Esophageal Tumors

Benign Tumors

Fewer than 10% of esophageal tumors are benign; leiomyoma is the most common one. Rare tumors include inflammatory polyps, squamous cell papilloma, lymphangioma, granular cell tumor, fibromyxoma, fibrolipoma, and lipoma. Usually asymptomatic, these tumors are often found incidentally on examination for unrelated complaints or at autopsy. Symptomatic tumors may produce dysphagia or upper GI bleeding. Diagnosis often is established by esophagoscopy and biopsy. Endoscopic ultrasound may help. Surgical resection is indicated only when the tumor produces bleeding or obstruction.

Malignant Tumors

Esophageal cancer accounts for only 4% of all GI cancers in the USA but is more common in China, central Asia, South Africa, and certain Mediterranean countries. It occurs in middle to late adulthood, predominantly in men, and is more common in blacks and in smokers. Mortality rates increase steadily with age, with 66 as the median age at death.

In the last decade, the incidence of esophageal adenocarcinoma has increased dramatically, and esophageal adenocarcinoma is more common than esophageal squamous cell carcinoma in the USA. Esophageal adenocarcinoma has the fastest rate of increase in incidence of any cancer in the USA, excluding skin cancer.

A number of carcinogenic factors and disease states, including chronic thermal injury, achalasia, alcohol, tobacco, and the Plummer-Vinson syndrome, are associated with esophageal squamous cell carcinoma. An estimated 10% of patients with Barrett's esophagus (an intestinal-type metaplasia that replaces the native squamous cell epithelium of the esophageal mucosa) eventually develop esophageal adenocarcinoma. A strong and probable causal relationship exists between gastroesophageal reflux and esophageal adenocarcinoma.

The middle and lower third of the esophagus are usually involved. Tumors may be infiltrative, ulcerative, or polypoid and may cause a stricture or mass.

Symptoms, Signs, and Diagnosis

The most common symptoms are progressive dysphagia and weight loss; others include odynophagia, hoarseness, recurrent respiratory infections, and hematemesis. Signs appear late in the course of disease and include regional lymphadenopathy, vocal cord paralysis, and pulmonary findings (eg, wheezes, rales). In the elderly, these symptoms and signs may be confused with primary neurologic or pulmonary conditions.

About 50% of patients have advanced disease at diagnosis, which is typically made by barium swallow followed by endoscopy. The upper GI series often shows a stricture or an eccentric or asymmetric mucosal irregularity. Endoscopy provides direct visualization of the lesion and tissue for microscopic examination. The combination of biopsy and brush cytology yields the diagnosis in > 95% of cases. Endoscopic ultrasound and CT are useful for tumor staging, especially in patients considered for curative surgery.

Prognosis and Treatment

The management of the esophageal cancer is determined by the disease stage. Although the overall cure rate for esophageal cancer is only about 5%, selected series show nearly a 25% survival rate in patients with cancer of the distal portion. Because the cure rate is so low, experimental protocols have been designed that combine radiation therapy and chemotherapy using cisplatin and other drugs; in some cases, tumor regression has been remarkable. Nonetheless, aggressive treatment is often not appropriate, especially in elderly persons with significant comorbidities.

Surgical therapy remains the best treatment of limited esophageal tumors. In patients with squamous cell carcinoma, the combination of chemotherapy and radiation therapy before surgery increases the probability of disease-free survival. The benefit of a similar approach in patients with adenocarcinoma is uncertain, although promising. The postoperative mortality rate for elderly patients is comparable with that in younger patients, but the elderly experience a greater incidence of postoperative cardiopulmonary complications.

Endoscopy can be used for palliation in high-risk elderly patients or in those with advanced disease. Dilatation, injection therapy, photodynamic therapy, and placement of an esophageal prosthesis can also provide palliation. Nutritional support may be accomplished by percutaneous endoscopic gastrostomy or jejunostomy tube feedings.

Esophageal cancer is often terminal and leads to a poor quality of life, primarily because patients cannot swallow their own saliva. Therefore, palliative procedures are often offered, even when cure is unlikely. When possible, resection and esophagogastrectomy are preferred. If the tumor cannot be resected, the favored procedure is colonic bypass, in which the esophagus is left in place. Rigid tubes (Celestin's or Souttar's) may be inserted through the tumor, but because these tubes tend to become displaced and cause perforation, they are not often used. Laser therapy or radiation therapy and chemotherapy can debulk obstructing tumors. Gastrostomy allows feeding but does not alleviate dysphagia. Pain should always be treated.

Stomach Tumors

Benign Tumors

Fewer than 5% of all stomach tumors are benign. Although leiomyoma is the most common benign stomach tumor in the general population, hyperplastic and adenomatous polyps are also common among the elderly. Hyperplastic polyps are small (usually < 1 cm in diameter), flat lesions that account for about 95% of all gastric epithelial polyps, while the remaining 5% are predominantly adenomatous polyps. Hyperplastic polyps carry no malignant potential, whereas adenomatous polyps do, usually when they are > 2 cm.

The Cronkhite-Canada syndrome, although a rare nonhereditary entity, represents the only nonfamilial GI polyposis syndrome occurring in the elderly. In addition to multiple hyperplastic polyps, the syndrome includes ectodermal changes (eg, increased pigmentation, alopecia, atrophic nails). Diarrhea is common.

Most benign stomach tumors are asymptomatic and are found during examinations performed for unrelated symptoms. The most common presenting finding is anemia from chronic occult bleeding. Less commonly, epigastric pain or acute GI bleeding from tumor ulceration occurs.

In most cases, endoscopy can be used for diagnosis and treatment. If the lesion is submucosal or if its size or location precludes endoscopic resection, surgery may be warranted if significant blood loss or other symptoms have developed.

Malignant Tumors

As of 1999 in the USA, about 22,000 new cases and 14,000 deaths from stomach cancer occurred annually, 70% of which affected patients >= 65 years. Stomach cancer in the elderly probably develops principally as well-differentiated carcinomas that then progress to poorly differentiated ones with time, in contrast with those of younger patients, most of which emerge in the very early phase as poorly differentiated tumors.

Adenocarcinoma accounts for 95% of all stomach cancers. Gastric adenocarcinoma is more common in blacks and among poor socioeconomic groups; the male:female ratio is 2:1. The worldwide incidence varies dramatically, with low rates in the USA and high rates in Japan, Chile, and Costa Rica. For an unknown reason, overall incidence has decreased worldwide during the past 50 years. Helicobacter pylori infection is likely the most important risk factor. This infection is also associated with mucosal-associated lymphoid tissue, a type of low-grade gastric lymphoma. Other risk factors include adenoma, chronic atrophic gastritis with intestinal metaplasia (with or without associated pernicious anemia), adenomatous polyps, stomach remnants after subtotal gastrectomy, and chronic gastric ulcer.

Lymphoma accounts for about 4% of stomach cancers. The stomach is the most common site of primary extranodal lymphoma, accounting for up to 75% of all cases of primary GI tract lymphomas. Gastric lymphoma occurs mainly in men in the 6th decade.

Symptoms and Signs

Stomach cancer is usually asymptomatic in its early stage. The most common presenting symptom is vague epigastric discomfort followed by anorexia, early satiety, hematemesis, melena, and severe abdominal pain as the tumor progresses. If the cardia is involved, dysphagia may occur. If the prepyloric antrum is involved, symptoms of partial or complete gastric outlet obstruction may occur. Patients with gastric lymphoma present with similar symptoms.

No specific signs occur in the early stage. In later stages, nausea, vomiting, dysphagia, weight loss, a palpable mass, and lymphadenopathy in the left supraclavicular region (Virchow's node) may be noted. Liver metastases can present as hepatomegaly. Dermatologic signs include acanthosis nigricans and dermatomyositis.

Diagnosis

An upper GI series is usually the initial test, but lesions are often missed. Endoscopy allows visualization of most lesions, obtains tissue for histologic examination, and yields the diagnosis in > 90% of patients. Stomach cancer is staged using CT and endoscopic ultrasound, which may indicate the depth of invasion. Available tumor markers (eg, carcinoembryonic antigen, fetal sulfoglycoprotein, CA 72-4) provide little help because their sensitivity and specificity are poor. The differential diagnosis includes peptic ulcer and pancreaticobiliary tract disease.

The radiographic appearance of lymphoma may be similar to that of adenocarcinoma, although large gastric folds and evidence of infiltration into the duodenum are more typical of lymphoma than of carcinoma. Endoscopy with multiple directed biopsies combined with brush cytology may confirm the diagnosis, but because the lesions are submucosal, laparotomy may be needed.

Prognosis and Treatment

With adenocarcinoma, the overall 5-year survival rate is < 10%. With early stomach cancer, 5-year survival rates of up to 95% have been reported. With primary gastric lymphoma, the 5-year survival rate approaches 50%. However, the prognosis is adversely affected by age; the 5-year survival rate for patients < 45 with gastric lymphoma is 57%; for those > 65, it is 32%.

Surgery is the only potential curative treatment available for stomach cancer. For distal gastric lesions, adequate resection involves subtotal gastrectomy, whereas total gastrectomy is performed for proximal lesions. Japanese and Western surgeons differ in their approach to lymph node dissection. Although a more radical lymph node dissection increases survival in Japan, it has not been shown to influence survival or prevent local recurrence in Western series. In elderly patients operated on for cure, perioperative morbidity (30 to 40%) and mortality (< 10%) rates are comparable with those obtained in younger patients. Five-year probability of survival after curative surgery for stomach cancer is up to 25%.

Radiation therapy alone is ineffective for adenocarcinoma. Radiation therapy after resection commonly produces good results in patients with gastric lymphoma. Chemotherapy, either as adjuvant therapy after surgery or as treatment for advanced disease, has failed to demonstrate a clear survival benefit in patients with adenocarcinoma. Chemotherapy for gastric lymphoma is much more effective, although perforation can occur during tumor lysis.

Palliative surgery for gastric cancer is difficult and produces higher complication rates than curative surgery. Therefore, palliative surgical procedures, if used at all, should only be performed in patients with few comorbid conditions and good overall function, not in those with advanced metastatic disease. In selected patients with lesions causing distal esophageal or gastric outlet obstruction, palliation has been achieved using endoscopic laser photoablation. Palliative chemotherapy may be an option for patients with advanced stages of gastric adenocarcinoma. One should be mindful of a proclivity to bone metastasis.

Because stomach cancer is often terminal, advance planning for end-of-life issues must be discussed early with elderly patients and their families when appropriate. The usefulness of treatment must be weighed against the adverse effects it causes. At the end of life, patients are likely to experience nausea, vomiting, pain, and weight loss and should be treated accordingly to control symptoms.