With clinical suspicion, most patients undergo an upper endoscopy to evaluate the symptoms. This modality allows for direct visualization of the lesion and for biopsy for tissue diagnosis.

Carcinoma of the Esophagus

73940.400 73941.400 esophagus distal mass ulcerating bleeding carcinoma of the esophagus endoscopy endoscopic view Courtesy Joshua Namias MD

Carcinoma of the Esophagus

73941.400 73941.400 esophagus distal mass ulcerating bleeding carcinoma of the esophagus endoscopy endoscopic view Courtesy Joshua Namias MD

Irregular Distal Esophagus with Stricture – Adenocarcinoma of the GE junction

75711c01 dysphagia esophagus distal irregular stricture dx adenocarcinoma of the esophagus barium swallow Courtesy Ashley Davidoff MD

Long Relatively Smooth Malignant Stricture

Courtesy Ashley Davidoff MD 01240 esophagus + fx irregular stricture + dx carcinoma + barium swallow upper GI UGI imaging radiology contrast X-Ray mass neoplasm malignant primary carcinoma cancer tumor

Long Mid Esophagus Malignant Stricture

Courtesy Ashley Davidoff MD 01246 code esophagus + fx irregular + fx long fx narrowed + dx squamous cell carcinoma + barium swallow upper GI UGI imaging radiology contrast X-Ray mass neoplasm malignant primary carcinoma cancer tumor

Depending on the initial complaint, a barium study may also be performed.

Findigs may include a polypoid or fungating form, ulcerating form, or infiltrating form. If ulcerated a filling defect would be apparent, anf if it involves the esophagus ircumferentially an apple core lesion or a focal narrowing will be present. Once the diameter of the lumen becomes less than 13mm partial obstruction occrs, the lumen becomes functionally compromised the proximal esophagus dilates and dysphagia ensues.Any mucosal irregularity that raises uspicion should be evaluated an biopsied as dwcided by the endoscopist at the tine.

CT shows wall thickening anf the relationship to
mediastinal structures. Ominous signs iclude 1oss of fat planes invasion into trachea, aorta, pericardium, and distant metastases

Once a lesion is found, further diagnostic studies are often performed. Endoscopic ultrasound allows for great definition of the extent of the disease. This modality allows for visualization of the layers of the esophagus, such that the level of invasion can be identified. Biopsies of involved tissue and nodes can be performed.

CT scan allows for evaluation for metastatic disease as well.

Staging by Non Invasive Method

29885c03 esophagus lymph nodes primary esophageal carcinoma probably squamous carcinoma metastases to lymph nodes mediastinal lymphadenopathy mediastinum irregular lumen long segment single contrast bariu swallow CTscan PET scan NM Courtesy Ashley Davidoff MD

Treatment

Treatment of esophageal cancer greatly depends on the staging of the disease.

If the tumor is localized to the esophagus, without evidence of direct or metastatic spread, surgery provides a cure. Typically an esophagectomy with gastric pull up is the treatment of choice. Mucosal resections, photodynamic therapies, and ablative techniques can be entertained.

If the disease is not resectable at the time of diagnosis, chemotherapy, often in combination with radiation therapy is undertaken.

Stent in the Distal Esophagus

18203c esophagus carcinoma stricture tube stent dilatation treatment Courtesy Ashley Davidoff MD

Gastric Pull Through

76384 esophagus carcinoma cancer gastric pull through surgery treatment differential diagnosis dd dilated esophagus barium swallow Courtesy Ashley Davidoff MD 76386c01

Prognosis

3 – 75% 5-year survival rate

For superficial superficial esophageal carcinoma 5-year survival rate is 75% and for advanced lesions 5-25% 5-year survival rate. The presence of involved Iymph node metastases at the time of resection reduces the 5 year survival.

esophagus, premalignant

Esophagus Barrett’s

Definition

Barrett’s esophagus is an abnormal growth disorder of the gastroesophageal junction characterized by metaplastic change of the squamous epithelium into a glandular columnar epithelium caused by longstanding gastroesophageal reflux. With this change there may be resulting alteration in the DNA rendering the entity a premalignant condition.

The clinical presentation is non specific and include the dyspeptic symptoms of GERD . The diagnosis is made by endoscopy, where the extension of the gastric mucosa into the squamous territory is seen as cracks of salmon colored areas in the white esophageal mucosa. Biopsies need to be taken to evaluate for dysplasia. In the absence of dysplasia treatment includes ongoing surveillance, with aggressive medical treatment of the GERD. In the presence of dysplasia removal of the dysplastic abnormalities is indicated. A variety of endoscopic ablative therapies and surgical options are available.

Normal Regular “Z” line (left) and and Barrett’s Esophagus (right)

73474 squamocolumnar junction gastroesophageal junction squamous epithelium gastric epithelium columnar epithelium normal Z line anatomy histology endoscopy endoscope Courtesy Joshua Namias MD

73475 squamocolumnar junction gastroesophageal junction squamous epithelium gastric epithelium columnar epithelium Extension of columnar epithelium toward the squamous epithelium space occupation Barrett’s esophagus endoscopy endoscope Courtesy Joshua Namias MD

Barretts Esophagus

12254 Gross specimen showing Barrett’s mucosa. The lighter white portion is normal esophageal squamous epithelium, and the tan-brown area is abnormal. This is where the normal squamous epithelium has been replaced by metaplastic columnar epithelium, comprising Barrett’s esophagus. Courtesy Barbara Banner MD

Barretts Esophagus

12255 Microscopic section at low power showing Barrett’s mucosa. Note that a glandular type of mucosa is present overlying the cluster of esophageal glands. This tells us that where there ought to be squamous epithelium, there is now columnar metaplasia, and this is the definition of Barrett’s esophagus. histopathology Courtesy Barbara Banner MD

Causes and Predisposing Factors

Barrett’s is a sequela of longstanding reflux. The mean age of diagnosis is 55, although can be seen earlier with rare cases reported in the pediatric population. The male to female ratio is 2 to 1 with a higher incidence in the white and Hispanic population. It is less frequently seen in the black and asian population.

With the continuous injury from acid, the esophagus attempts to protect itself by changing to the more acid resistant intestinal type mucosa. However, with this transformation, there is DNA alteration and malignant changes can occur.

Result

The specialized intestinal metaplasia is glandular mucosa with mucin-type cells and abundant goblet cells.

The risk of Barrett’s Esophagus is the conversion to carcinoma. This conversion can be thought of as a stepwise process.

GERD-à Erosive Esophagitisà Specialized Intestinal Metaplasiaà Low Grade Dysplasiaà High Grade Dysplasiaà Carcinoma

Esophageal carcinoma has increased 300% since the 1970s. There are many reasons for this but clearly detection by endoscopic procedure and routine surveillance for Barrett’s plays a large role.

Diagnosis

Barrett’s Esophagus is a endoscopic and histopathologic diagnosis. Upon endoscopic visualization of the GE junction, one can appreciate a regular appearing “Z” line. Again, this is the area of change from squamous esophageal mucosa to gastric type mucosa.

With Barrett’s, however, there is an irregularity of this line with finger like projections starting at the transition point and traversing more proximally. This salmon colored tissue is the specialized intestinal metaplasia.

Barrett’s Esophagus

01235c03 01235 by endoscopy Barret’s Courtesy Ashley Davidoff MD code esophagus epiphrenic ampulla hiatus hernia esophagitis barium swallow upper GI UGI imaging radiology contrast X-Ray

This finding is not uncommonly seen and it has been estimated that 1/100 “routine” EGDs will demonstrate Barrett’s, while those done for “reflux” will show a incidence of approximately 1/10.

Barrett’s itself does not have associated symptoms. Instead, patients are often symptomatic from the underlying reflux. There are cases where the first presenting symptom is dysphagia or odynophagia and endoscopy reveals Barrett’s and esophageal carcinoma. Autopsy studies have shown Barrett’s to be present in 1/57-1/105 cases.

Biopsies should be taken at the initial finding of Barrett’s. The biopsies are taken from 4 quadrants and in 1cm intervals. It is extremely important to have patients with no evidence of dysplasia to undergo surveillance endoscopies. After Barrett’s is found endoscopy should be performed annually for 2 years and the every three if no dysplasia is found.

Those patients who have progressed to dysplasia must consider further therapies. Regardless of the modality chosen (described below) annual endoscopies are required.

Barret’s Esophagus by Endoscopy No Specific Findings

Suspect in the Setting of Peptic Esohagitis and its Sequelae

This series of images of the esophagus from a double contrast barium swallow shows a focal irregular stricture at the GE junction (a) associated with a hiatus hernia. (d,e,f). The irregularity suggest ongoing esophagitis but Barrett’s esophagus has to be considered. The patient complained of cervical dysphagia. A 13mm barium pill was held up at the junction implying a significant narrowing. The stricture seen in d,e,f has a concentric ring like appearance reminiscent of a Schatki’s ring.

Courtesy Ashley Davidoff MD 38421c02 code esophagus stricture narrowing irregular HH Schatzki’s ring barium pill

Treatment

Strict acid suppression is key, but it should be noted that regression of Barrett’s is not thought to be possible. Instead, the acid suppression is thought to reduce the ongoing acid exposure and damage to the wall.

There are four main therapeutic options in patients with Barrett’s.

Endscopic Ablative Therapies include Multipolar Electrocoagulation (MPEC) which is heat induced damage to the Barrett’s mucosa. It is reserved for non-dysplastic cases. It carries a risk of perforation, stricture formation, and residual Barrett’s can be found in upto 28% of cases.

Argon Plasma Coagulation is another endoscopic modality that uses ionized electronically charged gas, again to burn the mucosa. Strictures can form in upto 10%.

Photodynamic therapy involves giving systemic administration of a photo-sensitizing agent and the endoscopic to laser light that causes and oxidative damage to the mucosa.

Endoscopic Mucosal Resection consists of an upper endoscopy with the removal of the esophageal mucosa.

Surgical esophagectomy is a invasive approach but one that completely removes the Barrett’s and the possible conversion to cancer. However, clearly, complications and post-surgical morbidity is higher. Patients can develop dysphagia, weight loss, dumping syndrome, in addition to the surgical complications.