Volume 1073, Issue 1 p. 505-511

Courses of Malignant Pheochromocytoma

Implications for Therapy

JAMES C. SISSON

JAMES C. SISSON

Division of Nuclear Medicine, Department of Radiology, University of Michigan Health System, Ann Arbor, Michigan 48109, USA

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BARRY L. SHULKIN

BARRY L. SHULKIN

Division of Nuclear Medicine, Department of Radiological Sciences, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA

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NAZANENE H. ESFANDIARI

NAZANENE H. ESFANDIARI

Division of Endocrinology and Metabolism, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, Michigan 48109, USA

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First published: 07 September 2006
Citations: 23
Address for correspondence: James C. Sisson, M.D., Division of Nuclear Medicine, UH B1 G505D, University of Michigan Health System, Ann Arbor, MI 48109-0028. Voice: 734-936-5387; fax: 734-936-8182.
 e-mail: [email protected]

Abstract

Abstract: Survival of patients with metastatic pheochromocytoma that have exceeded 30 years without therapy to reduce tumors have been reported. We reviewed the records of 38 patients with malignant pheochromocytoma who had received 131I-metaiodiobenzylguanidine (131I-MIBG) treatments between 1981 and 1996 to evaluate longevity. Survival from diagnosis to last follow-up exceeded 5 years in 21 of 38 (55%) and ≥10 years in 50%. In 17 of 21, the interval from diagnosis to 131I-MIBG therapy was greater than 5 years. Survival following 131I-MIBG was ≥5 years in 12 of 17 and ≥10 years in 7 of 17 patients despite continued evidence of excessive circulating catecholamines. Objective responses to 131I-MIBG therapy were seen in about 30% and were usually of a few years, duration, but one individual exhibited marked reductions in volume and function of tumors that have persisted for 21 years. No feature, including a remission of >5 years following surgical excision, was found to predict prolonged survival. In summary, many patients with malignant pheochromocytoma will follow a course extending over many years. The role of 131I-MIBG therapy in longevity is uncertain, but this radiopharmaceutical reduces evidence of tumors in some patients. Criteria for selecting patients who will benefit from treatment remain to be determined.