Metastatic Pheochromocytoma
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]
Current Clinical Trials
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
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Drasin H: Treatment of malignant pheochromocytoma. West J Med 128 (2): 106-11, 1978.
[PUBMED Abstract]
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Brennan MF, Keiser HR: Persistent and recurrent pheochromocytoma: the role of surgery. World J Surg 6 (4): 397-402, 1982.
[PUBMED Abstract]
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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.
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Bravo EL, Gifford RW Jr: Current concepts. Pheochromocytoma: diagnosis, localization and management. N Engl J Med 311 (20): 1298-303, 1984.
[PUBMED Abstract]
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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.
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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]
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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]
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Shapiro B, Fig LM: Management of pheochromocytoma. Endocrinol Metab Clin North Am 18 (2): 443-81, 1989.
[PUBMED Abstract]
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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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