Phaeochromocytoma (suspected)

Population Covered By The Guidance

This pathway provides guidance on the imaging of adult patients with a confirmed biochemical diagnosis of primary phaeochromocytoma.

Lead Researcher: Arjun Shivananda

Experts & Contributors: Ravinder Dhillon, David Hurley, Kyaw Thura

Editorial Panel: Core membership
Link to Editorial Panel

Date reviewed: November 2017

Date Published: April 2018

Image 1 (Computed Tomography): Round low attenuation lesion at upper pole of left kidney consistent with phaeochromocytoma (arrow).

Phaeochromocytoma

Image 2a (I123-MIBG) and 2b (Computed Tomography): High grade focus of uptake identified at the left side of the upper abdomen posteriorly (arrow). CT demonstrates a cystic phaeochromocytoma (arrow).

Phaeochromocytoma

Image 2a (I123-MIBG) and 2b (Computed Tomography): High grade focus of uptake identified at the left side of the upper abdomen posteriorly (arrow). CT demonstrates a cystic phaeochromocytoma (arrow).

Phaeochromocytoma

Image 3a (I123-MIBG) and 3b (SPECT): Low grade focus of uptake identified at the left side of the upper abdomen posteriorly (arrow). SPECT imaging confirms the focus of uptake at the upper pole of the left kidney, in the region of the left adrenal gland (arrow)

Phaeochromocytoma

Image 3a (I123-MIBG) and 3b (SPECT): Low grade focus of uptake identified at the left side of the upper abdomen posteriorly (arrow). SPECT imaging confirms the focus of uptake at the upper pole of the left kidney, in the region of the left adrenal gland (arrow)

Phaeochromocytoma

Image 4 (H&E, x10): Histological section of a phaechromocytoma showing a nested architecture (zellballen pattern) separated by thin fibrous septae. The cells demonstrate finely granular amphophilic cytoplasm.

Phaeochromocytoma

  • Biochemical diagnosis of phaeochromocytoma must be established prior to undertaking imaging studies except in critically ill patients
  • CT of the adrenals is the initial imaging modality of choice in the diagnosis of phaeochromocytoma
  • Functional imaging is a complimentary step that offers higher specificity than anatomical imaging
  • Nuclear Scintigraphy (MIBG) is necessary to exclude additional/metastatic lesions

Date of literature search: July 2017

References are graded from Level I to V according to the Oxford Centre for Evidence-Based Medicine, Levels of Evidence. Download the document

  1. Lenders JWM, Eisenhofer G. Update on Modern Management of Pheochromocytoma and Paraganglioma. Endocrinol Metab (Seoul). 2017;32(2):152-61. (Review article). View the reference
  2. Rufini V, Treglia G, Perotti G, Giordano A. The evolution in the use of MIBG scintigraphy in pheochromocytomas and paragangliomas. Hormones (Athens). 2013;12(1):58-68. (Reviw article). View the reference
  3. Pattou FN, Combemale FP, Poirette JF, Carnaille B, Wemeau JL, Huglo D, et al. Questionability of the benefits of routine laparotomy as the surgical approach for pheochromocytomas and abdominal paragangliomas. Surgery. 1996;120(6):1006-11. (Level III evidence). View the reference
  4. Neumann HP, Bausch B, McWhinney SR, Bender BU, Gimm O, Franke G, et al. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002;346(19):1459-66. (Level II evidence). View the reference
  5. Guller U, Turek J, Eubanks S, Delong ER, Oertli D, Feldman JM. Detecting pheochromocytoma: defining the most sensitive test. Ann Surg. 2006;243(1):102-7. (Level III evidence). View the reference
  6. Bornstein SR, Stratakis CA, Chrousos GP Adrenocortical tumors: recent advances in basic concepts and clinical management. Ann Intern Med. 1999;130(9):759-71. (Review artilce). View the reference
  7. Amar L, Eisenhofer G. Diagnosing phaeochromocytoma/paraganglioma in a patient presenting with critical illness: biochemistry versus imaging. Clinical Endocrinology. 2015;83(3):298-302. (Review article). View the reference
  8. Witteles RM, Kaplan EL, Roizen MF. Sensitivity of diagnostic and localization tests for pheochromocytoma in clinical practice. Arch Intern Med. 2000;160(16):2521-4. (Level IV evidence) View the reference
  9. Lucon AM, Pereira MA, Mendonca BB, Halpern A, Wajchenbeg BL, Arap S. Pheochromocytoma: study of 50 cases J Urol. 1997;157(4):1208-12. (Level III evidence). View the reference
  10. Sawka AM, Prebtani AP, Thabane L, Gafni A, Levine M, Young WF, Jr. A systematic review of the literature examining the diagnostic efficacy of measurement of fractionated plasma free metanephrines in the biochemical diagnosis of pheochromocytoma. BMC Endocr Disord. 2004;4(1):2. (Level II evidence). View the reference
  11. Berglund AS, Hulthen UL, Manhem P, Thorsson O, Wollmer P, Tornquist C. Metaiodobenzylguanidine (MIBG) scintigraphy and computed tomography (CT) in clinical practice. Primary and secondary evaluation for localization of phaeochromocytomas. J Intern Med. 2001;249(3):247-51. (Level II evidence). View the reference
  12. Maurea S, Cuocolo A, Reynolds JC, Tumeh SS, Begley MG, Linehan WM, et al. Iodine-131-metaiodobenzylguanidine scintigraphy in preoperative and postoperative evaluation of paragangliomas: comparison with CT and MRI. J Nucl Med. 1993;34(2):173-9. (Level III evidence). View the reference
  13. Sheps SG, Jiang NS, Klee GG. Diagnostic evaluation of pheochromocytoma. Endocrinol Metab Clin North Am. 1988;17(2):397-414. (Review article). View the reference
  14. van der Harst E, de Herder WW, Bruining HA, Bonjer HJ, de Krijger RR, Lamberts SW, et al. [(123)I]metaiodobenzylguanidine and [(111)In]octreotide uptake in begnign and malignant pheochromocytomas. J Clin Endocrinol Metab. 2001;86(2):685-93. (Level III evidence). View the reference
  15. Lenders JWM, Eisenhofer G. Update on Modern Management of Pheochromocytoma and Paraganglioma. Endocrinology and Metabolism. 2017;32(2):152-61. (Review article). View the reference
  16. Velchik MG, Alavi A, Kressel HY, Engelman K. Localization of pheochromocytoma: MIBG [correction of MIGB], CT, and MRI correlation. J Nucl Med. 1989;30(3):328-36. (Level III evidence). View the reference
  17. Ilias I, Divgi C, Pacak K. Current role of MIBG in the diagnosis of pheochromocytoma and medullary thyroid cancer. Seminars in nuclear medicine. 2011;41(5):364-8. (Review article). View the reference
  18. Hanson MW, Feldman JM, Beam CA, Leight GS, Coleman RE Iodine 131-labeled metaiodobenzylguanidine scintigraphy and biochemical analyses in suspected pheochromocytoma. Arch Intern Med. 1991;151(7):1397-402. (Level II/III evidence). View the reference
  19. Lumachi F, Tregnaghi A, Zucchetta P, Cristina Marzola M, Cecchin D, Grassetto G, et al. Sensitivity and positive predictive value of CT, MRI and 123I-MIBG scintigraphy in localizing pheochromocytomas: a prospective study. Nucl Med Commun. 2006;27(7):583-7. (Level II evidence). View the reference
  20. Hoegerle S, Nitzsche E, Altehoefer C, Ghanem N, Manz T, Brink I, et al. Pheochromocytomas: detection with 18F DOPA whole body PET--initial results. Radiology. 2002;222(2):507-12. (Level IV evidence). View the reference
  21. Hoefnagel CA. Metaiodobenzylguanidine and somatostatin in oncology: role in the management of neural crest tumours. Eur J Nucl Med. 1994;21(6):561-81. (Review article). View the reference
  22. Janssen I, Blanchet EM, Adams K, Chen CC, Millo CM, Herscovitch P, et al. Superiority of [68Ga]-DOTATATE PET/CT to Other Functional Imaging Modalities in the Localization of SDHB-Associated Metastatic Pheochromocytoma and Paraganglioma. Clin Cancer Res. 2015;21(17):3888-95. (Level III evidence). View the reference

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