Keratosis palmoplantaris with esophageal cancer
Overview
A very rare condition involving thickening and hardening of the horny layer of skin on the palms and soles as well as an frequent association with esophageal cancer.
Symptoms
* Hyperkeratosis of palms * Hyperkeratosis of soles * Esophageal cancer
Causes
The cause of esophageal cancer is unknown, but predisposing factors include chronic irritation caused by heavy smoking and excessive use of alcohol, stasis-induced inflammation, and nutritional deficiency. Esophageal tumors are usually fungating and infiltrating. Most arise in squamous cell epithelium, a few are adenocarcinomas, and fewer still are melanomas and sarcomas. Esophageal cancer has a 5-year survival rate below 10%, and regional metastasis occurs early by way of submucosal lymphatics. Metastasis produces such serious complications as tracheoesophageal fistulas, mediastinitis, and aortic perforation. Common sites of distant metastasis include the liver and lungs.
Treatment
Multimodal therapy is usually indicated. Whenever possible, treatment includes resection to maintain a passageway for food. This may require such radical surgery as esophagogastrectomy with jejunal or colonic bypass grafts. Palliative surgery may include a feeding gastrostomy. Chemotherapy with 5-fluorouracil or cisplatin may be used. Insertion of prosthetic tubes to bridge the tumor alleviates dysphagia. Other treatments to improve the patient's ability to swallow include endoscopic dilation of the esophagus (sometimes with placement of a stent) and photodynamic therapy. Treatment complications may be severe. Surgery may precipitate an anastomotic leak, a fistula, pneumonia, and empyema. Rarely, radiation may cause esophageal perforation, pneumonitis and pulmonary fibrosis, or myelitis of the spinal cord. Prosthetic tubes may dislodge and perforate the mediastinum or erode the tumor.