Gastrointestinal autonomic nerve tumors

Gastrointestinal autonomic nerve tumors (GAN-tumor) are rare malignant neur ogenic stromal tumors of the intestinal tract. The origin is suspected in t he 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 re sected and immunhistochemically verified as GAN-tumor. Within the follow-up of 29 months metastases appeared within the omentum majus with a diffuse p eritoneal spreading. Several trials of adjuvant chemotherapy (adriamycine/i fosamide, taxotere, gemcitabine/xyloda) were ineffective. 15 months after t he second operation the patient died. Since the first description of the GA N-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 sei ze of more than 10 cm is associated with recurrences in 64% of the cases wi thin 2 years. Since there is no option for medical treatment, surgical rese ction is the treatment of choice and has to be considered also in the case of recurrence.