Diagnostic Imaging Pathways - Phaeochromocytoma
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This pathway provides guidance on the imaging of adult patients with a confirmed biochemical diagnosis of primary phaeochromocytoma.
Date reviewed: November 2017
Date of next review: November 2020
Published: April 2018
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The relative radiation level (RRL) of each imaging investigation is displayed in the pop up box.
| SYMBOL | RRL | EFFECTIVE DOSE RANGE |
 | None | 0 |
 | Minimal | < 1 millisieverts |
 | Low | 1-5 mSv |
 | Medium | 5-10 mSv |
 | High | >10 mSv |
Images
Image GalleryNote: These images open in a new page | ||
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| 1 |  |
Phaeochromocytoma Image 1 (Computed Tomography): Round low attenuation lesion at upper pole of left kidney consistent with phaeochromocytoma (arrow). |
| 2a |  |
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). |
| 2b |  |
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| 3a |  |
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). |
| 3b |  |
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| 4 |  |
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. |
Teaching Points
Teaching Points
- 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
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Imaging in Phaeochromocytoma
- Imaging is used for localisation of phaeochromocytoma in patients with proven biochemical abnormality consistent with phaeochromocytoma and is not for diagnosis 1, 2
- Localisation of the tumour helps in surgical planning and reduces the chance of negative findings at laparotomy if the tumour happens to be located outside the abdomen 3
- Emerging evidence that a higher proportion of phaeochromocytomas than previously thought, are part of a hereditary syndromes 4
- Multiple endocrine neoplasia 3
- Von Hippel-Lindau disease
- Paraganglioma
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Biochemical Tests
- Biochemical diagnosis of phaeochromocytoma must be established prior to undertaking imaging studies to avoid unnecessary surgery because 3% to 7% of patients over the age of 50 have non-functioning adrenal nodules ("incidentalomas") 5, 6
- Imaging may take priority over biochemical testing in emergency situations with critically unwell patients 1, 7
- Total urinary levels of catecholamines and their metabolites over 24 hours have high sensitivity for diagnosing phaeochromocytoma 8, 9
- A more recent systematic review advocated the use of fractionated plasma free metanephrines in the biochemical diagnosis of phaeochromocytoma. For all patients in the review, the sensitivity of the test ranged from 96-100% (95% CI 82%-100%) whereas the specificity ranged from 85-100% (95 CI 78%-100%) 10
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Computed Tomography (CT)
- Having confirmed the diagnosis of phaeochromocytoma biochemically, it is important to localise the tumour and extent pre-operatively
- Initial imaging modality of choice for localisation of phaeochromocytomas 1, 2, 11
- High sensitivity (>90%) for localisation of abdominal and thoracic phaeochromocytoma 1, 11, 12
- Limitations 11
- Low (~50%) specificity as adrenal "incidentalomas" are common
- Not suitable for whole-body screening
- Can be difficult to interpret when there are anatomical changes or metal clips from surgery
- Cautions 13
- Intravenous iodinated contrast media have been reported to precipitate a hypertensive crisis in patients who are not pre-blocked with anti-adrenergic agents. While this is anecdotal, if a phaeochromocytoma is suspected, it is wise to block the patient before administration of contrast agents
- CT-guided percutaneous adrenal biopsy on unsuspected phaeochromocytoma has been associated with cardiovascular crises
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Meta-Iodobenzylguanidine (MIBG) Scintigraphy
- High sensitivity (85%) and specificity (>95%) for detection and localisation of phaeochromocytomas 11, 12, 14-16
- Either 123I- MIBG or 131I-MIBG can be used for imaging. However, 123I-MIBG is preferred because of better image quality, greater sensitivity and reduced radiation exposure due to higher photon flow and shorter half-life time 15, 17
- Indications 1, 5, 11, 12, 14, 18
- To detect extra-adrenal, metastatic, or recurrent sites of disease or when whole-body imaging is important
- When CT/MRI fails to detect the tumour
- To confirm the biochemical activity of CT/MRI detected masses and to identify or exclude extra-adrenal phaeochromocytomas
- When biochemical tests are inconclusive (MIBG can confirm or exclude the diagnosis)
- Limitations of MIBG scan 2
- Not all phaeochromocytomas concentrate MIBG
- Drugs such as labetalol, reserpine, calcium channel blockers and some tricyclic antidepressants are known to interfere with the uptake of MIBG
- The combination of MRI and MIBG has been reported as having a sensitivity and specificity of 100% 1, 19
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Other Imaging Modalities
Magnetic Resonance Imaging (MRI)
- MRI is useful for detecting extra-adrenal phaeochromocytomas and recurrence after resection, given increased signal intensity on T2- weighted images 1, 11, 12, 16
- The sensitivity of MRI in detecting phaeochromocytoma and extra-adrenal phaeochromocytoma is 93.3-100% 15, 19
- The combination of MRI and MIBG has been reported as having a sensitivity and specificity of 100% in the detection of biochemically proven phaeochromocytoma 19
- Imaging of choice in pregnant women and children 1
- Advantages: lack of exposure to ionising radiation and avoids the potential risk of contrast 1
Positron Emission Tomography (PET)
- 18F-DOPA-PET with fluorine-18-labelled dihydroxyphenylalanine has emerged as an important tool in the diagnosis and staging of phaeochromocytomas and extra-adrenal paraganglioma
- Early small case series have demonstrated sensitivity and specificity of 100% 20
- Advantages 1, 20
- Higher spatial resolution than conventional nuclear scintigraphy modalities (e.g. MIBG)
- Lack of uptake in normal adrenal glands (in contrast to MIBG where some uptake is seen in normal adrenal glands)
- Particularly recommended for both skull base and neck paragangliomas
- Due to rapid acquisition of functional data, feasibly could be combined with anatomical investigations at one sitting
Radio-labelled Somatostatin Analogue
- 111In-labelled DTPA-D-Phe-octreotide is dependent on the expression of somatostatin receptors by the tumour
- Sensitivity of 88% makes this test comparable to MIBG 21
- Newer modalities using somatostatin analogues such as 68Ga-DOTATATE has shown promise and will likely replace 123I-MIBG in the near future 1, 15, 22
References
References
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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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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