Current Clinical Trials

Standard treatment options:

Sites of metastatic disease most commonly observed include lymph nodes, bones, liver, and lungs. Liver involvement may be the result of direct extension from right-sided primary tumors. Biochemical confirmation of recurrence and localization of metastatic lesions with I¹³¹ meta-iodobenzylguanidine (I¹³¹ MIBG) scans can confirm the presence of metastasis. Aggressive surgical resection of accessible recurrent disease or metastases that will render the patient free of gross disease with the potential for normal biochemical determinations should be attempted. If successful, long-term survival may be achieved; however, careful monitoring for recurrent disease will be necessary indefinitely.[1,2]

No evidence exists that partial surgical debulking of tumor results in improved survival or reduction in symptoms. Surgical intervention or radiation therapy may be indicated for palliation of local complications due to metastatic disease. Long-term medical management of symptoms using adrenergic blockade and catecholamine synthesis inhibition is indicated and will prevent catastrophic complications from chronic and extreme catecholamine excess.[3,4]

Several single agents and drug combinations have been used in a limited number of patients with variable results.[5,6] The most active chemotherapy regimen appears to be the combination of cyclophosphamide, vincristine, and dacarbazine (CVD).[7] CVD has been shown to produce partial remissions of moderate duration in symptomatic patients. Analysis of 23 patients treated with CVD showed that 61% of the patients had objective evidence of tumor regression, and 74% of the patients had evidence of biochemical response. In addition, improved control of hypertension, reduced need for antihypertensive medications, and improvement in overall performance status was observed. Since hypertensive episodes have been reported following chemotherapy, patients need to be prepared with adrenergic blockers prior to treatment. No evidence exists that chemotherapy prolongs the survival of patients with metastatic disease. Chemotherapy should be used only for palliation in symptomatic patients.[5,7]

External radiation therapy can achieve palliation of painful bone metastases.

Treatment with targeted radiation therapy using I¹³¹ MIBG has met with limited success. In approximately 35% of patients screened, the tumor has sufficient uptake of the radioisotope to allow for a therapeutic dose.[5,8] Of 28 patients who were shown to have sufficient uptake of I¹³¹ MIBG, objective partial responses were observed in 29% of the patients, and biochemical improvement was noted in 43% of the patients.[9]

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic pheochromocytoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. Drasin H: Treatment of malignant pheochromocytoma. West J Med 128 (2): 106-11, 1978.  [PUBMED Abstract]
   
   
2. Brennan MF, Keiser HR: Persistent and recurrent pheochromocytoma: the role of surgery. World J Surg 6 (4): 397-402, 1982.  [PUBMED Abstract]
   
   
3. Young JB, Landsberg L: Catecholamines and the adrenal medulla: pheochromocytoma. In: Wilson JD, Foster DW, Kronenberg HM, et al., eds.: Williams Textbook of Endocrinology. 9th ed. Philadelphia, Pa: W.B. Saunders Company, 1998, pp 705-716. 
   
   
4. Bravo EL, Gifford RW Jr: Current concepts. Pheochromocytoma: diagnosis, localization and management. N Engl J Med 311 (20): 1298-303, 1984.  [PUBMED Abstract]
   
   
5. Kvols LK, Perry RR, Vinik AI, et al.: Neoplasms of the neuroendocrine system and neoplasms of the gastroenteropancreatic endocrine system. In: Holland JC, Frei E, eds.: Cancer Medicine e.5. 5th ed. Hamilton, Ontario: B.C. Decker Inc, 2000, pp 1121-1172. 
   
   
6. Schlumberger M, Gicquel C, Lumbroso J, et al.: Malignant pheochromocytoma: clinical, biological, histologic and therapeutic data in a series of 20 patients with distant metastases. J Endocrinol Invest 15 (9): 631-42, 1992.  [PUBMED Abstract]
   
   
7. Averbuch SD, Steakley CS, Young RC, et al.: Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Ann Intern Med 109 (4): 267-73, 1988.  [PUBMED Abstract]
   
   
8. Shapiro B, Fig LM: Management of pheochromocytoma. Endocrinol Metab Clin North Am 18 (2): 443-81, 1989.  [PUBMED Abstract]
   
   
9. Shapiro B, Sisson JC, Wieland DM, et al.: Radiopharmaceutical therapy of malignant pheochromocytoma with [131I]metaiodobenzylguanidine: results from ten years of experience. J Nucl Biol Med 35 (4): 269-76, 1991 Oct-Dec.  [PUBMED Abstract]
   
   

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