Korean J Thorac Cardiovasc Surg.  2000 Jan;33(1):112-115.

Esophageal Leiomyomatosis in a patient with Alport Syndrome

• Choi IS
• Park CC
• Lee JH

Abstract

A-13-urar-old with a history of Alport syndrome had been suffering from progressive dysphagia and postprandial vomiting for over 7 years. Exophagogram and manoemtric studies were consistent with achalasia. Barum study demonstrated marked esophageal dilatation and smooth tapered narrowing of the distal esophagus. However in spite of the medical treatment including the injection of the distal esophagus. However in spite of the medical treatment including the injection of botulinum toxin at the lesion site using an endoscope symptom did not improved and he suffered growth failure and malnutrition. Esophagectomy and esophagogastrostomy were performed to relieve the dysphagia. A firm circumferential intramural mass about 7x5x5 cm was found in the distal esophagus. The lumen of the esophagus was markedly dilated and esophageal wall was hypertrophied. Histologic examination of the neoplasm revealed a rather ill defined tumor tissue consisting of interlacing or whirling spindle cells without significant mitosis and esophageal leiomyo-matosis was confirmed. The patient was discharged uneventfully.

Keyword

Esophageal neoplasm; Leiomyomtosis

MeSH Terms

Botulinum Toxins
Deglutition Disorders
Dilatation
Endoscopes
Esophageal Achalasia
Esophageal Neoplasms
Esophagectomy
Esophagus
Humans
Leiomyomatosis*
Malnutrition
Mitosis
Nephritis, Hereditary*
Vomiting
Botulinum Toxins