A 15‐year experience with chemotherapy of patients with paraganglioma

Background. Paraganglioma is a rare tumor thought to arise from the neuroectodermally derived paraganglionic cells that are dispersed widely along the autonomic ganglia. They can metastasize to bones, lymph nodes, and lungs and occasionally present with spinal cord compression. Up to 60% of retroperitoneal paragangliomas can be functional, with symptoms and signs of norepinephrine overproduction. Method. A 15‐year experience with chemotherapy of patients with paraganglioma was reviewed. The patient population was identified through a search of the database 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 confirmed diagnosis of paraganglioma were treated with chemotherapy. The median age was 42 years (range, 25–67 years); there were eight males and five females. Primary sites included retroperitoneum (seven patients), head and neck (two patients), pelvis, bladder, mediastinum, and paravertebral (one patient each). Twelve patients received chemotherapy for metastatic disease, and 1 had an unresectable mediastinal primary tumor. Eleven patients received cyclophosphamide, doxorubicin, and DTIC/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 (response 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. Cyclophosphamide, doxorubicin, dacarbazine chemotherapy is active in the treatment of patients with paraganglioma.