[Gastrointestinal autonomic nerve tumor]

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 immunohistochemically 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.