- Squamous cell carcinomas, which account for 60% of esophageal cancer, arise from the surface epithelium, most commonly in the middle and lower esophagus. Adenocarcinomas, which constitute the remaining 35%, arise from the gastric fundus and develop in the lower third of the esophageal tract.

Causes and Incidence The etiology is not well defined, but risk factors associated with chronic esophageal irritation include smoking and alcohol abuse. The incidence is low in the United States, but the disease is endemic in central China and Southeast Africa, with reports of 50 cases per 100,000. This cancer is most common in older adults, with blacks affected three times as often as whites and men three times as often as women.

Disease Process A squamous cell carcinoma begins as a small mucosal patch that grows, ulcerates, and extends into the esophageal lumen and then the recurrent laryngeal nerve and tracheobronchial tree. Extension to the aorta and other adjacent structures also occurs. Metastasis to local and abdominal lymph nodes and to most body organs follows.

Symptoms Dysphagia is the most common presenting symptom. Regurgitation and weight loss may also occur.

Potential Complications The prognosis is poor, with less than 5% long-term survival. Complications of advanced disease include esophageal obstruction, hemorrhage, and perforation.

Diagnostic Tests The tumor is diagnosed with visualization on esophageal x-ray followed by esophagoscopy with a brush biopsy.

Treatments

Surgery
Resection of tumor for palliation; esophagectomy with Dacron graft replacement; esophageal dilation to aid eating.

Drugs
Preoperative systemic, cisplatinbased chemotherapy.

General
Radiation for palliation and to control pain; head of bed propped up on 4-inch blocks to prevent reflux; treatment of esophagitis.

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- A chronic, progressive cancer of the lymphoid tissue. (Also see Cancer.)

Causes and Incidence The cause of Hodgkin’s disease is unknown. Current theory holds that it is a low-grade graft-versus-host reaction with some type of infectious agent as a cause. Each year more than 7,000 new cases are diagnosed in the United States, and 1,600 deaths occur. Hodgkin’s disease is the most common cancer in young adults. It occurs most often in two age groups, 15 to 35 and 60 to 80, and it is more common in men.

Disease Process The disease begins with an abnormal proliferation of histiocytes (Reed-Sternberg cells) in one lymph node, which replaces the normal cellular structure and causes tissue necrosis and fibrosis. The disease spreads through the lymphatic channels to lymph nodes throughout the body and eventually metastasizes to the liver, spleen, bronchi, and vertebrae.

Symptoms Most individuals first notice a swelling in the cervical lymph nodes. It may be accompanied by itching, fever, night sweats, and weight loss. Later signs may include cough, dyspnea, chest and bone pain, ascites, and jaundice.

Potential Complications The prognosis for long-term survival is excellent with treatment. About 95% of individuals with stage I or stage II disease are cured with treatment. Untreated or advanced disease causes multiple organ failure and death.

Diagnostic Tests The definitive diagnosis is made by lymph node biopsy, which shows the presence of Reed-Sternberg cells.

Treatments

Surgery
Excision of tumors in advanced disease; therapeutic splenectomy.

Drugs
Systemic combination chemotherapy used with radiation.

General
Radiation is the primary therapy, used alone or in combination with chemotherapy, particularly for stage III and stage IV disease.

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- Renal cell and clear cell adenocarcinomas account for 80% of kidney cancer. Other tumor types include transitional cell, squamous cell, and nephroblastoma.

Causes and Incidence The cause of kidney cancer is unclear, but identifiable risk factors include use of tobacco products, acquired cystic disease, chronic irritation from renal calculi, exposure to petrochemical products, and radiation exposure. More than 18,000 new cases are diagnosed each year in the United States. About 20% of childhood malignancies and 2% of adult malignancies occur in the kidneys. The average age of diagnosis is 55 to 60, and men are affected twice as often as women.

Disease Process Tumor cells, which most often originate in the renal parenchyma, grow into a well-defined tumor, often surrounded by perinephric fat, which slows infiltration of adjacent tissues. Metastasis occurs by venous or lymphatic routes, and the most common metastatic sites are the lungs, bones, liver, and brain.

Symptoms Signs and symptoms develop late in the disease; hematuria is the most common presenting sign, followed by flank pain, a palpable abdominal mass, and/or fever of unknown origin.

Potential Complications The prognosis for metastatic lesions is poor because kidney tumors are resistant to radiation and chemotherapy. Complications include hypertension from pedicle compression.

Diagnostic Tests Abdominal ultrasound and computed tomography scans help detect masses that warrant further diagnostic study. Biopsy by needle aspiration or tissue sample is definitive.

Treatments

Surgery
Nephrectomy with removal of regional lymph nodes.

Drugs
None.

General
None.

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- Hepatocellular carcinomas are the most prevalent of the primary liver tumors, although cholangiocarcinomas, angiosarcomas, and hepatoblastomas also are seen.

Causes and Incidence Chronic hepatitis B virus is a known etiologic agent. Hepatitis C virus, cirrhosis, and hemochromatosis are also associated with the development of liver cancer. Risk factors include ethanol abuse, anabolic steroid abuse, pesticide and herbicide exposure, and ingestion of food contaminated with fungal aflatoxins. The incidence of primary liver cancer in the United States is low, with approximately 15,000 cases diagnosed each year. Most of these individuals have underlying cirrhosis. In certain areas of Africa and Southeast Asia, however, liver cancer is the leading malignancy and one of the leading causes of death. Most are associated with hepatitis B infections.

Disease Process Most cancer cells originate in the parenchyma and rapidly form a tumor that extends and invades adjacent structures such as the stomach and diaphragm. Metastasis occurs to the regional nodes, lung, bone, adrenal gland, and brain.

Symptoms Abdominal pain, right upper quadrant mass, epigastric fullness, and weight loss are the most common presenting signs and symptoms. Systemic metabolic signs may include hypoglycemia, hypercalcemia, hyperlipidemia, and erythrocytosis.

Potential Complications The prognosis in liver cancer is grim; the survival rate is only about 5%. Complications include liver failure, gastrointestinal hemorrhage, and cachexia.

Diagnostic Tests The alpha-fetoprotein blood value is elevated in 70% of cases; ultrasound and computed tomography scans can help visualize masses. A biopsy is needed for definitive diagnosis.

Treatments

Surgery
Resection of tumor is only a potentially curative modality.

Drugs
Chemotherapy is experimental and not particularly effective to date.

General
Radiation cannot be given in sufficient doses to be effective; radiolabeled antibodies have been used, with limited success.

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- The major histologic types of lung cancer are non–small cell cancers (squamous cell cancer, adenocarcinoma, and large cell undifferentiated cancer), which account for 90% of lung cancers, and small cell lung cancers, which make up the remaining 10%.

Causes and Incidence Cigarette smoking is implicated in approximately 80% to 90% of all cases of lung cancer. Occupational exposure to asbestos, radon, nickel, chromium, hydrocarbons, and arsenic is linked to 10% to 15% of lung cancers. The role of air pollution and home exposure to radon gas is unclear. Lung cancer is the leading cause of cancer death for both men and women in the United States. More than 170,000 cases are diagnosed each year, and the incidence for women is rising rapidly.

Disease Process Squamous cell carcinomas usually begin in the larger bronchi, often causing bronchial obstruction and spreading by direct extension and lymph node metastasis. Adenocarcinomas are peripheral tumors that begin in fibrotic lung tissue and spread through the bloodstream, commonly metastasizing to the brain, liver, and bone. Large cell undifferentiated carcinoma, which may arise in any area of the lung, disseminates early, spreading through the bloodstream. Small cell carcinoma is centrally located and is the fastest growing type of lung cancer, with rapid metastasis to the brain, liver, and bone.

Symptoms A chronic cough, a change in the volume and color of sputum, chronic upper respiratory tract infections, and aching in the chest are common presenting symptoms. Wheezing, fatigue, and chest tightness may also be present.

Potential Complications The prognosis is poor. The long-term survival rate in individuals with localized disease is only 35%, and most people have extension and metastasis upon diagnosis. Overall the survival rate for all individuals regardless of stage is 13%. Complications include superior vena cava syndrome, paraneoplastic syndromes, and cor pulmonale.

Diagnostic Tests A history of smoking and a chest x-ray are the principal sources of diagnostic suspicion. A sputum cytology test is positive in about 75% of cases. Retrieval of cells through bronchoscopy or needle or tissue biopsy provides the definitive diagnosis.

Treatments

Surgery
Resection of tumor and surrounding tissue; lobectomy or pneumonectomy.

Drugs
Systemic multidrug combination chemotherapy; biologic response modifiers.

General
Radiotherapy before and after surgery and for palliation; smoking cessation; prevention through education about dangers of tobacco and environmental irritants.

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