Malignant peripheral nerve sheath tumor of the esophagus.
BACKGROUND
Sarcomas of the esophagus are rare representing 0.1-1.5% of all esophageal tumors. We report a case of malignant peripheral nerve sheath tumor (MPNST) of the esophagus in a 60-year-old woman.


METHODS
The diagnosis was made preoperatively on endoscopic biopsy and confirmed after tumor resection by immunohistochemistry as well as electron microscopy. The patient underwent abdominal-thoracicen bloc esophagectomy with mediastinal lymphadenectomy and intrathoracic esophagogastrostomy.


RESULTS
Our therapeutic concept for the first case of a high-grade MPNST (malignant schwannoma) of the esophagus resulted in a recurrence-free interval of 4 years.


CONCLUSIONS
The therapy of choice was abdominal-thoracic en bloc esophagectomy with tumor-free resection margins and esophageal reconstruction with the stomach. After R0-resection we found no indication for adjuvant radio- and/or chemotherapy.
Manger
T
T
Department of Surgery, Otto von Guericke University, Magdeburg, Germany. thomas.manger@medizin-uni-magdeburg.de
Pross
M
M
Haeckel
C
C
Lippert
H
H
D004938:Esophageal Neoplasms / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D016629:Esophagectomy / Q000379:methods; D004947:Esophagus / Q000601:surgery; D005260:Female; D005773:Gastroscopy; D006801:Humans; D007120:Immunochemistry; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D019651:Plastic Surgery Procedures