A 15-YEAR EXPERIENCE WITH CHEMOTHERAPY OF PATIENTS WITH PARAGANGLIOMA

Background. Paraganglioma is a rare tumor thought to arise from the ne uroectodermally derived paraganglionic cells that are dispersed widely along the autonomic ganglia. They can metastasize to bones, lymph nod es, and lungs and occasionally present with spinal cord compression. U p to 60% of retroperitoneal paragangliomas can be functional, with sym ptoms and signs of norepinephrine overproduction. Method. A 15-year ex perience with chemotherapy of patients with paraganglioma was reviewed . The patient population was identified through a search of the databa se maintained by the Departments of Melanoma-Sarcoma Medical Oncology and Pathology at the University of Texas M.D. Anderson Cancer Center ( Houston, TX). Results. Thirteen of 84 patients with histologically con firmed diagnosis of paraganglioma were treated with chemotherapy. The median age was 42 years (range, 25-67 years); there were eight males a nd five females. Primary sites included retroperitoneum (seven patient s), head and neck (two patients), pelvis, bladder, mediastinum, and pa ravertebral (one patient each). Twelve patients received chemotherapy for metastatic disease, and 1 had an unresectable mediastinal primary tumor. Eleven patients received cyclophosphamide, doxorubicin, and DTI C/dacarbazine (CyADIC)/cyclophosphamide, vincristine, doxorubicin and DTIC/dacarbazine (CyVADIC), and 2 received doxorubicin and dacarbazine (ADIC) at standard doses for a median of 4 cycles (range, 2-12 cycles ). Six of 13 patients achieved an objective partial remission (respons e rate = 46%, 95% confidence interval = 19-73%); 6 other patients had stable disease, and one developed progressive disease, At the time of last follow-up, eight patients were alive with disease, four died, and one patient was alive with no evidence of disease, The median follow- up from diagnosis was 45 months (range, 12-300 months). Conclusion. Cy clophosphamide, doxorubicin, dacarbazine chemotherapy is active in the treatment of patients with paraganglioma.