[Leiomyosarcoma of the esophagus. Clinical aspects, diagnosis and therapy based on an individual case].
Leiomyosarcomas of the esophagus are rare tumors of mesenchymal origin. Apropos of a case we present clinicopathological features, diagnostic procedures and management of this seldom tumor of the esophagus. Primary gastrointestinal sarcomas cause less than 0.5% of all esophageal malign tumors, and present in ca. 5% as esophageal leiomyosarcomas. The most frequent incidence ranges between the fourth and fifth decade of life. The tumors originate from the muscular layers of the esophageal wall and are localized predominantly in the middle and distal third of the esophagus. Dysphagia is the most important and leading symptom although it presents late in the course of the illness. Endosonography is at the time the most accurate method to establish the tumor size. Differentiation between leiomyoma and leiomyosarcoma is only possible by histopathological examination and may be difficult in certain cases. Histopathological grading of the tumors as low- and high-grade sarcomas in dependence of the number of mitosis affects predominantly the prognosis of these patients. Differential diagnosis includes spindle cell carcinoma and carcinosarcoma of the esophagus. The most effective therapy consists in the complete operative removal of the tumor, in these cases five years survival rates of 30 to 40% are achieved, strongly influenced by tumor differentiation and size.
Pesarini
A C
AC
Medizinische Klinik I mit Poliklinik, Universitat Erlangen-Nürnberg.
Ernst
H
H
Ell
C
C
Wittekind
C
C
Hahn
E G
EG
D000328:Adult; D003937:Diagnosis, Differential; D019160:Endosonography; D004938:Esophageal Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D004945:Esophagoscopy; D004947:Esophagus / Q000473:pathology / Q000601:surgery; D006801:Humans; D007890:Leiomyosarcoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D008297:Male