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
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Dr. A. Beck
Klinik für Allgemein- und Thoraxchirurgie
Städtisches Klinikum Karlsruhe
Moltkestraße 90
D-76133 Karlsruhe
