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: January 2012
Date of next review: 2017/2018
Published: January 2012
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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
- CT of the adrenals is the initial imaging modality of choice in the diagnosis of phaeochromocytoma
- Nuclear Scintigraphy (MIBG) is necessary to exclude additional/metastatic lesions
biochem
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 nonfunctioning adrenal nodules ("incidentalomas") 4
- Total urinary levels of catecholamines and their metabolites over 24 hours have high sensitivity for diagnosing phaeochromocytoma 5,6
- 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%) 14
ct
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 1,7
- High sensitivity (>90%) for localisation of abdominal and thoracic phaeochromocytoma 7,8,9
- Limitations 7
- 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 1
- 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
mibg
Meta-Iodobenzylguanidine (MIBG) Scintigraphy
- High sensitivity (85%) and specificity (>95%) for detection and localisation of phaeochromocytomas 7-12
- 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
- Indications 7,8,11,13
- 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 1
- 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 MIBI has been reported as having a sensitivity and specificity of 100% 17
other
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 7-10
- The sensitivity of MRI in detecting phaeochromocytoma and extra-adrenal phaeochromocytoma is 93.3-100% 17
- The combination of MRI and MIBI has been reported as having a sensitivity and specificity of 100% in the detection of biochemically proven phaeochromocytoma 17
- Added advantage of lacking ionising radiation
- The potential risk of contrast is avoided
- Imaging of choice in pregnant women and children
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% 15
- Advantages 15
- 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)
- 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-Pheoctreotide is dependant on the expression of somatostatin receptors by the tumour
- Sensitivity of 88% makes this test comparable to MIBG 16
phaeo
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
- 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 2
- Emerging evidence that a higher proportion of phaeochromocytoma's than previously thought, are part of a hereditary syndomes 3
- Multiple endocrine neoplasia 2
- Von Hippel-Lindau disease
- Paraganglioma
References
References
References are graded from Level I to V according to the Oxford Centre for Evidence-Based Medicine, Levels of Evidence. Download the document
- Sheps SG, Jiang NS, Klee GG. Diagnostic evaluation of phaeochromocytoma. Endocrinol Metab Clin North Am. 1998;17:397-414. (Review article)
- Pattou F, Combermale F, Poirette JF, et al. Questionability of the benefits of routine laparotomy as the surgical approach for phaeochromocytomas and abdominal paragangliomas. Surgery. 1996;120:1006-12. (Level III evidence)
- Neumann H, Bausch B, McWhinney S, et al. Germ-line mutations in non-syndromic pheochromocytoma. N Engl J Med. 2002;346(19):1459-66. (Level II evidence). View the reference
- Bornstein SR, Stratakis CA, Chrousos GP. Adrenocortical tumors: recent advances in basic concepts and clinical management. Ann Intern Med. 1999;130:759-71. (Review article)
- Wittles RM, Kaplan EL, Roizen MF. Sensitivity of diagnostic and localization tests for phaeochromocytoma in clinical practice. Arch Intern Med. 2000;160:2521-4. (Level IV evidence)
- Lucon AM, Pereira MA, Mendonca BB, et al. Phaeochromocytoma: study of 50 cases. J Urol. 1997;157:1208-12. (Level III evidence)
- Berglund AS, Hulthen UL, Manhem P, et al. Metaiodobenzylguanidine (MIBG) scintigraphy and computed tomography in clinical practice: primary and secondary evaluation for localization of phaeochromocytomas. J Int Med. 2001;249(3):247-51. (Level II evidence). View the reference
- Maurea S, Cuocolo A, Reynolds JC, et al. Iodine-131-metaiodobenzylguanidine scintigraphy in preoperative evaluation of paragangliomas: comparison with CT and MRI. J Nucl Med. 1993;34:173-9. (Level III evidence)
- Jalil ND, Pattou FN, Combemale F, et al. Effectiveness and limitations of pre-operative imaging studies for localisation of phaeochromocytomas: a review of 282 cases. Eur J Surg. 1998;164:23-8. (Level III evidence)
- Velchik M, Alavi A, Kressel H, et al. Localization of phaeochromocytoma: MIBG, CT, and MRI correlation. J Nucl Med. 1989;30:328-36. (Level III evidence)
- Shapiro B, Copp JE, Sisson JC, et al. Iodine-131 metaiodobenzylguanidine for the locating of suspected pheochromocytoma: experience in 400 cases. J Nucl Med. 1985;26:576-85. (Level II/III evidence)
- Van der Harst E, de Herder WW, Bruining HA, et al. 123I metaiodobenzylguanidine and 111-In octreotide uptake in benign and malignant pheochromocytomas. J Clin Endocrinol Metab. 2001;86:685-93.(Level III evidence)
- Hanson MW, Feldman JM, Beam CA, et al. Iodine 131-labeled metaiodobenzlguanidine scintigraphy and biochemical analyses in suspected phaeochromocytoma. Arch Intern Med. 1991;151:1397-1402. (Level II/III evidence)
- Sawka A, Prebtani A, Thabane L et al. A Stematic review of the literature examining the diagnostic efficacy of measurement of fractionated plasma free metanephrines in the biochemical diagnosis of phaeochromocytoma. BMC Endocr Disord. 2004;4:2 (Level II evidence). View the reference
- Hoegerle S, Nitzxche E, Altehoefer C, et al. Pheochromocytomas: detection with 18F-DOPA whole-body PET - initial results. Radiology. 2002;222:507-12. (Level IV evidence)
- Hoefnagel C Metaiodobenzylguanidine and somatostatin in oncology: role in the management of neural crest tumours. Eur J Nuc Med. 1994;21:561-81. (Review article)
- Lumachi F, Tregnaghi A, Zucchetta P. Sensitivity and positive predictive value of CT, MRI and 123I-MIBG scintigraphy in localising pheochromocytomas: a prospective study. Nuclear Medicine Communications. 2006;27(7):583-7. (Level II evidence). View the reference
Further Reading
- Walther MM, Keiser HR, Linehan WM. Pheochromocytoma: evaluation, diagnosis, and treatment. World J Urol. 1999;17:35-9. (Review article)
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