The management of benign and malignant pheochromocytoma and abdominal paraganglioma

Eur J Surg Oncol. 2003 Apr;29(3):278-83. doi: 10.1053/ejso.2002.1413.

Abstract

Aims: To report treatment and outcome in patients with malignant and benign pheochromocytoma and abdominal paraganglioma.

Methods: Review of clinical and therapeutic features in 85 patients with pheochromocytoma or abdominal paraganglioma between 1976 and 1999.

Results: Thirty-nine of 85 patients presented with symptoms other than classical paroxysmal attacks. Paragangliomas were more often malignant (7/15) than pheochromocytomas (7/70). No recurrences have occurred in 71 patients with tumours initially classified as benign after a median follow-up time of 144 months (range 7-287). Nine of 14 patients with tumours classified as malignant have developed metastasis and/or local recurrence. Treatment of malignant tumours with cyclophosphamide, vincristine and dacarbazine (CVD) improved or stabilised the disease in three of four patients.

Conclusion: Life long follow-up of patients with benign pheochromocytoma is not necessary. Combination chemotherapy (CVD) is a valid option in the treatment of malignant pheochromocytomas and abdominal paragangliomas.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abdominal Neoplasms / pathology
  • Abdominal Neoplasms / therapy*
  • Adolescent
  • Adrenal Gland Neoplasms / pathology
  • Adrenal Gland Neoplasms / therapy*
  • Adult
  • Aged
  • Combined Modality Therapy
  • Female
  • Humans
  • Male
  • Middle Aged
  • Paraganglioma / pathology
  • Paraganglioma / therapy*
  • Pheochromocytoma / pathology
  • Pheochromocytoma / therapy*
  • Retrospective Studies
  • Treatment Outcome