Malignant peripheral nerve sheath tumor of the esophagus

Dig Surg. 2000;17(6):627-631. doi: 10.1159/000051973.


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.

Conclusion: 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.

Publication types

  • Case Reports

MeSH terms

  • Esophageal Neoplasms / diagnosis
  • Esophageal Neoplasms / metabolism
  • Esophageal Neoplasms / pathology
  • Esophageal Neoplasms / surgery*
  • Esophagectomy* / methods
  • Esophagus / surgery
  • Female
  • Gastroscopy
  • Humans
  • Immunochemistry
  • Middle Aged
  • Nerve Sheath Neoplasms / diagnosis
  • Nerve Sheath Neoplasms / metabolism
  • Nerve Sheath Neoplasms / pathology
  • Nerve Sheath Neoplasms / surgery*
  • Plastic Surgery Procedures