Korean J Thorac Cardiovasc Surg.
2000 Jan;33(1):112-115.
Esophageal Leiomyomatosis in a patient with Alport Syndrome
- Affiliations
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- 1Department of Thoracic and Cardiovascular Surgery, College of Medicine, Kyung Hee University.
- 2Deparment of Anatomical Pathology, College of Medicine, Kyung Hee University.
Abstract
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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.