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Vol. 17, No. 6, 2000
Issue release date: 2000
Dig Surg 2000;17:627–631

Malignant Peripheral Nerve Sheath Tumor of the Esophagus

Manger T. · Pross M. · Haeckel C. · Lippert H.
Departments of aSurgery and bPathology, Otto von Guericke University, Magdeburg, Germany

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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.

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  1. Lauwers GY, Erlandson RA, Casper ES, Brennan MF, Woodruff JM: Gastrointestinal autonomic nerve tumors. A clinicopathological, immunohistochemical, and ultrastructural study of 12 cases. Am J Surg Pathol 1993;17:887–897.
  2. Drews G, Keine S, Emmrich P, Lehmann J. Malignant tumor of the autonomic nervous system of the stomach (GAN-tumor): Case report of a new entity (in German). Zentralbl Chir 1992;117:564–58.
  3. Perch SJ, Soffen EM, Whittington R, Brooks JJ: Esophageal sarcoma. Surg Oncol 1991;48:194–198.
  4. Lukash WM, Hyams VJ, Nielson OF: Neurogenic neoplasms of the small bowel: Benign nonchromaffin paragangliom of the duodenum. Am J Dig Dis 1966;7:575.
  5. Herrera GA, DeMorales HP, Grizzle WE, Han SG: Malignant small bowel neoplasm of enteric plexus derivation (plexosarcoma). Light and electron microscopic study confirming the origin of the neoplasms. Dig Dis Sci 1984;29:275.
  6. Pinedo Moraleda F, Martinez Gonzales MA, Ballestin Carcavilla C, Vargas Castrillon J: Gastrointestinal autonomic nerve tumors: A case report with ultrastructural and immunohistochemical studies. Histopathology 1992;20:323–329.
  7. Ducatman BS, Scheithauer BW: Malignant peripheral nerve sheath tumor with divergent differentiation. Cancer 1984;54:1049–1057.

    External Resources

  8. Enzinger FM, Weiss SS: Soft Tissue Tumors, ed 3. St Louis, Mosby, 1995, pp 889–928.
  9. Woodruff JM: Tumors and tumorlike conditions of the peripheral nerve; in Ninfo V, Chung EB, Cavazzana AO (eds): Tumors and Tumor-Like Lesions of Soft Tissue. Contemporary Issues in Surgical Pathology. New York, Churchill Livingstone, 1991, vol 18, pp 205–228.
  10. Brooks JSJ, Freeman M, Enterline HAT: Malignant ‘Triton’ tumors. Natural history and immunohistochemistry of nine cases with literature review. Cancer 1985;55:2543–2585.
  11. Weiss SW: Histological Typing of Soft Tissue Tumours, ed 2. WHO International Histological Classification of Tumours. Springer, Berlin, 1994.
  12. Wittekind Ch, Wagner G: TNM-Klassifikation maligner Tumoren, 5 Aufl. Berlin, Springer, 1997.
  13. Van Unnik JAM, Coindre JM, Contesso C, Albus-Lutter CE, Schiodt T, Sylvester R, et al: Grading of soft tissue sarcomas: Experience of the EORTC Soft Tissue and Bone Sarcoma Group. Eur J Cancer 1993;29A:2089–2093.
  14. Hermanek P, Henson DE, Hutter RVP, Sobin LH (eds): UICC TNM Supplement 1993. A Commentary on Uniform Use. Berlin, Springer, 1993.
  15. Bloch MJ, Iozzo RV, Edmunds LH, Brooks JJ: Polypoid synovial sarcoma of the esophagus. Gastroenterology 1987;92:229–233.

    External Resources

  16. Choh JH, Khazei AH, Ihm HJ: Leiomyosarcoma of the esophagus: Report of a case and review of the literature. J Surg Oncol 1986;32:223–226.

    External Resources

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