Skip to main content
SpringerLink
Log in

Das Leiomyosarkom des Ösophagus

Leiomyosarcoma of the esophagus: Case report, clinicopathological features, diagnosis and management

Klinik, Diagnostik und Therapie anhand eines Falles

  • Klinisch-Pathologische Konferenz
  • Published:
Medizinische Klinik Aims and scope Submit manuscript

Zusammenfassung

Das Leiomyosarkom des Ösophagus ist ein, seltener Tumor mesenchymalen Ursprungs. Anhand eines Falles wird die klinische Symptomatik und das diagnostische Vorgehen dargestellt sowie auf Therapie und Pathohistologie eingegangen. Primäre gastrointestinale Sarkome stellen weniger als 0,5% aller Ösophagusmalignome dar, sie befallen die Speiseröhre in etwa 5%. Die höchste Inzidenz liegt in der vierten und fünften Lebensdekade. Die Tumoren sind submukös lokalisiert und betreffen die, muskulären Wandschichten überwiegend im mittleren und distalen Anteil des Ösophagus. Dysphagie als klinisches Leitsymptom tritt erst spät im Verlauf der Erkrankung auf. Die Endosonographie ist die derzeit genaueste diagnostische Methode zur Bestimmung der Tumorgröße. Eine Differenzierung zwischen benignem Leiomyom und malignem Leiomyosarkom ist nur histopathologisch möglich und kann in Einzelfällen schwierig sein. Von prognostischer Bedeutung ist die Differenzierung in „low-” und „high-grade” Sarkome aufgrund der Mitosenrate. Die Differentialdiagnose umfaßt das Spindelzellkarzinom und das Karzinosarkom der Speiseröhre. Die operative Entfernung des Tumors stellt die Therapie der Wahl dar; die Fünf-Jahres-Überlebensrate liegt zwischen 30 und 40% und ist von der Tumordifferenzierung und der Tumorgröße abhängig.

Summary

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 symptome 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 leio-myosarcoma 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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Literatur

  1. Aagaard, M. T., I. B. Kristensen, O. Lund, J. M. Hasenkam, H. H. Kimose: Primary malignant non-epithelial tumors of the thoracic oesophagus and cardia in a 25-year surgical material. Scand. J. Gastroent. 25 (1990), 876–882.

    Article  PubMed  CAS  Google Scholar 

  2. Aimoto, T., K. Sasajima, S. Kyono, E. Uchida, M. Watanabe, K. Yamashita, K. Takubo, M. Onda: Leiomyosarcoma of the esophagus: report of a case and preoperative evaluation by CT scan, endoscopic ultrasonography and angiography. Gastroent. Jap. 27 (1992), 773–779.

    CAS  Google Scholar 

  3. Akwari, O. E., R. R. Dozois, L. H. Weiland, O. H. Beahrs: Leiomyosarcoma of the small and large bowel. Cancer (Philad.) 42 (1978), 1375–1384.

    Article  CAS  Google Scholar 

  4. Bardi, G., B. Johansson, N. Pandis, S. Heim, N. Mandahl, E. Bak Jensen, H. Frederiksen, A. Andren Sandberg, F. Mitelman: Recurrent chromosome aberrations in abdominal smooth muscle tumors. Cancer Gen. Cytogen. 62 (1992), 43–46.

    Article  CAS  Google Scholar 

  5. Bloch, M. J., R. V. Iozzo, L. H. Edmunds, J. J. Brooks: Polypoid synovial sarcoma of the esophagus. Gastroenterology 92 (1987), 229–233.

    PubMed  CAS  Google Scholar 

  6. Boyce, G. A., M. V. Sivak, T. Roesch, M. Classen, D. E. Fleischer, H. W. Boyce, C. J. Lightdale, J. F. Bodet, R. H. Hawes, G. A. Lehman: Evaluation of submucosal upper gastrointestinal tract lesions by endoscopic ultrasound. Gastrointest. Endosc. 37 (1991), 449–454.

    Article  PubMed  CAS  Google Scholar 

  7. Boyce, H. W.: Tumors of the esophagus. In: Sleisenger, M. H., J. S. Fordtran: Gastrointestinal disease. Pathophysiology, diagnosis, management. 5. Aufl. Saunders & Co., Philadelphia-London 1993, p. 401–418.

    Google Scholar 

  8. Caldwell, C. B., M. S. Bains, M. E. Burt: Unusual malignant neoplasms of the esophagus. Oat cell carcinoma, melanoma, and sarcoma. J. thorac. cardiovasc. Surg. 101 (1991), 100–107.

    PubMed  CAS  Google Scholar 

  9. Camishion, R. C., J. H. Gibbon, J. V. Templeton: Leiomyosarcomas of the esophagus. Ann. Surg. 53 (1961), 951–956.

    Article  Google Scholar 

  10. Chiba, W., T. Konishi, S. Sawai, R. Hatakenaka, Y. Matsubara, S. Ikeda: A resected case of leiomyosarcoma of the esophagus — the diagnosis was supported with DNA content analysis. Nippon Kyobu Geka Gakkai Zasshi 41 (1993), 140–144.

    PubMed  CAS  Google Scholar 

  11. Choh, J. H., A. H. Khazei, H. J. Ihm: Leiomyosarcoma of the esophagus: report of a case and review of the literature. J Surg. Oncol. 32 (1986), 223–226.

    Article  PubMed  CAS  Google Scholar 

  12. De Mas, C. R., G. Schnepper, E. Seifert, M. Stolte: Das gestielt polypoide Karzinosarkom: ein sehr seltener Ösophagustumor. Leber Magen Darm 25 (1995), 180–182.

    PubMed  Google Scholar 

  13. De Muth, W. E.: Rhabdomyosarcoma of the esophagus. J. thorac. cardiovasc. Surg. 32 (1956), 115–119.

    Google Scholar 

  14. Dittler, H. J., A. C. Pesarini, J. R. Siewert: Endoscopic classification of esophageal cancer: correlation which the T-Stage. Gastrointest. Endosc. 38 (1992), 662–668.

    Article  PubMed  CAS  Google Scholar 

  15. Dittler, H. J., J. R. Siewert: Role of endoscopic ultrasonography in esophageal carcinoma. Endoscopy 25 (1993), 156–161.

    Article  PubMed  CAS  Google Scholar 

  16. Dougherty, M. J., C. Compton, M. Talbert, W. C. Wood: Sarcomas of the gastrointestinal tract. Separation into favorable and unfavorable prognostic groups by mitotic count. Ann. Surg. 214 (1991), 569–574.

    Article  PubMed  CAS  Google Scholar 

  17. Dvorak, H. J.: Sarcoma of the esophagus. Arch. Surg. 22 (1931), 794–798.

    Google Scholar 

  18. Evans, H. L.: Smooth muscle tumors of the gastrointestinal tract. A study of 56 cases followed for a minimum of 10 years. Cancer (Philad.) 56 (1985), 2242–2250.

    Article  CAS  Google Scholar 

  19. Fockens, P., J. F. Bartelsman, G. N. Tytgat: Benign and malignant esophageal tumors other than squamous and adenocarcinoma. Gastrointest. Endosc. Clin. N. Amer. 4 (1994), 791–801.

    CAS  Google Scholar 

  20. Franklin, G. O., A. S. Antler, W. L. Thelmo, W. S. Rosenthal: Esophageal leiomyosarcoma. N. Y. St. J. Med. 82 (1982), 1100–1103.

    CAS  Google Scholar 

  21. Fujikura, T., T. Koishizawa, N. Hayashi, J. Kokubo, K. Nonaka, T. Fujiki, K. Ikeda, K. Sudoh, S. Takayasu, F. Kikuchi: Operative removal of esophageal leiomyosarcoma: a case report. Kyobu Geka 48 (1995), 967–970.

    PubMed  CAS  Google Scholar 

  22. Goodner, J. T., T. R. Miller, W. L. Watson: Sarcomas of the esophagus. Amer. J. Radiol. 89 (1963), 132–136.

    CAS  Google Scholar 

  23. Gordon, M. S., S. Hajdu, M. S. Bains, M. E. Burt: Soft tissue sarcomas of the chest wall. Results of surgical resection. J. thorac. cardiovasc. Surg. 101 (1991), 843–854.

    PubMed  CAS  Google Scholar 

  24. Hanada, M., K. Nakano, Y. Ii, H. Yamashita: Carcinosarcoma of the esophagus with osseous and cartilagenous production. A combined study of keratin immunohistochemistry and electron microscopy. Acta path. jap. 34 (1984), 669–678.

    PubMed  CAS  Google Scholar 

  25. Jia, Q. R.: Differential diagnosis and analysis of 34 cases with esophageal and cardiac leiomyoma and leiomyosarcoma. Chung-Hua Wai Ko Tsa Chih 28 (1990), 214–215.

    PubMed  CAS  Google Scholar 

  26. Kajiyama, T., M. Sakai, A. Torii, H. Kishimoto, G. Kin, S. Uose, S. Ueda, M. Okuma, K. Inoue: Endoscopic aspiration lumpectomy of esophageal leiomyomas derived from the muscularis mucosae. Amer. J. Gastroent. 90 (1995), 417–422.

    PubMed  CAS  Google Scholar 

  27. Koga, H., M. Iida, H. Suekane, K. Aoyagi, T. Yao, Y. Kimura, N. Masuda, M. Fujishima: Rapidly growing esophageal leiomyosarcoma: case report and review of the literature. Abdom. Imag. 20 (1995), 15–19.

    Article  CAS  Google Scholar 

  28. Lee, Y. T.: Leiomyosarcoma of the gastro-intestinal tract: general pattern of metastasis and recurrence. Cancer Treatm. Rev. 10 (1983), 91–101.

    Article  CAS  Google Scholar 

  29. Martin, J. D., J. M. Grisamore: Leiomyosarcoma of the esophagus. Surg. Gynec. Obstet. 107 (1958), 238–242.

    PubMed  Google Scholar 

  30. Matsumori, M., T. Mukai, T. Tsukube, Y. Watanabe, T. Ienaga, H. Sato, A. Sasada, K. Nakamura: A two-stage operation successfully performed for giant leiomyosarcoma of the esophagus with hepatic metastasis. Surg. Today 22 (1992), 543–547.

    Article  PubMed  CAS  Google Scholar 

  31. McIntyre, M., J. N. Webb, G. C. Browning: Osteosarcoma of the esophagus. Hum. Path. 13 (1982), 680–682.

    Article  PubMed  CAS  Google Scholar 

  32. Moran, C. A., S. Suster, G. Perino, M. Kaneko, M. N. Koss: Malignant smooth muscle tumors presenting as mediastinal soft tissue masses. A clinicopathologic study of 10 cases. Cancer (Phila.) 74 (1994), 2251–2260.

    Article  CAS  Google Scholar 

  33. Nakajima, J., G. Shindo, H. Makuuchi, M. Mathison, K. Tanaka, J. Deguchi, A. Furuse, O. Kobori: Successful resection of mediastinal and abdominal recurrent tumors of esophageal leiomyosarcoma 12 years after surgery. Kyobu Geka 43 (1990), 102–105.

    PubMed  CAS  Google Scholar 

  34. Ohnishi, T., H. Yoshioka, O. Ishida: MR imaging of gastrointestinal leiomyosarcoma. Radiat. Med. 9 (1991), 114–117.

    PubMed  CAS  Google Scholar 

  35. Patel, S. R., N. Anandarao: Leiomyosarcoma of the esophagus. N. Y. St. J. Med. 90 (1990), 371–373.

    CAS  Google Scholar 

  36. Perch, S. J., E. M. Soffen, R. Whittington, J. J. Brooks: Esophageal sarcomas. J. surg. Oncol. 48 (1991), 194–198.

    Article  PubMed  CAS  Google Scholar 

  37. Rainer, W. G., A. Bras: Leiomyosarcomas of the esophagus: review of the literature and report of three cases. Surgery 58 (1963), 343–347.

    Google Scholar 

  38. Ranchod, M., R. L. Kempson: Smooth muscle tumors of the gastrointestinal tract and retroperitoneum: a pathologic analysis of 100 cases. Cancer (Philad.) 39 (1977), 255–262.

    Article  CAS  Google Scholar 

  39. Roesch, T., M. Classen, H. J. Dittler: Gastroenterologic Endosonography. Textbook and Atlas. Thieme, Stuttgart 1992.

    Google Scholar 

  40. Roesch, T., R. Lorenz, H. Dancygier, A. von Wickert, M. Classen: Endosonographic diagnosis of submucosal upper gastrointestinal tract tumors. Scand. J. Gastroent. 27 (1992), 1–8.

    Article  Google Scholar 

  41. Roth, J. A., A. S. Lichter, J. B. Putnam, A. A. Forastiere: Cancer of the esophagus. In: De Vita, V. T., S. Hellman, S. A. Rosenberg: Cancer: principles and practice of oncology. 4. Aufl. Lippincott. Philadelphia 1993, p. 776–812.

    Google Scholar 

  42. Shiraishi, M., T. Takahashi, M. Yamashiro, H. Hashimoto, T. Noro, Y. Hino, T. Hirashima, K. Kuroiwa, K. Takubo: A report of leiomyosarcoma of the esophagus). Nippon Ronen Igakkai Zasshi 32 (1995, 286–291.

    PubMed  CAS  Google Scholar 

  43. Shiu, M. H., G. H. Farr, D. N. Papachristou, S. I. Hajdu: Myosarcomas of the stomach: natural history, prognostic factors and management. Cancer (Philad.) 49 (1982), 177–187.

    Article  CAS  Google Scholar 

  44. Sons, H. U., F. Borchard: Esophageal cancer. Autopsy findings in 171 cases. Arch. Path. Lab. Med. 108 (1984), 983–988.

    PubMed  CAS  Google Scholar 

  45. Sugihira, N., K. Matsumoto, T. Tada, I. Fujino, H. Urata, T. Yamamoto, H. Suzuki: DNA flow cytometry in smooth muscle tumors of the gastrointestinal tract. Gan To Kagaku Ryoho 20 (1993), 713–716.

    PubMed  CAS  Google Scholar 

  46. Tauchi, K., Y. Tsutsumi, S. Yoshimura, K. Watanabe: Immunohistochemical and immunoblotting detection of cytokeratin in smooth muscle tumors. Acta path. jap. 40 (1990), 574–580.

    PubMed  CAS  Google Scholar 

  47. Tio, T. L., G. N. Tytgat, F. C. den Hartog-Jager: Endoscopic ultrasonography for the evaluation of smooth muscle tumors in the upper gastrointestinal tract: an experience with 42 cases. Gastrointest. Endosc. 36 (1990), 342–350.

    Article  PubMed  CAS  Google Scholar 

  48. Tsushima, K., L. M. Rainwater, J. R. Goellner, J. A. van Heerden, M. M. Lieber: Leiomyosarcomas and benign smooth muscle tumors of the stomach: nuclear DNA patterns studied by flow cytometry. Mayo Clin. Proc. 62 (1987), 275–280.

    PubMed  CAS  Google Scholar 

  49. Vezeridis, M. P., H. J. Wanebo: Gastrointestinal sarcomas. In: Ahlgren, J. D., J. S. Macdonald: Gastrointestinal oncology. Lippincott, Philadelphia 1992, p. 499–506.

    Google Scholar 

  50. Vrettou, E., G. Karkavelas, B. Christoforidou, S. Meditskou, C. S. Papadimitriou: Immunohistochemical phenotyping and PCNA detection in gastrointestinal stromal tumors. Anticancer Res. 15 (1995), 943–949.

    PubMed  CAS  Google Scholar 

  51. Wang, X. J., W. H. Tang, P. L. Yu: Histological and immunohistochemical study and AgNOR analysis of smooth muscle tumors of the digestive tract. Chung Hua Chung Liu Tsa Chih 16 (1994), 128–131.

    PubMed  CAS  Google Scholar 

  52. Wang, Z. Y., M. Itabashi, T. Hirota, H. Watanabe, H. Kato: Immunohistochemical study of the histogenesis of esophageal carcinosarcoma. Jap. J. clin. Oncol. 22 (1992), 377–386.

    CAS  Google Scholar 

  53. Watanabe, H., J. R. Jass, I. H. Sobin: International histological classification of tumours: Histological typing of oesophageal and gastric tumours. 2. Aufl. Springer, Berlin-Heidelberg-New York 1990.

    Google Scholar 

  54. Wen, J.: Biological behavior of smooth muscle tumors of the alimentary tract — a pathological analysis of 501 cases. Chung Hua Chung Liu Tsa Chih 13 (1991), 217–219.

    PubMed  CAS  Google Scholar 

  55. Wen, J.: An immunohistochemical and ultrastructural survey on smooth muscle tumors of the alimentary tract. Chung Hua Ping Li Hsueh Tsa Chih 21 (1992), 146–148.

    PubMed  CAS  Google Scholar 

  56. Wolfel, D. A.: Leiomyosarcoma of the esophágus. Amer. J. Radiol. 89 (1963), 127–131.

    CAS  Google Scholar 

  57. Yang, P. C., S. Davis: Incidence of cancer of the esophagus in the US by histologic type. Cancer (Philad.) 61 (1988), 612–617.

    Article  CAS  Google Scholar 

  58. Yasuda, K., E. Cho, M. Nakajima, K. Kawai: Diagnosis of submucosal lesions of the upper gastrointestinal tract by endoscopic ultrasonography. Gastrointest. Endosc. 36, Suppl. 2 (1990), S17-S20.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Medizinische Klinik I mit Poliklinik, Universitätsstraße 12, D-91054, Erlangen

    Andrea -Carlo Pesarini, Helmut Ernst, Christian Ell & Eckhart Georg Hahn

  2. Abteilung für Pathologie an der Chirurgischen Klinik der Universität Erlangen-Nürnberg, Erlangen-Nürnberg, Deutschland

    Christian Wittekind

Authors
  1. Andrea -Carlo Pesarini
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Helmut Ernst
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Christian Ell
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Christian Wittekind
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Eckhart Georg Hahn
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Pesarini, A.C., Ernst, H., Ell, C. et al. Das Leiomyosarkom des Ösophagus. Med Klin 92, 234–240 (1997). https://doi.org/10.1007/BF03043265

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF03043265

Search

Navigation