---
title: "Pheochromocytoma"
docid: "7d3c4062-643c-4030-8783-f85184ad8132"
authors:
- key: "c3463c5c-31d3-4489-bbfe-6b895abdb86d"
value: "Mitchell Tublin, MD"
- key: "b1738976-d5a8-48bc-a435-ed1434cd451a"
value: "Mark D. Sugi, MD"
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name: "Genitourinary"
slug: "genitourinary"
treeNodeId: "bd0eb4fe-d465-4faa-a3b7-526e8f01802d"
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name: "Diagnosis"
slug: "diagnosis"
treeNodeId: "e82a3e55-c0be-4ed1-acd6-b03ae9167c31"
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name: "Adrenal"
slug: "adrenal"
treeNodeId: "d3b85dea-43cb-4be3-b103-902e38d0336e"
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name: "Benign Neoplasms"
slug: "benign-neoplasms"
treeNodeId: "eeebc0ba-f71a-4ae6-8daf-525d0d18fa16"
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name: "Pheochromocytoma"
slug: "pheochromocytoma"
treeNodeId: null
category: "Genitourinary"
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documentVersionId: "f0bbd6b2-6e0d-4040-84b6-3152423baa47"
imageCount: 21
lastUpdated: "10/04/21"
pageDescription: "Pheochromocytoma"
pageKeywords: "Genitourinary, Diagnosis, Adrenal, Benign Neoplasms, Pheochromocytoma"
pageTitle: "Pheochromocytoma | STATdx"
enhancedTitle: "Pheochromocytoma"
type: "DX"
references: true
breadcrumbs:
- "Genitourinary"
- "Diagnosis"
- "Adrenal"
- "Benign Neoplasms"
- "Pheochromocytoma"
---
# KEY FACTS
- ## Terminology
- Tumor arising from chromaffin cells of adrenal medulla or extraadrenal paraganglia
- ## Imaging
- Adrenal medulla (90%)
- Extraadrenal (10%)
- Along sympathetic chain: Anywhere from neck to urinary bladder
- Subdiaphragmatic (98%) or thoracic (1-2%)
- Organ of Zuckerkandl and near urinary bladder are relatively common sites
- Hereditary pheochromocytomas
- Small, bilateral adrenal lesions in younger patient
- Sporadic pheochromocytoma
- Large (> 3 cm), unilateral adrenal mass in older patients
- US: Hypoechoic suprarenal lesion ± cystic change
- Cystic components may be identified
- NECT: > 10 HU
- CECT: Heterogeneous enhancement
- Delayed washout kinetics typically similar to adrenal carcinoma and metastases, but rapid washout (like adenoma) possible
- Heterogeneous enhancement: Necrosis, cystic degeneration, and hemorrhage
- MR
- Variable T1/T2 signal due to hemorrhage, cystic degeneration, and necrosis
- Traditional classic imaging feature: T2 ("light bulb") hyperintensity
- Hypervascular solid components
- Ga-68 DOTATATE: Ectopic, recurrent, and metastatic tumors
- ## Pathology
- Autosomal dominant familial syndromes
- von Hippel-Lindau, multiple endocrine neoplasia 2, neurofibromatosis type 1
- ## Diagnostic Checklist
- Pheochromocytoma is not distinguished from other tumors by imaging appearance alone
- Clinical history and lab values are necessary for diagnosis
- Urinary and plasma fractionated metanephrines and catecholamines
# TERMINOLOGY
- ## Definitions
- Tumor arising from chromaffin cells of adrenal medulla or sympathetic nervous system
- Extraadrenal pheochromocytoma: Paraganglioma, ganglioneuroma
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Adrenal mass with appropriate clinical history and supporting biochemical studies (elevated catecholamines)
- Characteristic T2 hyperintensity described in very early literature, though imaging appearance varies at MR (and other modalities)
- ### Location
- Adrenal medulla (90%) or extraadrenal (10%)
- Extraadrenal, along sympathetic chain: Neck to urinary bladder
- Subdiaphragmatic (98%) or thoracic (1-2%)
- Organ of Zuckerkandl and near bladder are relatively common sites
- ### Size
- Usually larger (> 3 cm) in sporadic (nonsyndromic) or clinically silent cases
- ### Morphology
- Well-circumscribed, encapsulated tumor
- Solitary (sporadic) or multiple (familial)
- Key concepts
- Classic teaching: Rule of 10s (or 10% tumor)
- 10% extraadrenal (paraganglioma)
- 10% bilateral
- 10% malignant
- 10% extraabdominal
- 10% familial
- 10% pediatric
- 10% silent
- 10% autosomal dominant transmission
- Demographics evolving
- ↑ detection of incidental, clinically silent adrenal lesions (including pheochromocytoma) on CT
- Large imaging series: 23-58% of pheochromocytomas clinically occult
- Prompts aggressive biochemical evaluation of incidental adrenal lesion
- Larger percentage of syndromic tumors likely: More aggressive screening for predisposing hereditary conditions
- Multiple endocrine neoplasia 2 (MEN2), von Hippel-Lindau (VHL)
- Neuroectodermal disorders: Neurofibromatosis type 1 (NF1), Sturge-Weber syndrome, Carney triad
- 25% of patients with apparent sporadic pheochromocytomas are carriers of gene mutations
- Extraadrenal tumors arise from sympathetic ganglia
- Neck, mediastinum, pelvis, or urinary bladder
- Aortic bifurcation (organ of Zuckerkandl): Ganglia at origin of inferior mesenteric artery
- Imaging: Difficult to distinguish benign from malignant
- Distant metastases indicate malignancy
- ## CT Findings
- ### NECT
- Attenuation ranges from low density to soft tissue attenuation
- Attenuation almost always > 10 HU and usually ≥ 40 HU, though rare intracellular lipid-containing pheochromocytomas reported
- ± areas of ↑ density (hemorrhage)
- ± areas of ↓ density (cystic degeneration, necrosis)
- ± areas of curvilinear or mural calcification
- ### CECT
- Heterogeneous enhancement due to tissue necrosis, cystic degeneration, and hemorrhage
- Modified criteria: 1 min ≥ 160 HU; 15 min ≥ 70 HU; or intralesional cystic degeneration at both 1 min and 15 min
- Solid components briskly enhance
- Hyperenhancement (> 110 HU) on late arterial phase may suggest pheochromocytoma
- Early, marked enhancement also possible with vascular metastases (hepatocellular carcinoma, renal cell carcinoma)
- Initial literature suggested pheochromocytoma washout characteristics similar to adrenal carcinoma and metastases
- Recent work suggests large percentage of pheochromocytomas with washout percentages similar to adenomas
- Theoretical risk of induction of hyperadrenergic symptoms with iodinated contrast administration discounted by retrospective series utilizing nonionic material
- ## MR Findings
- ### T1WI
- Isointense to muscle and hypointense to liver
- Heterogeneous signal
- Due to areas of hemorrhage and necrosis
- ± areas of ↑ signal
- Due to acute or subacute hemorrhage
- ### T2WI
- T2 heterogeneity typical
- Due to ↑ water content as result of necrosis, cystic degeneration
- Markedly hyperintense ("light bulb") characteristic in early MR series, later discounted
- ↓ T2 signal does not exclude pheochromocytoma
- ### DWI
- Unlike lesion size, ADC values generally not useful for differentiating benign vs. malignant
- ### T1WI C+
- Characteristic salt and pepper pattern (due to ↑ tumor vascularity)
- Salt: Represents enhancing parenchyma
- Pepper: Represents flow void of vessels
- Can show marked early as well as prolonged contrast enhancement
- ## Ultrasonographic Findings
- ### Grayscale ultrasound
- Variable echogenicity: Majority iso- to hypoechoic
- Intralesional hemorrhage may be echogenic
- Round and well-circumscribed mass
- Cystic components may be identified
- Malignant features include large size, irregular shape, poorly defined margins, heterogeneity, and hypervascularity
- 75% of malignant pheochromocytomas are hypoechoic; 20% show mixed echogenicity and cystic necrosis
- ## Angiographic Findings
- Conventional
- Hypervascular tumor
- ## Nuclear Medicine Findings
- I-131 or I-123 MIBG
- Most common and available technique
- MIBG is norepinephrine analogue: Uptake proportional to number of neurosecretory granules within lesion
- I-123 MIBG has largely replaced I-131 MIBG (lower radiation dose, improved image quality)
- I-123 MIBG sensitivity: 77-90%; specificity: 95-100%
- Hybridized MIBG SPECT/CT improves diagnostic accuracy
- Particularly useful for extraadrenal paraganglioma detection, malignant pheochromocytoma staging
- In-111 pentetreotide (somatostatin analog): Potentially useful for dedifferentiated pheochromocytoma
- PET
- Ga-68 DOTATATE: Largely replaced MIBG as primary imaging modality
- Neuroendocrine tumors express somatostatin receptors
- F-18: Can be utilized in imaging of faster-growing pheochromocytomas
- ## Imaging Recommendations
- Helical NE + CECT
- Hypertensive crises not documented with IV administration of nonionic contrast material
- Routine **premedication** (α and β blockade) is**not recommended**
- MR ± contrast
- Ga-68 DOTATATE PET/CT
- For ectopic, recurrent, and metastatic tumors
# DIFFERENTIAL DIAGNOSIS
- [Adrenal Adenoma](/document/adrenal-adenoma/e2916d86-5f9f-4dd3-9576-1a7b89d8dda0)
- NECT: Well-defined mass < 10 HU (lipid rich)
- CECT: Enhancing mass that washes out rapidly
- Early hyperenhancement and marked wash out of pheochromocytomas are reported and may mimic adenomas
- Adenoma with hemorrhage or necrosis may resemble pheochromocytoma
- [Adrenal Carcinoma](/document/adrenal-cortical-carcinoma/bdc7a08b-a64f-4bd2-9dfc-24331728e85e)
- Rare; usually unilateral
- Large, unilateral adrenal mass with invasive margins
- ± calcification (30% of cases); variable enhancement
- Metastatic tumor spread: Lungs, liver, nodes, and bone
- Inferior vena cava tumor thrombus
- [Adrenal Metastases and Lymphoma](/document/adrenal-lymphoma/44639c90-bd04-4e2a-a470-2c28a0e2ff78)
- Adrenal metastases
- e.g., lung, breast, renal cell carcinoma, and melanoma
- Unilateral or bilateral; central necrosis ± hemorrhage
- History of primary malignancy
- Adrenal lymphoma
- Usually as part of diffuse disease
- Rarely limited to adrenals
- Non-Hodgkin most common; usually bilateral
- CECT: Mild enhancement (hypovascular)
- [Adrenal Myelolipoma](/document/adrenal-myelolipoma/5813a554-06a4-4696-af71-7ce50693039d)
- Rare benign tumor (fat + hematopoietic elements)
- Unilateral fatty adrenal tumor (-100 to -30 HU)
- T1WI: Typically hyperintense; size varies (2-10 cm)
- Signal loss on T1WI with fat suppression
- [Adrenal Hemorrhage](/document/adrenal-hemorrhage/5812e5c4-ca8a-4af5-884b-f75795bcde0f)
- Etiology: Septicemia, burns, trauma, stress, hypotension, and hematological abnormalities
- CT findings
- Usually bilateral
- Old hemorrhage: Soft tissue attenuation (20-35 HU)
- Recent hemorrhage: ↑ attenuation values
- MR findings: T1WI and T2WI
- Varied signal depending on hematoma age
- Subacute phase: Usually ↑ signal (methemoglobin)
- Perilesional dark ring (hemosiderin or ferritin)
- [Adrenal Tuberculosis and Fungal Infection](/document/adrenal-tuberculosis-and-fungal-in-/56a3b7b4-f9bc-4f89-87bb-98b80ddee00a)
- e.g., tuberculosis, histoplasmosis, other fungal diseases
- Usually bilateral, heterogeneous, poorly enhancing (acute)
- Chronic: Small and calcified adrenals
- Diagnosis: Clinical history and lab data
# PATHOLOGY
- ## General Features
- ### Etiology
- Chromaffin cells of adrenal medulla or extraadrenal paraganglioma
- Adrenal medulla: Pheochromocytoma
- Extraadrenal: Paraganglioma
- ### Associated abnormalities
- Classic associated syndromes
- VHL syndrome
- Pheochromocytoma may be only manifestation of VHL or occur along with other tumors
- NF1
- MEN syndromes types 2A and 2B
- Tuberous sclerosis; Sturge-Weber syndrome
- Carney triad
- Functional extraadrenal paraganglioma, pulmonary chondroma, gastric leiomyosarcoma
- Adage that only 10% of pheochromocytomas are hereditary discounted with recent advances in molecular studies
- 25% of patients with previously considered sporadic pheochromocytomas are carriers of gene mutations
- Embryology/anatomy
- Neoplasm of chromaffin cells derived from neural crest or neuroectoderm
- ## Gross Pathologic & Surgical Features
- Round, tan-pink to violaceous, encapsulated mass
- ± cystic, mucoid, serosanguineous hemorrhage
- ## Microscopic Features
- Large cells: Granular cytoplasm and pleomorphic nuclei
- Chromaffin reaction: Cells stained + chromium salt
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Symptoms may be episodic or paroxysmal
- Crisis: Headaches, hypertension, palpitations, diaphoresis, tremors, arrhythmias, pain
- Classic triad: Headache, palpitations, diaphoresis
- 90% specific but uncommon presentation (10-36%)
- Atypical: Labile hypertension, myocardial infarction, stroke
- Often clinically silent
- ### Clinical profile
- Young patient with paroxysmal attacks of headache, palpitations, sweating, and tremors
- Lab data
- 24-hour urine-fractionated metanephrine evaluation often initial biochemical test
- Excretion of metanephrine, normetanephrine, and 3-methoxytyramine (dopamine metabolite) measured
- Sensitivity: 90-97%; specificity: 69-98%
- Plasma-fractionated metanephrines also measured, though low positive predictive value and ↓ specificity results in high false-positive rates
- ## Demographics
- ### Age
- Sporadic pheochromocytoma: Older patients (mean: 44 years)
- Hereditary pheochromocytoma: Younger patients (mean: 25 years)
- Pheochromocytomas are exceedingly rare in pediatric patients
- Higher genetic predisposition and malignancy incidence
- ### Sex
- F > M
- ### Epidemiology
- Incidence
- 0.13% in autopsy series; accounts for 0.1-0.5% of patients with hypertension
- Prevalence likely underestimated
- ## Natural History & Prognosis
- Complications: During hypertensive crisis
- Cerebrovascular accidents
- Pregnancy + pheochromocytoma: Mortality (48%)
- Malignancy in 2-14% cases
- Prognosis
- Noninvasive and nonmetastatic: Good prognosis
- Malignant and metastatic: Poor prognosis
- 5-year survival rate: < 50%
- ## Treatment
- Medical therapy: Before, during, and after surgery
- α-adrenergic blockers
- Phenoxybenzamine, phentolamine
- β-adrenergic blocker: Propranolol
- Surgical resection: Benign and malignant
- Laparoscopic resection preferred
- Posterior retroperitoneoscopic adrenalectomy associated with ↓ morbidity compared to transabdominal laparoscopic approach
- Partial adrenalectomy may be performed with bilateral pheochromocytomas
- Cortical-sparing adrenalectomy in patients with hereditary pheochromocytoma shows survival similar to total adrenalectomy but recurrence in 13%
- HSA I-131 MIBG and tumor debulking for metastatic, unresectable, or locally advanced malignant pheochromocytomas
- Combination chemotherapy: Cyclophosphamide + vincristine + dacarbazine
# DIAGNOSTIC CHECKLIST
- ## Consider
- Clinical history, supporting biochemical data ultimately drive diagnosis
- ## Reporting Tips
- CT and MR features (vascularity, cystic change, T2 heterogeneity) may suggest pheochromocytoma, but overlap precludes definitive diagnosis solely by imaging
- Possibility of pheochromocytoma should be raised, but lab analysis confirms or excludes diagnosis
483df3a0-a77f-4ff8-9024-1fa2032a515a
## References
# Selected References
1. [Jain A et al: Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management. Pediatr Nephrol. 35(4):581-94, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=30603807%5Bpmid%5D)
1. [Kang S et al: Distinguishing pheochromocytoma from adrenal adenoma by using modified computed tomography criteria. Abdom Radiol (NY). 46(3):1082-90, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32951125%5Bpmid%5D)
1. [Alshahrani MA et al: Bilateral adrenal abnormalities: imaging review of different entities. Abdom Radiol (NY). 44(1):154-79, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=29938331%5Bpmid%5D)
1. [Canu L et al: CT characteristics of pheochromocytoma: relevance for the evaluation of adrenal incidentaloma. J Clin Endocrinol Metab. 104(2):312-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30383267%5Bpmid%5D)
1. [Gong X et al: Ultrasonographic findings of 1385 adrenal masses: a retrospective study of 1319 benign and 66 malignant masses. J Ultrasound Med. 38(9):2249-57, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=29194699%5Bpmid%5D)
1. [Goroshi M et al: Radiological differentiation of phaeochromocytoma from other malignant adrenal masses: importance of wash-in characteristics on multiphase CECT. Endocr Connect. 8(7):898-905, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31252396%5Bpmid%5D)
1. [Neumann HPH et al: Comparison of pheochromocytoma-specific morbidity and mortality among adults with bilateral pheochromocytomas undergoing total adrenalectomy vs cortical-sparing adrenalectomy. JAMA Netw Open. 2(8):e198898, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31397861%5Bpmid%5D)
1. [Pryma DA et al: Efficacy and safety of high-specific-activity 131I-MIBG therapy in patients with advanced pheochromocytoma or paraganglioma. J Nucl Med. 60(5):623-30, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30291194%5Bpmid%5D)
1. [Foti G et al: Characterization of adrenal lesions using MDCT wash-out parameters: diagnostic accuracy of several combinations of intermediate and delayed phases. Radiol Med. 123(11):833-40, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29923085%5Bpmid%5D)
1. [Mohammed MF et al: Pheochromocytomas versus adenoma: role of venous phase CT enhancement. AJR Am J Roentgenol. 210(5):1073-8, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29570377%5Bpmid%5D)
1. [Woo S et al: Pheochromocytoma as a frequent false-positive in adrenal washout CT: a systematic review and meta-analysis. Eur Radiol. 28(3):1027-36, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29026974%5Bpmid%5D)
1. [Kim DW et al: Assessment of clinical and radiologic differences between small and large adrenal pheochromocytomas. Clin Imaging. 43:153-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28324715%5Bpmid%5D)
1. [Mendiratta-Lala M et al: Adrenal imaging. Endocrinol Metab Clin North Am. 46(3):741-59, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28760236%5Bpmid%5D)
1. [Schieda N et al: Update on CT and MRI of adrenal nodules. AJR Am J Roentgenol. 1-12, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28225653%5Bpmid%5D)
1. [Zhang GM et al: Differentiating pheochromocytoma from lipid-poor adrenocortical adenoma by CT texture analysis: feasibility study. Abdom Radiol (NY). 42(9):2305-13, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28357529%5Bpmid%5D)
1. [Northcutt BG et al: Adrenal adenoma and pheochromocytoma: comparison of multidetector CT venous enhancement levels and washout characteristics. J Comput Assist Tomogr. 40(2):194-200, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26978001%5Bpmid%5D)
1. [Schieda N et al: Comparison of quantitative MRI and CT washout analysis for differentiation of adrenal pheochromocytoma from adrenal adenoma. AJR Am J Roentgenol. 206(6):1141-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27011100%5Bpmid%5D)
1. [Borhani AA et al: Quantitative versus qualitative methods in evaluation of T2 signal intensity to improve accuracy in diagnosis of pheochromocytoma. AJR Am J Roentgenol. 205(2):302-10, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26204279%5Bpmid%5D)
1. [Derlin T 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. 38(1):e1-6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=22996238%5Bpmid%5D)
1. [Leung K et al: Pheochromocytoma: the range of appearances on ultrasound, CT, MRI, and functional imaging. AJR Am J Roentgenol. 200(2):370-8, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23345359%5Bpmid%5D)
1. [Northcutt BG et al: MDCT of adrenal masses: can dual-phase enhancement patterns be used to differentiate adenoma and pheochromocytoma? AJR Am J Roentgenol. 201(4):834-9, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24059372%5Bpmid%5D)
1. [Patel J et al: Can established CT attenuation and washout criteria for adrenal adenoma accurately exclude pheochromocytoma? AJR Am J Roentgenol. 201(1):122-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23789665%5Bpmid%5D)
1. [Raja A et al: Multimodality imaging findings of pheochromocytoma with associated clinical and biochemical features in 53 patients with histologically confirmed tumors. AJR Am J Roentgenol. 201(4):825-33, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24059371%5Bpmid%5D)
1. [Dong Y et al: Differentiation of malignant from benign pheochromocytomas with diffusion-weighted and dynamic contrast-enhanced magnetic resonance at 3.0 T. J Comput Assist Tomogr. 36(4):361-6, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22805661%5Bpmid%5D)
1. [Timmers HJ et al: Current and future anatomical and functional imaging approaches to pheochromocytoma and paraganglioma. Horm Metab Res. 44(5):367-72, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22399235%5Bpmid%5D)
1. [Miller FH et al: Utility of diffusion-weighted MRI in characterization of adrenal lesions. AJR Am J Roentgenol. 194(2):W179-85, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20093571%5Bpmid%5D)
1. [Blake MA et al: Pheochromocytoma: an imaging chameleon. Radiographics. 24 Suppl 1:S87-99, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15486252%5Bpmid%5D)
## Images
### Selected Images
, surgically proven to be a pheochromocytoma. ~ 1-6% of patients with NF1 develop pheochromocytoma. Note IVC filter 
.](images/app.statdx.com_image_thumbnail_01941c57-74a7-49dc-b4de-8bab12f661f3_annotated_true_size_900_quality_90_6698453e6de25ca49e5b282982ca7fb293891624.jpg)
*Coronal CECT in a 74-year-old man with neurofibromatosis type 1 (NF1) shows a 10-cm, cystic and solid right adrenal mass , surgically proven to be a pheochromocytoma. ~ 1-6% of patients with NF1 develop pheochromocytoma. Note IVC filter .*
*Coronal CECT in a 74-year-old man with neurofibromatosis type 1 (NF1) shows a 10-cm, cystic and solid right adrenal mass , surgically proven to be a pheochromocytoma. ~ 1-6% of patients with NF1 develop pheochromocytoma. Note IVC filter .*
.](images/app.statdx.com_image_thumbnail_db15012b-f8e8-4298-abd8-af04a701fbec_annotated_true_size_900_quality_90_59ce28b051ddeeb3a1d3fb9b474564110e43e09e.jpg)
*Axial T2 FS MR in an 80-year-old man with sporadic pheochromocytoma shows a rounded right adrenal mass with heterogeneous signal. Sporadic pheochromocytoma occurs more often in older patients. Note incidental hepatic cyst .*
*Axial T2 FS MR in a 40-year-old woman with headaches, palpitations, and chronic diaphoresis shows an intermediate-signal, 5-cm left adrenal mass with internal areas of hyperintensity suggesting cystic degeneration or necrosis.*
*Axial T1 C+ FS MR in the same patient shows heterogeneous enhancement of the left adrenal mass with cystic degeneration or necrosis . Surgical pathology showed pheochromocytoma, which was sporadic in this patient with no family history.*
in a 51-year-old woman with abdominal pain, headaches, and markedly elevated 24-hour urine metanephrines. The mass was surgically proven to be pheochromocytoma.](images/app.statdx.com_image_thumbnail_e68ce282-b820-464f-bf8f-bc69c6c726df_annotated_true_size_900_quality_90_84cb7ae20bf7e4d64bcda1f7494537a110df6911.jpg)
*Coronal CECT shows a heterogeneous right adrenal mass in a 51-year-old woman with abdominal pain, headaches, and markedly elevated 24-hour urine metanephrines. The mass was surgically proven to be pheochromocytoma.*
following motor vehicle collision. The mass was resected via right posterior retroperitoneoscopic adrenalectomy and pheochromocytoma was confirmed.](images/app.statdx.com_image_thumbnail_93636528-3a50-424d-ba2a-64e438f43df2_annotated_true_size_900_quality_90_7c65c9ff0137c19c6e8d89d4205682c7821fd50d.jpg)
*Axial CECT in a 44-year-old man with hypertension shows an incidental right adrenal mass following motor vehicle collision. The mass was resected via right posterior retroperitoneoscopic adrenalectomy and pheochromocytoma was confirmed.*
*Axial T2 FS MR in a 66-year-old woman with ↑ 24-hour metanephrines shows a heterogeneous mass in the hepatorenal space . Heterogeneous T2 signal is often seen due to varying degrees of hemorrhage and necrosis.*
*Axial T1 FS C+ MR in the same patient shows heterogeneous enhancement of the surgically proven pheochromocytoma . The patient was treated with alpha-blockade (phenoxybenzamine) for 1 month prior to intervention.*
.](images/app.statdx.com_image_thumbnail_6d6d1a54-7ac7-482b-9dad-618ec5aabd10_annotated_true_size_900_quality_90_4c124aaf6d05e412672fef67f62d8ab8b6ebb1a9.jpg)
*Axial CECT in a 79-year-old man with locally recurrent pheochromocytoma shows an irregular, enhancing mass in the left adrenal fossa abutting the anterior left kidney . Note incidental chronic aortic dissection .*
*Axial Ga-68 DOTATATE PET/CT in the same patient shows avid tracer uptake in the left adrenal fossa , corresponding to the enhancing mass on CT and consistent with locally recurrent pheochromocytoma.*
*Axial CECT shows bilateral, centrally necrotic adrenal masses in a 10 year old with von Hippel-Lindau (VHL) syndrome and pheochromocytomas. Cortical-sparing adrenalectomy was performed on the left mass. Up to 30% of patients with VHL develop pheochromocytoma.*
*Axial Cu-61 DOTATATE PET/CT in the same patient shows minimal peripheral uptake by the bilateral adrenal masses due to extensive necrosis. Note physiologic uptake in the left kidney .*
*Axial T2 FS MR in a 21-year-old man with neurofibromatosis type 1 (NF1) shows a heterogeneous right adrenal mass proven to be pheochromocytoma. While classically described as "light bulb bright," the T2 signal of this neoplasm is highly variable.*
*Axial CECT shows a heterogeneously enhancing right adrenal mass in a 55-year-old man with elevated 24-hour urine metanephrine and pheochromocytoma shown at surgical pathology.*
*Transverse US of the RUQ for pleuritic chest pain in a 20-year-old man shows a round mass posterior to the right hepatic lobe with centrally decreased echogenicity suggesting necrosis.*
*Axial CECT in the same patient shows a centrally necrotic right adrenal mass , surgically proven pheochromocytoma. Multiple pancreatic cysts are also noted, and the diagnosis of familial VHL syndrome was subsequently confirmed.*
### Additional Images
*Axial I-123 MIBG SPECT/CT in a 79-year-old man with locally recurrent pheochromocytoma shows avid tracer uptake in the left adrenal fossa extending to the perisplenic space , consistent with recurrent pheochromocytoma.*
*Axial CECT in a 36-year-old woman shows a 7-cm, heterogeneous left adrenal mass . The differential includes adrenal carcinoma, pheochromocytoma, and metastasis. Urinary metanephrines were ↑, and laparoscopic resection (after α and β blockade) confirmed hemorrhagic pheochromocytoma.*
*Axial T2 MR in the same patient shows a hyperintense ("light bulb") left adrenal lesion . Although this appearance was historically thought to be characteristic of pheochromocytoma, it is neither sensitive nor specific. Elevated 24-hour urine metanephrines confirmed pheochromocytoma in this case.*
*Axial T1 C+ MR in a 52-year-old woman to evaluate an incidental adrenal lesion previously identified on CT shows a 2-cm, vascular left adrenal mass .*
*Axial T2 MR in the same patient shows a slightly intense right adrenal lesion and adjacent renal/hepatic cysts . Elevated 24-hour urinary metanephrines indicated (sporadic) unilateral pheochromocytoma, although the imaging appearance is nonspecific.*