eMedicine Specialties > Endocrinology > Adrenal Gland

Pheochromocytoma: Follow-up

Author: Ann T Sweeney, MD, Associate Professor, Department of Medicine, Division of Endocrinology, Tufts University School of Medicine
Coauthor(s): Michael A Blake, MRCPI, FRCR, Assistant Professor, Department of Radiology, Harvard Medical School; Staff Radiologist, Division of Abdominal Imaging, Massachusetts General Hospital
Contributor Information and Disclosures

Updated: Jul 31, 2009

Follow-up

Further Inpatient Care

  • Test plasma free metanephrines 2 weeks postoperatively. If results are within the reference range, resection is deemed complete and patient survival approaches age-matched controls.
  • Assure resolution of the hypertension and any associated complications.

Further Outpatient Care

  • Obtain plasma metanephrine levels yearly for 5 years. Assure that blood pressure is under control.
  • The 5-year survival rate for people with nonmalignant pheochromocytomas is greater than 95%. In those with malignant pheochromocytomas, the 5-year survival rate is less than 50%.

Patient Education

  • For excellent patient education material, see the patient education article High Blood Pressure.

Miscellaneous

Medicolegal Pitfalls

  • The major pitfalls are in not considering the diagnosis in a timely manner or in ordering a test that provokes a hypertensive crisis with complications. The symptoms of pheochromocytoma may be quite vague and misrepresented by the patient. Most difficult are the true episodic secreting pheochromocytomas wherein the blood pressure may appear normal between paroxysms. Because this constitutes failure to diagnose a potentially curable problem with otherwise life-threatening consequences, pheochromocytomas have attracted litigation.

Special Concerns

  • Pregnancy
    • Pheochromocytoma occurring during pregnancy carries a grave prognosis, with maternal and fetal mortality rates of 48% and 55%, respectively.
    • Maternal mortality is virtually eliminated and the fetal mortality rate is reduced to 15% if the diagnosis is made antenatally.
    • Administer alpha-adrenergic blockade (phenoxybenzamine) as soon as the diagnosis is confirmed.
    • Surgically remove the tumor as soon as possible during the first 2 trimesters after proper preparation. Pregnancy need not be terminated.
    • Spontaneous abortion is very likely.
    • During the third trimester, as soon as fetal lung maturity is confirmed, perform surgical removal of the tumor and follow with cesarean delivery.
 
Acknowledgments

The authors and editors of this topic gratefully acknowledge the contributions of previous coauthor James C Melby, MD, to the development and writing of this article.



More on Pheochromocytoma

Overview: Pheochromocytoma
Differential Diagnoses & Workup: Pheochromocytoma
Treatment & Medication: Pheochromocytoma
Follow-up: Pheochromocytoma
Multimedia: Pheochromocytoma
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Sheps SG, Jiang NS, Klee GG, van Heerden JA. Recent developments in the diagnosis and treatment of pheochromocytoma. Mayo Clin Proc. Jan 1990;65(1):88-95. [Medline].

  2. Beard CM, Sheps SG, Kurland LT, et al. Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clin Proc. Dec 1983;58(12):802-4. [Medline].

  3. Boulkina LS, Newton CA, Drake AJ 3rd, Tanenberg RJ. Acute myocardial infarction attributable to adrenergic crises in a patient with pheochromocytoma and neurofibromatosis 1. Endocr Pract. May-Jun 2007;13(3):269-73. [Medline].

  4. Neumann HP, Berger DP, Sigmund G, et al. Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. N Engl J Med. Nov 18 1993;329(21):1531-8. [Medline].

  5. Gergics P, Patocs A, Toth M, et al. Germline VHL gene mutations in Hungarian families with von Hippel-Lindau disease and patients with apparently sporadic unilateral phaeochromocytomas. Eur J Endocrinol. Jul 2 2009;[Medline].

  6. Kudva YC, Sawka AM, Young WF Jr. Clinical review 164: The laboratory diagnosis of adrenal pheochromocytoma: the Mayo Clinic experience. J Clin Endocrinol Metab. Oct 2003;88(10):4533-9. [Medline].

  7. de Jong WH, Eisenhofer G, Post WJ, et al. Dietary influences on plasma and urinary metanephrines: implications for diagnosis of catecholamine-producing tumors. J Clin Endocrinol Metab. Jun 30 2009;[Medline].

  8. Baid SK, Lai EW, Wesley RA, Ling A, Timmers HJ, Adams KT, et al. Brief communication: radiographic contrast infusion and catecholamine release in patients with pheochromocytoma. Ann Intern Med. Jan 6 2009;150(1):27-32. [Medline].

  9. Bessell-Browne R, O'Malley ME. CT of pheochromocytoma and paraganglioma: risk of adverse events with i.v. administration of nonionic contrast material. AJR Am J Roentgenol. Apr 2007;188(4):970-4. [Medline]. [Full Text].

  10. Faria JF, Goldman SM, Szejnfeld J, Melo H, Kater C, Kenney P, et al. Adrenal masses: characterization with in vivo proton MR spectroscopy--initial experience. Radiology. Dec 2007;245(3):788-97. [Medline]. [Full Text].

  11. Kim S, Salibi N, Hardie AD, Xu J, Lim RP, Lee VS, et al. Characterization of adrenal pheochromocytoma using respiratory-triggered proton MR spectroscopy: initial experience. AJR Am J Roentgenol. Feb 2009;192(2):450-4. [Medline].

  12. Wong C, Yu R. Preoperative preparation for pheochromocytoma resection: physician survey and clinical practice. Exp Clin Endocrinol Diabetes. Jul 16 2009;[Medline].

  13. Blake MA, Kalra MK, Maher MM, et al. Pheochromocytoma: an imaging chameleon. Radiographics. Oct 2004;24 Suppl 1:S87-99. [Medline].

  14. Blake MA, Krishnamoorthy SK, Boland GW, et al. Low-density pheochromocytoma on CT: a mimicker of adrenal adenoma. AJR Am J Roentgenol. Dec 2003;181(6):1663-8.

  15. Bravo EL, Gifford RW Jr. Current concepts. Pheochromocytoma: diagnosis, localization and management. N Engl J Med. Nov 15 1984;311(20):1298-303. [Medline].

  16. Bravo EL, Gifford RW Jr. Pheochromocytoma. Endocrinol Metab Clin North Am. Jun 1993;22(2):329-41. [Medline].

  17. Cruz SR, Colwell JA. Pheochromocytoma and ileus. JAMA. Feb 21 1972;219(8):1050-1. [Medline].

  18. Eisenhofer G, Lenders JW, Linehan WM, et al. Plasma normetanephrine and metanephrine for detecting pheochromocytoma in von Hippel-Lindau disease and multiple endocrine neoplasia type 2. N Engl J Med. Jun 17 1999;340(24):1872-9. [Medline].

  19. Forman BH, Marban E, Kayne RD, et al. Ectopic ACTH syndrome due to pheochromocytoma: case report and review of the literature. Yale J Biol Med. Mar-Apr 1979;52(2):181-9. [Medline].

  20. Gifford RW, Kvale WF, Maher FT. Clinical features, diagnosis and treatment of pheochromocytoma: A review of 76 cases. Mayo Clin Proc. Apr 1964;39:281-302. [Medline].

  21. Heath H 3rd, Edis AJ. Pheochromocytoma associated with hypercalcemia and ectopic secretion of calcitonin. Ann Intern Med. Aug 1979;91(2):208-10. [Medline].

  22. Jiménez C, Cote G, Arnold A, Gagel RF. Review: Should patients with apparently sporadic pheochromocytomas or paragangliomas be screened for hereditary syndromes?. J Clin Endocrinol Metab. Aug 2006;91(8):2851-8. [Medline].

  23. Keiser HR. Pheochromocytoma and other diseases of the sympathetic nervous system. In: Becker KL, Bilezikian JP, Bremner WJ, et al, eds. Principles and Practice of Endocrinology and Metabolism. 2nd ed. Philadelphia, Pa: JB Lippincott; 1995. 762-70.

  24. Kudva YC, Young WF Jr. Lightheaded spells and hypertension. Lancet. Oct 18 1997;350(9085):1140. [Medline].

  25. Lenders JW, Keiser HR, Goldstein DS, et al. Plasma metanephrines in the diagnosis of pheochromocytoma. Ann Intern Med. Jul 15 1995;123(2):101-9. [Medline].

  26. Lenders JW, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: which test is best?. JAMA. Mar 20 2002;287(11):1427-34. [Medline].

  27. Manger WM, Gifford RW. Clinical and Experimental Pheochromocytoma. 2nd ed. Cambridge, Mass:. Blackwell Science;1996.

  28. Mayo CH. Paroxysmal hypertension with tumor of the retroperitoneal nerve: report of a case. JAMA. 1927;89:1047-50.

  29. Samaan NA, Hickey RC. Pheochromocytoma. Semin Oncol. Sep 1987;14(3):297-305. [Medline].

  30. Sweeney AT, Malabanan AO, Blake MA, et al. Megacolon as the presenting feature in pheochromocytoma. J Clin Endocrinol Metab. Nov 2000;85(11):3968-72. [Medline].

  31. Szolar DH, Korobkin M, Reittner P, et al. Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT. Radiology. Feb 2005;234(2):479-85. [Medline].

  32. Trampal C, Engler H, Juhlin C, et al. Pheochromocytomas: detection with 11C hydroxyephedrine PET. Radiology. 2004;230:423-428. [Medline].

  33. van Heerden JA. First encounters with pheochromocytoma. The story of Mother Joachim. Am J Surg. Aug 1982;144(2):277-9. [Medline].

  34. Young WF Jr. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med. Feb 8 2007;356(6):601-10. [Medline].

  35. Young WF Jr, Maddox DE. Spells: in search of a cause. Mayo Clin Proc. Aug 1995;70(8):757-65. [Medline].

[ CLOSE WINDOW ]

Keywords

pheochromocytoma, adrenal gland, adrenal glands, catecholamine, catecholamines, paraganglioma, multiple endocrine neoplasia, catecholamine-secreting tumor, extra-adrenal pheochromocytomas, familial pheochromocytoma, sporadic pheochromocytoma, multiple endocrine neoplasia 2A, multiple endocrine neoplasia 2B, MEN 2A, MEN 2B, neurofibromatosis, von Hippel-Lindau disease, VHL disease, pheochromocytoma-induced hypertensive crises, hypertensive encephalopathy, Von Recklinghausen disease, Sipple syndrome, tuberous sclerosis, Bourneville disease, Epiloia, Sturge-Weber syndrome, Cushing syndrome, postural hypotension, hypertensive retinopathy, cafe au lait spots

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Ann T Sweeney, MD, Associate Professor, Department of Medicine, Division of Endocrinology, Tufts University School of Medicine
Ann T Sweeney, MD is a member of the following medical societies: American Association of Clinical Endocrinologists and Endocrine Society
Disclosure: Nothing to disclose.

Coauthor(s)

Michael A Blake, MRCPI, FRCR, Assistant Professor, Department of Radiology, Harvard Medical School; Staff Radiologist, Division of Abdominal Imaging, Massachusetts General Hospital
Michael A Blake, MRCPI, FRCR is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Radiological Society of North America, Royal College of Physicians of Ireland, and Royal College of Surgeons in Ireland
Disclosure: Nothing to disclose.

Medical Editor

Daniel Einhorn, MD, FACP, FACE, Medical Director, Scripps/Whittier Diabetes Institute, Department of Medicine, Associate Clinical Professor of Medicine, University of California at San Diego School of Medicine
Daniel Einhorn, MD, FACP, FACE is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, Phi Beta Kappa, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Romesh Khardori, MD, Chief, Division of Endocrinology, Metabolism and Molecular Medicine, Professor, Department of Internal Medicine, Southern Illinois University School of Medicine
Romesh Khardori, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society of Andrology, Endocrine Society, and Illinois State Medical Society
Disclosure: Nothing to disclose.

CME Editor

Mark Cooper, MBBS, PhD, FRACP, Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University
Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD, Professor of Medicine, St Louis University School of Medicine
George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.