Download PDF
Zentralbl Chir 2001; 126(9): 702-706
DOI: 10.1055/s-2001-18245
Der interessante Fall

J.A.Barth Verlag in Medizinverlage Heidelberg GmbH & Co.KG

Der gastrointestinale autonome Nerventumor

Gastrointestinal autonomic nerve tumorsA. Beck1 , J. Jonas1 , H. Frenzel2 , R. Bähr1
  • 1Klinik für Allgemein- und Thoraxchirurgie (Direktor: Prof. Dr. R. Bähr) und
  • 2Pathologisches Institut (Direktor: Prof. Dr. H. Frenzel), Städtisches Klinikum Karlsruhe
Further Information

Publication History

Publication Date:
06 November 2001 (online)

Also available at
    Permissions and Reprints

Gastrointestinal autonomic nerve tumors

Summary

Gastrointestinal autonomic nerve tumors (GAN-tumor) are rare malignant neurogenic stromal tumors of the intestinal tract. The origin is suspected in the autonomic nerve plexus Meissner or Auerbach with the interstitial cells of Cajal as precursors. We report on a 53-year-old patient with a clinical apparent and radiological 5 cm measuring tumor of the jejunum, which was resected and immunhistochemically verified as GAN-tumor. Within the follow-up of 29 months metastases appeared within the omentum majus with a diffuse peritoneal spreading. Several trials of adjuvant chemotherapy (adriamycine/ifosamide, taxotere, gemcitabine/xyloda) were ineffective. 15 months after the second operation the patient died. Since the first description of the GAN-tumor in 1984 87 patients were reported in the literature. No recurrences or metastasis were seen in tumors with a seize less than 5 cm. A tumor seize of more than 10 cm is associated with recurrences in 64 % of the cases within 2 years. Since there is no option for medical treatment, surgical resection is the treatment of choice and has to be considered also in the case of recurrence.

Zusammenfassung

Der gastrointestinale autonome Nerventumor (GAN-Tumoren) ist ein seltener, maligner, neurogener Stromatumor des Intestinaltraktes. Wir berichten über einen 53jährigen Patienten mit einem 5 cm großen, vorwiegend extramural wachsenden Tumor des Jejunums, welcher immunhistologisch als GAN-Tumor differenziert wurde. 29 Monate nach der Erstdiagnose fanden sich große Tumormetastasen im Omentum majus, der linken paracolischen Rinne und fein verteilt über das gesamte Peritoneum. Eine adjuvante Chemotherapie mit Adriamycin/Ifosamid, Taxotere, sowie Gemcitabin/Xyloda war erfolglos. 15 Monate nach der Tumorreduktion bzw. 44 Monate nach der Erstdiagnose verstarb der Patient. Lokalrezidive und Lebermetastasen nach Entfernung von kleinen GAN-Tumoren (< 5 cm) wurden bisher nicht beschrieben. Prognostisch ungü nstige Kriterien scheinen eine große Tumormasse und eine hohe Mitoserate zu sein. Größere Tumore entwickeln peritoneale Rezidive und bevorzugt Lebermetastasen. Die operative Resektion ist derzeit auch beim Auftreten eines Rezidivs die Therapie der Wahl. Eine Empfehlung für eine adjuvante Chemotherapie kann nicht gegeben werden.

Key words

Gastrointestinal autonomic nerve tumor - GANT - plexosarcoma - skeinoid fibers

Schlüsselwörter

Gastrointestinaler Nerventumor - Plexosarkom - Skeinoid fibers

Literatur

  • 1 Akle C A, Wardle D G, Bomanji J B, Talbot I. MIBG diagnosis and therapy in smooth musle tumours of the small bowel.  Eur J Nucl Med. 1997;  24 1196-1199
    CrossrefPubMedGoogle Scholar
  • 2 Blei E, Gonzales Crussi F. The intriguing nature of gastric tumors in Carney's triad.  Cancer. 1992;  69 292-300
    PubMedGoogle Scholar
  • 3 Böhm J, Radebold K, Meister P, Hofler H. Tumor des autonomen enterischen Nervensystems des Magens (GANTumor): Fallbericht einer seltenen neuen Entität.  Verh Dt Ges Pathol. 1991;  75 283-285
    PubMedGoogle Scholar
  • 4 Damiani S, Schildkraut P, Riguzzi P, Biasiucci A, Tardio M L, Eusebi V. Gastrointestinal autonomic nerve tumor (GANT) associated with von Recklinghausen's disease.  Pathologica. 1998;  90 792-797
    PubMedGoogle Scholar
  • 5 Dhimes P, Lopez C M, Ortega S M, Garcia-Munoz H. Gastrointestinal autonomic nerve tumors and their separation from other gastrointestinal stroma tumors.  Virchows Arch. 1995;  426 27-35
    PubMedGoogle Scholar
  • 6 Donner L R. Gastrointestinal autonomic nerve tumor.  Ultrastruct Pathol. 1997;  21 419-424
    PubMedGoogle Scholar
  • 7 Drews G, Liene S, Emmrich P, Lehmann J. Maligner Tumor des autonomen Nervensystems des Magens (GAN-Tumor).  Zentralbl Chir. 1992;  117 564-568
    PubMedGoogle Scholar
  • 8 Flückiger R, Wegmann W, Huber A. Tumor des gastrointestinalen autonomen Nervensystems GAN- Tumor oder Plexosarkom.  Chirurg. 1996;  67 371-379
    PubMedGoogle Scholar
  • 9 Garcia-Rostan Y, Perez G M, Diaz M M, Bragado F G. Jejunal stromal tumor with skeinoid fibers or myenteric plexoma: A case report.  Pathol Internat. 1997;  47 774-800
    PubMedGoogle Scholar
  • 10 Herrera G A, De Morales H P, Gizzle W E, Han S G. Malignant small bowel neoplasm of enteric plexus derivation (plexosarkoma).  Dig Dis Sci. 1984;  29 275-284
    CrossrefPubMedGoogle Scholar
  • 11 Herrera G A, Cerezo L, Jones J E, Sack J. Gastrointestinal autonomic nerve tumors (plexosarcomas).  Arch Pathol Lab Med. 1989;  113 846-853
    PubMedGoogle Scholar
  • 12 Hirota S, Okazaki T, Kitamura Y, O'Brian P, Kapusta L, Dardick I. Cause of familial and multiple gastrointestinal autonomic nerve tumors with hyperplasia of interstitial cells of Cajal is germline mutation of the c-kit gene.  Am J Surg Pathol. 2000;  24/2 326-327
    PubMedGoogle Scholar
  • 13 Honda K, Mikami T, Ohkusa T. et al . Gastrointestinal autonomic nerve tumor with giant abscess.  J Clin Gastroenterol. 1997;  24 280-285
    CrossrefPubMedGoogle Scholar
  • 14 Ishida T, Wada I, Horiuchi H, Oka T, Machinami R. Multiple small intestinal stromal tumors with skeinoid fibers in association with neurofibromatosis (von Recklinghausen's disease).  Pathol Int. 1996;  46 689-695
    PubMedGoogle Scholar
  • 15 Isimbaldi G, Santangelo M, Cenacchi G. et al . Gastrointestinal autonomic nerve tumor (plexosarcoma).  Acta Cytologica. 1998;  42 1189-1194
    PubMedGoogle Scholar
  • 16 Kerr J Z, Hicks M J, Nuchtern J G. et al . Gastrointestinal autonomic nerve tumors in pedatric population.  Cancer. 1999;  85 220-230
    CrossrefPubMedGoogle Scholar
  • 17 Kodet R, Snajdauf J, Smelhaus V. Gastrointestinal autonomic nerve tumor.  Pediatr Pathol. 1994;  14 1005-1016
    PubMedGoogle Scholar
  • 18 Lam K Y, Law S Y, Chu K M, Ma L T. Gastrointestinal autonomic nerve tumor of the esophagus.  Cancer. 1996;  78 1651-1659
    CrossrefPubMedGoogle Scholar
  • 19 Lauwers G Y, Erlandson R A, Casper E S, Brennan M F, Woodruff J M. Gastrointestinal autonomic nerve tumors.  Am J Surg Pathol. 1993;  17 887-897
    PubMedGoogle Scholar
  • 20 Lespi J, Drut R. Gastrointestinal autonomic tumor associated with von Recklinghausen's disease.  Acta Gastroenterol Latinoam. 1997;  27 271-274
    PubMedGoogle Scholar
  • 21 MacLeod C B, Tsokos M. Gastrointestinal nerve tumor.  Ultrastruct Pathol. 1991;  15 49-55
    PubMedGoogle Scholar
  • 22 Min K W. Skeinoid fibers: an ultrastructural marker of neurologic spindle cell tumors.  Ultrastruct Pathol. 1991;  15 603-611
    PubMedGoogle Scholar
  • 23 Min K-W. Small intestinal stromal tumors with skeinoid fibers.  Am J Surg Pathol. 1992;  16 145-155
    PubMedGoogle Scholar
  • 24 Minni F, Casadei R, Santini D. et al . Gastrointestinal autonomic nerve tumor of the jejunum.  Italy J Gastroenterol Hepatol. 1997;  29 558-563
    PubMedGoogle Scholar
  • 25 Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors.  Human Pathol. 1999;  30/10 1213-1220
    PubMedGoogle Scholar
  • 26 Nerlich A, Remberger K. Metastasierendes „Plexosarkom” des Jejunums.  Pathologe. 1990;  11 240-243
    PubMedGoogle Scholar
  • 27 O'Brian P, Kapusta L, Dardick I, Axler J, Gnidec A. Multiple familial gastrointestinal autonomic nerve tumors and small intestinal neuronal dysplasia.  Am J Surg Path. 1999;  23 198-204
    CrossrefPubMedGoogle Scholar
  • 28 Ojanguren I, Ariza A, Navas-Palacios J J. Gastrointestinal autonomic nerve tumor.  Human Pathol. 1996;  27 1311-1318
    PubMedGoogle Scholar
  • 29 Perez-Atayde A R, Shamberger R C, Kozakewich H WP. Neuroectodermal differentiation of the gastrointestinal tumors in the Carney Triad.  Am J Surg Pathol. 1991;  17 706-714
    PubMedGoogle Scholar
  • 30 Pinedo Moralda F, Martinez Gonzales M A. et al . Gastrointestinal autonomic nerve tumors.  Histopathology. 1992;  20 323-329
    PubMedGoogle Scholar
  • 31 Pross M, Manger Th, Schulz H U. et al . Gastrointestinale Stromatumoren (GIST).  Chirurg. 1999;  70 807-812
    CrossrefPubMedGoogle Scholar
  • 32 Rosai J. Stromal tumors. In: Rosai J (ed). Ackermann's surgical pathology. Mosby, St. Louis, Baltimore, Boston 1995; 645
    Google Scholar
  • 33 Sakaguchi N, Sano K, Ito M, Baba T, Fukuzawa M, Hotchi M. A case of von Recklinghausen's disease with bilateral pheochromocytoma-malignant peripheral nerve sheath tumors of the adrenal and gastrointestinal autonomic nerve tumors.  Am J Surg Pathol. 1996;  20 889-897
    CrossrefPubMedGoogle Scholar
  • 34 Segal A, Carello S, Caterina P L. Gastrointestinal autonomic nerve tumors and their separation from other gastrointestinal stromal tumors.  Virchows Arch. 1994;  426 27-35
    PubMedGoogle Scholar
  • 35 Shanks J H, Harris M, Banerjee SS, Eyden B P. Gastrointestinal autonomic nerve tumors.  Histopathology. 1996;  29 111-121
    CrossrefPubMedGoogle Scholar
  • 36 Thomas J R, Mrak R E, Liuit N. Gastrointestinal autonomic nerve tumor presenting as high grade sarcoma.  Dig Dis Sci. 1994;  39 2051-2055
    PubMedGoogle Scholar
  • 37 Tornoczky T, Kalman E, Hegedus G. et al . High mitotic index associated with poor prognosis in gastrointestinal autonomic nerve tumor.  Histopathology. 1999;  35 121-128
    CrossrefPubMedGoogle Scholar
  • 38 Tortella B J, Antonioli D A, Dvorak A M, Matthewsj A, Silen W. Gastric autonomic nerve (GAN) tumor and extra-adrenal paraganglioma in Carney's triad.  Ann Surg. 1987;  205 221-225
    PubMedGoogle Scholar
  • 39 Tsagatakis T, Karayiannis G, Fragiadaki H. et al . Stroma GAN tumor of the stomach.  Arch Hellenic Medicine. 1998;  15 81-84
    PubMedGoogle Scholar
  • 40 Ueyama T, Guo K-J, Hashimoto H, Daimaru Y, Muenetomo E. A clinicopathologic and immunohistochemic study of gastrointestinal stromal tumors.  Cancer. 1991;  69 947-955
    PubMedGoogle Scholar
  • 41 Walker P, Dvorak A M. Gastrointestinal autonomic nerve (GAN) tumor: ultrastructural evidence for a newly recognized entity.  Arch Pathol Lab Med. 1986;  110 309-316
    PubMedGoogle Scholar
  • 42 Walsh N M, Bodurtha A. Auerbachs myenteric plexus.  Pathol Lab Med. 1990;  114 522-525
    PubMedGoogle Scholar
  • 43 Wang B Y, Brandwein M S, Gordon R E, Chahinian P A, Strauchen J A, Harpaz N. Gastrointestinal autonomic nerve tumor of the colon.  Internat J Surg Pathol. 1998;  6 171-176
    PubMedGoogle Scholar

Dr. A. Beck

Klinik für Allgemein- und Thoraxchirurgie
Städtisches Klinikum Karlsruhe

Moltkestraße 90

D-76133 Karlsruhe

      >