Regional Pheochromocytoma
Current Clinical Trials
Standard treatment options: [1-6]
Pheochromocytoma with regional lymphatic metastasis or local extension should
be treated by aggressive surgical resection with an attempt to remove all gross
evidence of disease. If this is accomplished, and confirmed by biochemical
determinations, long-term survival may be achieved. These patients, however,
will require careful monitoring indefinitely for recurrent disease. If
regional disease remains, the hypertension and symptoms caused by catecholamine
excess should be treated by adrenergic blockade and catecholamine synthesis
inhibition as necessary.
Radiation therapy or combination chemotherapy may be palliative for symptoms or
morbidity resulting from local invasion by tumor. Treatment with targeted radiation
therapy using I131 meta-iodobenzylguanidine (I131 MIBG) has met with limited
success. In approximately 35% of the patients screened, the tumor has sufficient
uptake of the radioisotope to allow for a therapeutic dose.[7,8] In a group of
28 patients 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]
Several single agents and drug combinations have been evaluated in a limited
number of patients with variable results.[7] The most active chemotherapy
regimen appears to be the combination of cyclophosphamide, vincristine, and
dacarbazine (CVD).[10] CVD has been shown to produce partial remissions of
moderate duration in symptomatic patients. Analysis of 23 patients treated
with CVD showed 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
contributes to improved patient survival. Chemotherapy should be used only for
palliation in symptomatic patients.[7,11]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with regional 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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Manger WM, Gifford RW: Pheochromocytoma. New York: Springer-Verlag, 1977.
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Norton JA: Adrenal tumors. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2005, pp 1528-39.
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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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Remine WH, Chong GC, Van Heerden JA, et al.: Current management of pheochromocytoma. Ann Surg 179 (5): 740-8, 1974.
[PUBMED Abstract]
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McEwan AJ, Shapiro B, Sisson JC, et al.: Radio-iodobenzylguanidine for the scintigraphic location and therapy of adrenergic tumors. Semin Nucl Med 15 (2): 132-53, 1985.
[PUBMED Abstract]
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Drasin H: Treatment of malignant pheochromocytoma. West J Med 128 (2): 106-11, 1978.
[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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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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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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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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