Advertisement

Diagnosis and Management of Pheochromocytoma: A Practical Guide to Clinicians

  • Joseph M. PappachanEmail author
  • Diana Raskauskiene
  • Rajagopalan Sriraman
  • Mahamood Edavalath
  • Fahmy W. Hanna
Secondary Hypertension: Nervous System Mechanisms (J Bisognano, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Secondary Hypertension: Nervous System Mechanisms

Abstract

Pheochromocytomas (PCCs) are rare catecholamine producing neuroendocrine tumors. The majority of these tumors (85 %) arise from the adrenal medulla. Those arising from the extra-adrenal neural ganglia are called paragangliomas (PGLs). Paroxysmal hypertension with sweating, headaches and palpitation are the usual presenting features of PCCs/ PGLs. Gene mutations are reported in 32–79 % of cases, making genetic screening mandatory in all the cases. The malignancy rates are 10–15 % for PCCs and 20–50 % for PGLs. Measurement of plasma or 24–hour urinary fractionated metanephrines is the best biochemical diagnostic test. Computed tomography or magnetic resonance imaging has high sensitivity (90–100 %) and reasonable specificity (70–90 %) for the anatomical localization. The functionality is assessed by different radionuclide imaging modalities such as metaiodobenzylguanidine (MIBG) scintigraphy, positron emission tomography or single photon emission computed tomography. The only modality of curative treatment is tumor excision. Proper peri-operative management improves the surgical outcomes. Annual follow up with clinical and biochemical assessment is recommended in all the cases after treatment. Children, pregnant women and older people have higher morbidity and mortality risk. De-bulking surgery, chemotherapy, radiotherapy, molecular agents like sunitinib and everolimus, radionuclide agents and different ablation procedures may be useful in the palliation of inoperable/metastatic disease. An update on the diagnostic evaluation and management of PCCs and PGLs is presented here.

Keywords

Pheochromocytomas (PCCs), Paragangliomas (PGLs) Hypertension Adrenal incidentaloma Fractionated metanephrines 

Notes

Acknowledgement

We are thankful to Professor. Richard N Clayton PhD, FRCP, for the critical review of the manuscript and the suggestions for modifications.

Compliance with Ethics Guidelines

Conflict of Interest

Joseph M. Pappachan, Diana Raskauskiene, Rajagopalan Sriraman, Mahamood Edavalath, and Fahmy W. Hanna declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Kasperlik-Zaluska AA, Roslonowska E, Slowinska-Srzednicka J, et al. 1,111 patients with adrenal incidentalomas observed at a single endocrinological center: incidence of chromaffin tumors. Ann N Y Acad Sci. 2006;1073:38–46.PubMedCrossRefGoogle Scholar
  2. 2.
    Ross JJ, Desai AS, Chutkow WA, Economy KE, Dec Jr GW. Interactive medical case. A crisis in late pregnancy. N Engl J Med. 2009;361:e45.PubMedCrossRefGoogle Scholar
  3. 3.
    DeLellis RA, Lloyd RV, Heitz PU, et al. World Health Organization Classification of Tumours. Pathology and genetics of tumours of endocrine organs. Lyon: IARC Press; 2004.Google Scholar
  4. 4.
    Nieman LK. Approach to the patient with an adrenal incidentaloma. J Clin Endocrinol Metab. 2010;95:4106–13.PubMedCentralPubMedCrossRefGoogle Scholar
  5. 5.
    Arnaldi G, Boscaro M. Adrenal incidentaloma. Best Pract Res Clin Endocrinol Metab. 2012;26:405–19.PubMedCrossRefGoogle Scholar
  6. 6.••
    Mazza A, Armigliato M, Marzola MC, et al. Anti-hypertensive treatment in pheochromocytoma and paraganglioma: current management and therapeutic features. Endocrine. 2013 Jul 2. [Epub ahead of print]. This recent paper outlines the updated evidence on the utility of different antihypertensive medication regimes on the peri-operative management of PCCs and PGLs. The paper also adds on to the hypertensive complications resulting from these tumors. The points for differential diagnosis of pheochromocytoma and pseudo-pheochromocytomas are also discussed. Google Scholar
  7. 7.
    Yu R, Nissen NN, Chopra P, Dhall D, Phillips E, Wei M. Diagnosis and treatment of pheochromocytoma in an academic hospital from 1997 to 2007. Am J Med. 2009;122:85–95.PubMedCrossRefGoogle Scholar
  8. 8.
    Desai AS, Chutkow WA, Edelman E, Economy KE, Dec Jr GW. Clinical problem-solving. A crisis in late pregnancy. N Engl J Med. 2009;361:2271–7.PubMedCrossRefGoogle Scholar
  9. 9.
    Mootha VK, Feldman J, Mannting F, Winters GL, Johnson W. Pheochromocytoma-induced cardiomyopathy. Circulation. 2000;102:e11–3.PubMedCrossRefGoogle Scholar
  10. 10.
    Salathe M, Weiss P, Ritz R. Rapid reversal of heart failure in a patient with phaeochromocytoma and catecholamine-induced cardiomyopathy who was treated with captopril. Br Heart J. 1992;68:527–8.PubMedCentralPubMedCrossRefGoogle Scholar
  11. 11.
    Giavarini A, Chedid A, Bobrie G, Plouin PF, Hagège A, Amar L. Acute catecholamine cardiomyopathy in patients with phaeochromocytoma or functional paraganglioma. Heart. 2013;99:1438–44.PubMedCrossRefGoogle Scholar
  12. 12.
    Ayala-Ramirez M, Feng L, Johnson MM, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab. 2011;96:717–25.PubMedCrossRefGoogle Scholar
  13. 13.••
    Parenti G, Zampetti B, Rapizzi E, et al. Updated and new perspectives on diagnosis, prognosis, and therapy of malignant pheochromocytoma/ paraganglioma. J Oncol. 2012; 2012: 872713. A comprehensive review on the histopathological, biochemical, molecular and genetic markers that suggest malignant potential and risk of metastatis in PCCs/PGLs. The paper also highlights the therapeutic strategies for the malignant disease. Google Scholar
  14. 14.
    Goffredo P, Sosa JA, Roman SA. Malignant pheochromocytoma and paraganglioma: a population level analysis of long-term survival over two decades. J Surg Oncol. 2013;107:659–64.PubMedCrossRefGoogle Scholar
  15. 15.
    Goldstein RE, O’Neill Jr JA, Holcomb 3rd GW, et al. Clinical experience over 48 years with pheochromocytoma. Ann Surg. 1999;229:755–64.PubMedCentralPubMedCrossRefGoogle Scholar
  16. 16.
    Fishbein L, Nathanson KL. Pheochromocytoma and paraganglioma: understanding the complexities of the genetic background. Cancer Genet. 2012;205:1–11.PubMedCentralPubMedCrossRefGoogle Scholar
  17. 17.
    Fishbein L, Merrill S, Fraker D, et al. Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol. 2013;20:1444–50.PubMedCrossRefGoogle Scholar
  18. 18.
    Cascon A, Pita G, Burnichon N, et al. Genetics of pheochromocytoma and paraganglioma in Spanish patients. J Clin Endocrinol Metab. 2009;94:1701–5.PubMedCrossRefGoogle Scholar
  19. 19.•
    Crona J, Nordling M, Maharjan R, et al. Integrative genetic characterization and phenotype correlations in pheochromocytoma and paraganglioma tumours. PLoS One. 2014;9:e86756. A comprehensive paper with details of the different genetic mutations identified to date in patients with PCCs/ PGLs. PubMedCentralPubMedCrossRefGoogle Scholar
  20. 20.••
    van Berkel A, Lenders JW, Timmers HJ. DIAGNOSIS OF ENDOCRINE DISEASE: biochemical diagnosis of phaeochromocytoma and paraganglioma. Eur J Endocrinol. 2014;170:R109–19. A review that highlights all the biochemical tests useful for the diagnosis of PCCs/ PGLs with their sensitivities, specificities and predictive values. The paper gives details of the laboratory conditions and precautions necessary for the optimal performance of the tests. The details about the interfering drugs and chemicals giving rise to false positive and false negative results are also discussed. PubMedCrossRefGoogle Scholar
  21. 21.
    Chen H, Sippel RS, O’Dorisio MS, Vinik AI, Lloyd RV, Pacak K. North American Neuroendocrine Tumor Society (NANETS). The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas. 2010;39:775–83.PubMedCentralPubMedCrossRefGoogle Scholar
  22. 22.
    Lenders JW, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA. 2002;287:1427–34.PubMedCrossRefGoogle Scholar
  23. 23.
    Sawka AM, Jaeschke R, Singh RJ, Young Jr WF. A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines. J Clin Endocrinol Metab. 2003;88:553–8.PubMedCrossRefGoogle Scholar
  24. 24.
    Eisenhofer G, Goldstein DS, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: how to distinguish true- from false-positive test results. J Clin Endocrinol Metab. 2003;88:2656–66.PubMedCrossRefGoogle Scholar
  25. 25.
    Eisenhofer G, Lenders JW, Siegert G, et al. Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur J Cancer. 2012;48:1739–49.PubMedCentralPubMedCrossRefGoogle Scholar
  26. 26.
    Maurea S, Cuocolo A, Reynolds JC, et al. Iodine-131-metaiodobenzylguanidine scintigraphy in preoperative and postoperative evaluation of paragangliomas: comparison with CT and MRI. J Nucl Med. 1993;34:173–9.PubMedGoogle Scholar
  27. 27.
    Maurea S, Cuocolo A, Reynolds JC, Neumann RD, Salvatore M. Diagnostic imaging in patients with paragangliomas. computed tomography, magnetic resonance and MIBG scintigraphy comparison. Q J Nucl Med. 1996;40:365–71.PubMedGoogle Scholar
  28. 28.
    Northcutt BG, Raman SP, Long C, et al. MDCT of adrenal masses: can dual-phase enhancement patterns be used to differentiate adenoma and pheochromocytoma? AJR Am J Roentgenol. 2013;201:834–9.PubMedCrossRefGoogle Scholar
  29. 29.
    Patel J, Davenport MS, Cohan RH, Caoili EM. Can established CT attenuation and washout criteria for adrenal adenoma accurately exclude pheochromocytoma? AJR Am J Roentgenol. 2013;201:122–7.PubMedCrossRefGoogle Scholar
  30. 30.
    Derlin T, Busch JD, Wisotzki C, et al. Intraindividual comparison of 123I-mIBG SPECT/MRI, 123I-mIBG SPECT/CT, and MRI for the detection of adrenal pheochromocytoma in patients with elevated urine or plasma catecholamines. Clin Nucl Med. 2013;38:e1–6.PubMedCrossRefGoogle Scholar
  31. 31.
    Shapiro B, Copp JE, Sisson JC, Eyre PL, Wallis J, Beierwaltes WH. Iodine-131 metaiodobenzylguanidine for the locating of suspected pheochromocytoma: experience in 400 cases. J Nucl Med. 1985;26:576–85.PubMedGoogle Scholar
  32. 32.
    Lynn MD, Shapiro B, Sisson JC, et al. Pheochromocytoma and the normal adrenal medulla: improved visualization with I-123 MIBG scintigraphy. Radiology. 1985;155:789–92.PubMedCrossRefGoogle Scholar
  33. 33.
    Wiseman GA, Pacak K, O’Dorisio MS, et al. Usefulness of 123I-MIBG scintigraphy in the evaluation of patients with known or suspected primary or metastatic pheochromocytoma or paraganglioma: results from a prospective multicenter trial. J Nucl Med. 2009;50:1448–54.PubMedCrossRefGoogle Scholar
  34. 34.
    Bhatia KS, Ismail MM, Sahdev A, et al. 123I-metaiodobenzylguanidine (MIBG) scintigraphy for the detection of adrenal and extra-adrenal phaeochromocytomas: CT and MRI correlation. Clin Endocrinol (Oxf). 2008;69:181–8.CrossRefGoogle Scholar
  35. 35.
    Tenenbaum F, Lumbroso J, Schlumberger M, et al. Comparison of radiolabeled octreotide and meta-iodobenzylguanidine (MIBG) scintigraphy in malignant pheochromocytoma. J Nucl Med. 1995;36:1–6.PubMedGoogle Scholar
  36. 36.
    van der Harst E, de Herder WW, Bruining HA, et al. [(123)I]metaiodobenzylguanidine and [(111)In]octreotide uptake in benign and malignant pheochromocytomas. J Clin Endocrinol Metab. 2001;86:685–93.PubMedGoogle Scholar
  37. 37.
    Buchmann I, Henze M, Engelbrecht S, et al. Comparison of 68Ga-DOTATOC PET and 111In-DTPAOC (Octreoscan) SPECT in patients with neuroendocrine tumours. Eur J Nucl Med Mol Imaging. 2007;34:1617–26.PubMedCrossRefGoogle Scholar
  38. 38.
    Rufini V, Treglia G, Castaldi P, Perotti G, Giordano A. Comparison of metaiodobenzylguanidine scintigraphy with positron emission tomography in the diagnostic work-up of pheochromocytoma and paraganglioma: a systematic review. Q J Nucl Med Mol Imaging. 2013;57:122–33.PubMedGoogle Scholar
  39. 39.•
    Taïeb D, Timmers HJ, Hindié E, et al. EANM 2012 guidelines for radionuclide imaging of phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2012;39:1977–95. Guidelines of the European Association of Nuclear Medicine and the Society of Nuclear Medicine on various functional radionuclide imaging techniques for approaches for accurate diagnosis, staging and tumour characterization of PCCs and PGLs. The guidelines help the nuclear medicine experts in performing, interpreting and reporting the results of SPECT and PET imaging techniques for these tumors specifically in a European perspective, although it can be universalized. PubMedCrossRefGoogle Scholar
  40. 40.••
    Tsirlin A, Oo Y, Sharma R, Kansara A, Gliwa A, Banerji MA. Pheochromocytoma: a review. Maturitas. 2014. A very comprehensive review updating the current patho-physiological, genetic, diagnostic and therapeutic aspects of PCCs and PGLs. The paper highlights the different genetic syndromes associated with PCCs/ PGLs. Some aspects of malignant disease and their diagnostic and therapeutic options are also discussed. Google Scholar
  41. 41.
    Lebuffe G, Dosseh ED, Tek G, et al. The effect of calcium channel blockers on outcome following the surgical treatment of phaeochromocytomas and paragangliomas. Anaesthesia. 2005;60:439–44.PubMedCrossRefGoogle Scholar
  42. 42.
    Siddiqi HK, Yang HY, Laird AM, et al. Utility of oral nicardipine and magnesium sulfate infusion during preparation and resection of pheochromocytomas. Surgery. 2012;152:1027–36.PubMedCrossRefGoogle Scholar
  43. 43.
    Weingarten TN, Cata JP, O’Hara JF, et al. Comparison of two preoperative medical management strategies for laparoscopic resection of pheochromocytoma. Urology. 2010;76:508–e6.PubMedGoogle Scholar
  44. 44.
    Shen WT, Grogan R, Vriens M, Clark OH, Duh QY. One hundred two patients with pheochromocytoma treated at a single institution since the introduction of laparoscopic adrenalectomy. Arch Surg. 2010;145:893–7.PubMedCrossRefGoogle Scholar
  45. 45.
    Li QY, Li F. Laparoscopic adrenalectomy in pheochromocytoma: retroperitoneal approach versus transperitoneal approach. J Endourol. 2010;24:1441–5.PubMedCrossRefGoogle Scholar
  46. 46.
    Akiba M, Kodama T, Ito Y, Obara T, Fujimoto Y. Hypoglycemia induced by excessive rebound secretion of insulin after removal of pheochromocytoma. World J Surg. 1990;14:317–24.PubMedCrossRefGoogle Scholar
  47. 47.
    Buhl T, Mortensen J, Kjaer A. I-123 MIBG imaging and intraoperative localization of metastatic pheochromocytoma a case report. Clin Nucl Med. 2002;27:183–5.PubMedCrossRefGoogle Scholar
  48. 48.••
    Jimenez C, Rohren E, Habra MA, et al. Current and future treatments for malignant pheochromocytoma and sympathetic paraganglioma. Curr Oncol Rep. 2013;15:356–71. An updated review on the therapeutic options and follow up of malignant PCCs and PGLs. The newer therapeutic and investigational agents emerging in the field are discussed in detail. A comprehensive algorithm on the management of malignant disease helps clinicians to treat and follow up of these rare forms of cancers. PubMedCrossRefGoogle Scholar
  49. 49.
    Averbuch SD, Steakley CS, Young RC, et al. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Ann Intern Med. 1988;109:267–73.PubMedCrossRefGoogle Scholar
  50. 50.•
    Ayala-Ramirez M, Feng L, Habra MA, et al. Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas: insights from the largest single-institutional experience. Cancer. 2012;118:2804–12. A useful paper detailing the different chemotherapeutic regimens available, and their efficacy, for the management of malignant and metastatic PCCs and PGLs. The article empowers the clinicians to design optimal therapeutic combinations for the types of cases they encounter in the day-to-day clinical practice. PubMedCrossRefGoogle Scholar
  51. 51.
    Gonias S, Goldsby R, Matthay KK, et al. Phase II study of high-dose 131I metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma. J Clin Oncol. 2009;27:4162–8.PubMedCentralPubMedCrossRefGoogle Scholar
  52. 52.•
    van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EP. 131I-MIBG therapy for malignant paraganglioma and pheochromocytoma: systematic review and meta-analysis. Clin Endocrinol (Oxf). 2013 Oct 1. [Epub ahead of print]. The article helps the scientific community to update their patients on the benefit and limitations of 131 I-MIBG therapy for treatment of malignant and metastatic incurable PCCs/ PGLs not amenable to other treatment modalities. Google Scholar
  53. 53.
    Gulenchyn KY, Yao X, Asa SL, Singh S, Law C. Radionuclide therapy in neuroendocrine tumours: a systematic review. Clin Oncol (R Coll Radiol). 2012;24:294–308.CrossRefGoogle Scholar
  54. 54.
    Bomanji JB, Papathanasiou ND. 111In-DTPA0-octreotide (Octreoscan), 131I-MIBG and other agents for radionuclide therapy of NETs. Eur J Nucl Med Mol Imaging. 2012;39 Suppl 1:S113–25.PubMedCrossRefGoogle Scholar
  55. 55.
    Carrasquillo JA, Pandit-Taskar N, Chen CC. Radionuclide therapy of adrenal tumors. J Surg Oncol. 2012;106:632–42.PubMedCrossRefGoogle Scholar
  56. 56.
    Fishbein L, Bonner L, Torigian DA, et al. External beam radiation therapy (EBRT) for patients with malignant pheochromocytoma and non-head and -neck paraganglioma: combination with 131I-MIBG. Horm Metab Res. 2012;44:405–10.PubMedCrossRefGoogle Scholar
  57. 57.
    Chow LQ, Eckhardt SG. Sunitinib: from rational design to clinical efficacy. J Clin Oncol. 2007;25:884–96.PubMedCrossRefGoogle Scholar
  58. 58.•
    Ayala-Ramirez M, Chougnet CN, Habra MA, et al. Treatment with sunitinib for patients with progressive metastatic pheochromocytomas and sympathetic paragangliomas. J Clin Endocrinol Metab. 2012;97:4040–50. A clinical trial that shows the therapeutic benefits of the new targeted molecular therapy for the management of malignant metastatic PCCs/ PGLs not benefitting from other forms of treatment. The paper also highlights the important adverse effects related to the treatment that is useful for counseling patients before starting therapy. PubMedCentralPubMedCrossRefGoogle Scholar
  59. 59.
    von Wichert G, Jehle PM, Hoeflich A, et al. Insulin-like growth factor-I is an autocrine regulator of chromogranin A secretion and growth in human neuroendocrine tumor cells. Cancer Res. 2000;60:4573–81.Google Scholar
  60. 60.
    Oh DY, Kim TW, Park YS, et al. Phase 2 study of everolimus monotherapy in patients with nonfunctioning neuroendocrine tumors or pheochromocytomas/ paragangliomas. Cancer. 2012;118:6162–70.PubMedCrossRefGoogle Scholar
  61. 61.
    McBride JF, Atwell TD, Charboneau WJ, Young Jr WF, Wass TC, Callstrom MR. Minimally invasive treatment of metastatic pheochromocytoma and paraganglioma: efficacy and safety of radiofrequency ablation and cryoablation therapy. J Vasc Interv Radiol. 2011;22:1263–70.PubMedCrossRefGoogle Scholar
  62. 62.
    Watanabe D, Tanabe A, Naruse M, et al. Transcatheter arterial embolization for the treatment of liver metastases in a patient with malignant pheochromocytoma. Endocr J. 2006;53:59–66.PubMedCrossRefGoogle Scholar
  63. 63.
    Hidaka S, Hiraoka A, Ochi H, et al. Malignant pheochromocytoma with liver metastasis treated by transcatheter arterial chemo-embolization (TACE). Intern Med. 2010;49:645–51.PubMedCrossRefGoogle Scholar
  64. 64.
    Kumar P, Bryant T, Breen D, Stedman B, Hacking N. Transarterial embolization and doxorubicin eluting beads-transarterial chemoembolization (DEB-TACE) of malignant extra-adrenal pheochromocytoma. Cardiovasc Intervent Radiol. 2011;34:1325–9.PubMedCrossRefGoogle Scholar
  65. 65.
    Plouin PF, Amar L, Lepoutre C. Phaeochromocytomas and functional paragangliomas: clinical management. Best Pract Res Clin Endocrinol Metab. 2010;24:933–41.PubMedCrossRefGoogle Scholar
  66. 66.
    Amar L, Servais A, Gimenez-Roqueplo AP, Zinzindohoue F, Chatellier G, Plouin PF. Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma. J Clin Endocrinol Metab. 2005;90:2110–6.PubMedCrossRefGoogle Scholar
  67. 67.
    Diner EK, Franks ME, Behari A, Linehan WM, Walther MM. Partial adrenalectomy: the National Cancer Institute experience. Urology. 2005;66:19–23.PubMedCrossRefGoogle Scholar
  68. 68.
    Walz MK, Peitgen K, Diesing D, Petersenn S, Janssen OE, Philipp T, et al. Partial versus total adrenalectomy by the posterior retroperitoneoscopic approach: early and long-term results of 325 consecutive procedures in primary adrenal neoplasias. World J Surg. 2004;28:1323–9.PubMedCrossRefGoogle Scholar
  69. 69.
    Grubbs EG, Rich TA, Ng C, et al. Long-term outcomes of surgical treatment for hereditary pheochromocytoma. J Am Coll Surg. 2013;216:280–9.PubMedCrossRefGoogle Scholar
  70. 70.
    Pham TH, Moir C, Thompson GB, et al. Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center. Pediatrics. 2006;118:1109–17.PubMedCrossRefGoogle Scholar
  71. 71.
    Beltsevich DG, Kuznetsov NS, Kazaryan AM, Lysenko MA. Pheochromocytoma surgery: epidemiologic peculiarities in children. World J Surg. 2004;28:592–6.PubMedCrossRefGoogle Scholar
  72. 72.
    Volkin D, Yerram N, Ahmed F, et al. Partial adrenalectomy minimizes the need for long-term hormone replacement in pediatric patients with pheochromocytoma and von Hippel-Lindau syndrome. J Pediatr Surg. 2012;47:2077–82.PubMedCrossRefGoogle Scholar
  73. 73.••
    Lenders JW. Pheochromocytoma and pregnancy: a deceptive connection. Eur J Endocrinol. 2012;166:143–50. A very useful paper that gives the practical implications of management of PCCs and PGLs during pregnancy. The risks and complications related to the disease in pregnancy are highlighted. The article also gives the clinicians an idea about the optimal work up, intra and postpartum management of PCCs/ PGLs and the precautions necessary for operative management. PubMedCrossRefGoogle Scholar
  74. 74.
    Oliva R, Angelos P, Kaplan E, Bakris G. Phaeochromocytoma in pregnancy: a case series and review. Hypertension. 2010;55:600–6.PubMedCrossRefGoogle Scholar
  75. 75.
    Harrington JL, Farley DR, van Heerden JA, Ramin KD. Adrenal tumours and pregnancy. World J Surg. 1999;23:182–6.PubMedCrossRefGoogle Scholar
  76. 76.
    Mannelli M, Bemporad D. Diagnosis and management of phaeochromocytoma during pregnancy. J Endocrinol Invest. 2002;25:567–71.PubMedCrossRefGoogle Scholar
  77. 77.
    Song Y, Liu J, Li H, Zeng Z, Bian X, Wang S. Outcomes of concurrent Caesarean delivery and pheochromocytoma resection in late pregnancy. Int Med J. 2013;43:588–91.CrossRefGoogle Scholar
  78. 78.
    Cammarano WB, Gray AT, Rosen MA, Lim KH. Anesthesia for combined cesarean section and extra-adrenal phaeochromocytoma resection: a case report and literature review. Int J Obstet Anesth. 1997;6:112–7.PubMedCrossRefGoogle Scholar
  79. 79.
    Kopetschke R, Slisko M, Kilisli A, et al. Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma. Eur J Endocrinol. 2009;161:355–61.PubMedCrossRefGoogle Scholar
  80. 80.
    Jiang CX, Zeng Z, Wang T, Liu X, Liu R, Li Y. Pheochromocytomas in adrenal medulla or extra-adrenal and multiple endocrine neoplasms: a clinicopathologic analysis of 181 cases. Zhonghua Bing Li Xue Za Zhi. 2011;40:762–6.PubMedGoogle Scholar
  81. 81.
    Bittner 4th JG, Gershuni VM, Matthews BD, Moley JF, Brunt LM. Risk factors affecting operative approach, conversion, and morbidity for adrenalectomy: a single-institution series of 402 patients. Surg Endosc. 2013;27:2342–50.PubMedCrossRefGoogle Scholar
  82. 82.
    Khorram-Manesh A, Ahlman H, Nilsson O, et al. Long-term outcome of a large series of patients surgically treated for pheochromocytoma. J Intern Med. 2005;258:55–66.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  • Joseph M. Pappachan
    • 1
    Email author
  • Diana Raskauskiene
    • 1
  • Rajagopalan Sriraman
    • 2
  • Mahamood Edavalath
    • 3
  • Fahmy W. Hanna
    • 3
  1. 1.Department of EndocrinologyWalsall Manor HospitalWest MidlandsUK
  2. 2.Department of EndocrinologyLincoln County HospitalLincolnUK
  3. 3.Department of EndocrinologyUniversity Hospital of North StaffordshireStoke-on-TrentUK

Personalised recommendations