510 lines
37 KiB
Markdown
510 lines
37 KiB
Markdown
---
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title: "Pheochromocytoma"
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docid: "7d3c4062-643c-4030-8783-f85184ad8132"
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authors:
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- key: "c3463c5c-31d3-4489-bbfe-6b895abdb86d"
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value: "Mitchell Tublin, MD"
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- key: "b1738976-d5a8-48bc-a435-ed1434cd451a"
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value: "Mark D. Sugi, MD"
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breadcrumbs:
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-
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name: "Genitourinary"
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slug: "genitourinary"
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treeNodeId: "bd0eb4fe-d465-4faa-a3b7-526e8f01802d"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "e82a3e55-c0be-4ed1-acd6-b03ae9167c31"
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-
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name: "Adrenal"
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slug: "adrenal"
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treeNodeId: "d3b85dea-43cb-4be3-b103-902e38d0336e"
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-
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name: "Benign Neoplasms"
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slug: "benign-neoplasms"
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treeNodeId: "eeebc0ba-f71a-4ae6-8daf-525d0d18fa16"
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-
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name: "Pheochromocytoma"
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slug: "pheochromocytoma"
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treeNodeId: null
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category: "Genitourinary"
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cmeTopicId: "138504cb-d068-4dea-b92b-476704f8c54e"
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documentVersionId: "f0bbd6b2-6e0d-4040-84b6-3152423baa47"
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imageCount: 21
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lastUpdated: "10/04/21"
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pageDescription: "Pheochromocytoma"
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pageKeywords: "Genitourinary, Diagnosis, Adrenal, Benign Neoplasms, Pheochromocytoma"
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pageTitle: "Pheochromocytoma | STATdx"
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enhancedTitle: "Pheochromocytoma"
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type: "DX"
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references: true
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breadcrumbs:
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- "Genitourinary"
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- "Diagnosis"
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- "Adrenal"
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- "Benign Neoplasms"
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- "Pheochromocytoma"
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---
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# KEY FACTS
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- ## Terminology
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- Tumor arising from chromaffin cells of adrenal medulla or extraadrenal paraganglia
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- ## Imaging
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- Adrenal medulla (90%)
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- Extraadrenal (10%)
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- Along sympathetic chain: Anywhere from neck to urinary bladder
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- Subdiaphragmatic (98%) or thoracic (1-2%)
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- Organ of Zuckerkandl and near urinary bladder are relatively common sites
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- Hereditary pheochromocytomas
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- Small, bilateral adrenal lesions in younger patient
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- Sporadic pheochromocytoma
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- Large (> 3 cm), unilateral adrenal mass in older patients
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- US: Hypoechoic suprarenal lesion ± cystic change
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- Cystic components may be identified
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- NECT: > 10 HU
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- CECT: Heterogeneous enhancement
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- Delayed washout kinetics typically similar to adrenal carcinoma and metastases, but rapid washout (like adenoma) possible
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- Heterogeneous enhancement: Necrosis, cystic degeneration, and hemorrhage
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- MR
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- Variable T1/T2 signal due to hemorrhage, cystic degeneration, and necrosis
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- Traditional classic imaging feature: T2 ("light bulb") hyperintensity
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- Hypervascular solid components
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- Ga-68 DOTATATE: Ectopic, recurrent, and metastatic tumors
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- ## Pathology
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- Autosomal dominant familial syndromes
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- von Hippel-Lindau, multiple endocrine neoplasia 2, neurofibromatosis type 1
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- ## Diagnostic Checklist
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- Pheochromocytoma is not distinguished from other tumors by imaging appearance alone
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- Clinical history and lab values are necessary for diagnosis
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- Urinary and plasma fractionated metanephrines and catecholamines
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# TERMINOLOGY
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- ## Definitions
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- Tumor arising from chromaffin cells of adrenal medulla or sympathetic nervous system
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- Extraadrenal pheochromocytoma: Paraganglioma, ganglioneuroma
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Adrenal mass with appropriate clinical history and supporting biochemical studies (elevated catecholamines)
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- Characteristic T2 hyperintensity described in very early literature, though imaging appearance varies at MR (and other modalities)
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- ### Location
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- Adrenal medulla (90%) or extraadrenal (10%)
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- Extraadrenal, along sympathetic chain: Neck to urinary bladder
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- Subdiaphragmatic (98%) or thoracic (1-2%)
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- Organ of Zuckerkandl and near bladder are relatively common sites
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- ### Size
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- Usually larger (> 3 cm) in sporadic (nonsyndromic) or clinically silent cases
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- ### Morphology
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- Well-circumscribed, encapsulated tumor
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- Solitary (sporadic) or multiple (familial)
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- Key concepts
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- Classic teaching: Rule of 10s (or 10% tumor)
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- 10% extraadrenal (paraganglioma)
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- 10% bilateral
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- 10% malignant
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- 10% extraabdominal
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- 10% familial
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- 10% pediatric
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- 10% silent
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- 10% autosomal dominant transmission
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- Demographics evolving
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- ↑ detection of incidental, clinically silent adrenal lesions (including pheochromocytoma) on CT
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- Large imaging series: 23-58% of pheochromocytomas clinically occult
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- Prompts aggressive biochemical evaluation of incidental adrenal lesion
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- Larger percentage of syndromic tumors likely: More aggressive screening for predisposing hereditary conditions
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- Multiple endocrine neoplasia 2 (MEN2), von Hippel-Lindau (VHL)
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- Neuroectodermal disorders: Neurofibromatosis type 1 (NF1), Sturge-Weber syndrome, Carney triad
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- 25% of patients with apparent sporadic pheochromocytomas are carriers of gene mutations
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- Extraadrenal tumors arise from sympathetic ganglia
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- Neck, mediastinum, pelvis, or urinary bladder
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- Aortic bifurcation (organ of Zuckerkandl): Ganglia at origin of inferior mesenteric artery
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- Imaging: Difficult to distinguish benign from malignant
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- Distant metastases indicate malignancy
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- ## CT Findings
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- ### NECT
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- Attenuation ranges from low density to soft tissue attenuation
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- Attenuation almost always > 10 HU and usually ≥ 40 HU, though rare intracellular lipid-containing pheochromocytomas reported
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- ± areas of ↑ density (hemorrhage)
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- ± areas of ↓ density (cystic degeneration, necrosis)
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- ± areas of curvilinear or mural calcification
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- ### CECT
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- Heterogeneous enhancement due to tissue necrosis, cystic degeneration, and hemorrhage
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- Modified criteria: 1 min ≥ 160 HU; 15 min ≥ 70 HU; or intralesional cystic degeneration at both 1 min and 15 min
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- Solid components briskly enhance
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- Hyperenhancement (> 110 HU) on late arterial phase may suggest pheochromocytoma
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- Early, marked enhancement also possible with vascular metastases (hepatocellular carcinoma, renal cell carcinoma)
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- Initial literature suggested pheochromocytoma washout characteristics similar to adrenal carcinoma and metastases
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- Recent work suggests large percentage of pheochromocytomas with washout percentages similar to adenomas
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- Theoretical risk of induction of hyperadrenergic symptoms with iodinated contrast administration discounted by retrospective series utilizing nonionic material
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- ## MR Findings
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- ### T1WI
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- Isointense to muscle and hypointense to liver
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- Heterogeneous signal
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- Due to areas of hemorrhage and necrosis
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- ± areas of ↑ signal
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- Due to acute or subacute hemorrhage
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- ### T2WI
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- T2 heterogeneity typical
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- Due to ↑ water content as result of necrosis, cystic degeneration
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- Markedly hyperintense ("light bulb") characteristic in early MR series, later discounted
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- ↓ T2 signal does not exclude pheochromocytoma
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- ### DWI
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- Unlike lesion size, ADC values generally not useful for differentiating benign vs. malignant
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- ### T1WI C+
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- Characteristic salt and pepper pattern (due to ↑ tumor vascularity)
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- Salt: Represents enhancing parenchyma
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- Pepper: Represents flow void of vessels
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- Can show marked early as well as prolonged contrast enhancement
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- ## Ultrasonographic Findings
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- ### Grayscale ultrasound
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- Variable echogenicity: Majority iso- to hypoechoic
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- Intralesional hemorrhage may be echogenic
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- Round and well-circumscribed mass
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- Cystic components may be identified
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- Malignant features include large size, irregular shape, poorly defined margins, heterogeneity, and hypervascularity
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- 75% of malignant pheochromocytomas are hypoechoic; 20% show mixed echogenicity and cystic necrosis
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- ## Angiographic Findings
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- Conventional
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- Hypervascular tumor
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- ## Nuclear Medicine Findings
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- I-131 or I-123 MIBG
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- Most common and available technique
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- MIBG is norepinephrine analogue: Uptake proportional to number of neurosecretory granules within lesion
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- I-123 MIBG has largely replaced I-131 MIBG (lower radiation dose, improved image quality)
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- I-123 MIBG sensitivity: 77-90%; specificity: 95-100%
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- Hybridized MIBG SPECT/CT improves diagnostic accuracy
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- Particularly useful for extraadrenal paraganglioma detection, malignant pheochromocytoma staging
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- In-111 pentetreotide (somatostatin analog): Potentially useful for dedifferentiated pheochromocytoma
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- PET
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- Ga-68 DOTATATE: Largely replaced MIBG as primary imaging modality
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- Neuroendocrine tumors express somatostatin receptors
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- F-18: Can be utilized in imaging of faster-growing pheochromocytomas
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- ## Imaging Recommendations
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- Helical NE + CECT
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- Hypertensive crises not documented with IV administration of nonionic contrast material
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- Routine **premedication** (α and β blockade) is**not recommended**
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- MR ± contrast
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- Ga-68 DOTATATE PET/CT
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- For ectopic, recurrent, and metastatic tumors
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# DIFFERENTIAL DIAGNOSIS
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- [Adrenal Adenoma](/document/adrenal-adenoma/e2916d86-5f9f-4dd3-9576-1a7b89d8dda0)
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- NECT: Well-defined mass < 10 HU (lipid rich)
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- CECT: Enhancing mass that washes out rapidly
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- Early hyperenhancement and marked wash out of pheochromocytomas are reported and may mimic adenomas
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- Adenoma with hemorrhage or necrosis may resemble pheochromocytoma
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- [Adrenal Carcinoma](/document/adrenal-cortical-carcinoma/bdc7a08b-a64f-4bd2-9dfc-24331728e85e)
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- Rare; usually unilateral
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- Large, unilateral adrenal mass with invasive margins
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- ± calcification (30% of cases); variable enhancement
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- Metastatic tumor spread: Lungs, liver, nodes, and bone
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- Inferior vena cava tumor thrombus
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- [Adrenal Metastases and Lymphoma](/document/adrenal-lymphoma/44639c90-bd04-4e2a-a470-2c28a0e2ff78)
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- Adrenal metastases
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- e.g., lung, breast, renal cell carcinoma, and melanoma
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- Unilateral or bilateral; central necrosis ± hemorrhage
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- History of primary malignancy
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- Adrenal lymphoma
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- Usually as part of diffuse disease
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- Rarely limited to adrenals
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- Non-Hodgkin most common; usually bilateral
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- CECT: Mild enhancement (hypovascular)
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- [Adrenal Myelolipoma](/document/adrenal-myelolipoma/5813a554-06a4-4696-af71-7ce50693039d)
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- Rare benign tumor (fat + hematopoietic elements)
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- Unilateral fatty adrenal tumor (-100 to -30 HU)
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- T1WI: Typically hyperintense; size varies (2-10 cm)
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- Signal loss on T1WI with fat suppression
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- [Adrenal Hemorrhage](/document/adrenal-hemorrhage/5812e5c4-ca8a-4af5-884b-f75795bcde0f)
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- Etiology: Septicemia, burns, trauma, stress, hypotension, and hematological abnormalities
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- CT findings
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- Usually bilateral
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- Old hemorrhage: Soft tissue attenuation (20-35 HU)
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- Recent hemorrhage: ↑ attenuation values
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- MR findings: T1WI and T2WI
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- Varied signal depending on hematoma age
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- Subacute phase: Usually ↑ signal (methemoglobin)
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- Perilesional dark ring (hemosiderin or ferritin)
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- [Adrenal Tuberculosis and Fungal Infection](/document/adrenal-tuberculosis-and-fungal-in-/56a3b7b4-f9bc-4f89-87bb-98b80ddee00a)
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- e.g., tuberculosis, histoplasmosis, other fungal diseases
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- Usually bilateral, heterogeneous, poorly enhancing (acute)
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- Chronic: Small and calcified adrenals
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- Diagnosis: Clinical history and lab data
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Chromaffin cells of adrenal medulla or extraadrenal paraganglioma
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- Adrenal medulla: Pheochromocytoma
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- Extraadrenal: Paraganglioma
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- ### Associated abnormalities
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- Classic associated syndromes
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- VHL syndrome
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- Pheochromocytoma may be only manifestation of VHL or occur along with other tumors
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- NF1
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- MEN syndromes types 2A and 2B
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- Tuberous sclerosis; Sturge-Weber syndrome
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- Carney triad
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- Functional extraadrenal paraganglioma, pulmonary chondroma, gastric leiomyosarcoma
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- Adage that only 10% of pheochromocytomas are hereditary discounted with recent advances in molecular studies
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- 25% of patients with previously considered sporadic pheochromocytomas are carriers of gene mutations
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- Embryology/anatomy
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- Neoplasm of chromaffin cells derived from neural crest or neuroectoderm
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- ## Gross Pathologic & Surgical Features
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- Round, tan-pink to violaceous, encapsulated mass
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- ± cystic, mucoid, serosanguineous hemorrhage
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- ## Microscopic Features
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- Large cells: Granular cytoplasm and pleomorphic nuclei
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- Chromaffin reaction: Cells stained + chromium salt
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Symptoms may be episodic or paroxysmal
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- Crisis: Headaches, hypertension, palpitations, diaphoresis, tremors, arrhythmias, pain
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- Classic triad: Headache, palpitations, diaphoresis
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- 90% specific but uncommon presentation (10-36%)
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- Atypical: Labile hypertension, myocardial infarction, stroke
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- Often clinically silent
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- ### Clinical profile
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- Young patient with paroxysmal attacks of headache, palpitations, sweating, and tremors
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- Lab data
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- 24-hour urine-fractionated metanephrine evaluation often initial biochemical test
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- Excretion of metanephrine, normetanephrine, and 3-methoxytyramine (dopamine metabolite) measured
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- Sensitivity: 90-97%; specificity: 69-98%
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- Plasma-fractionated metanephrines also measured, though low positive predictive value and ↓ specificity results in high false-positive rates
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- ## Demographics
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- ### Age
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- Sporadic pheochromocytoma: Older patients (mean: 44 years)
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- Hereditary pheochromocytoma: Younger patients (mean: 25 years)
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- Pheochromocytomas are exceedingly rare in pediatric patients
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- Higher genetic predisposition and malignancy incidence
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- ### Sex
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- F > M
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- ### Epidemiology
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- Incidence
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- 0.13% in autopsy series; accounts for 0.1-0.5% of patients with hypertension
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- Prevalence likely underestimated
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- ## Natural History & Prognosis
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- Complications: During hypertensive crisis
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- Cerebrovascular accidents
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- Pregnancy + pheochromocytoma: Mortality (48%)
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- Malignancy in 2-14% cases
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- Prognosis
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- Noninvasive and nonmetastatic: Good prognosis
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- Malignant and metastatic: Poor prognosis
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- 5-year survival rate: < 50%
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- ## Treatment
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- Medical therapy: Before, during, and after surgery
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- α-adrenergic blockers
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- Phenoxybenzamine, phentolamine
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- β-adrenergic blocker: Propranolol
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- Surgical resection: Benign and malignant
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- Laparoscopic resection preferred
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- Posterior retroperitoneoscopic adrenalectomy associated with ↓ morbidity compared to transabdominal laparoscopic approach
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- Partial adrenalectomy may be performed with bilateral pheochromocytomas
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- Cortical-sparing adrenalectomy in patients with hereditary pheochromocytoma shows survival similar to total adrenalectomy but recurrence in 13%
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- HSA I-131 MIBG and tumor debulking for metastatic, unresectable, or locally advanced malignant pheochromocytomas
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- Combination chemotherapy: Cyclophosphamide + vincristine + dacarbazine
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# DIAGNOSTIC CHECKLIST
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- ## Consider
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- Clinical history, supporting biochemical data ultimately drive diagnosis
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- ## Reporting Tips
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- CT and MR features (vascularity, cystic change, T2 heterogeneity) may suggest pheochromocytoma, but overlap precludes definitive diagnosis solely by imaging
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- Possibility of pheochromocytoma should be raised, but lab analysis confirms or excludes diagnosis
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483df3a0-a77f-4ff8-9024-1fa2032a515a
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## References
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# Selected References
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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## Images
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### Selected Images
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*Coronal CECT in a 74-year-old man with neurofibromatosis type 1 (NF1) shows a 10-cm, cystic and solid right adrenal mass <img src='img/arrows/CO.png'/>, surgically proven to be a pheochromocytoma. ~ 1-6% of patients with NF1 develop pheochromocytoma. Note IVC filter <img src='img/arrows/WC.png'/>.*
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*Coronal CECT in a 74-year-old man with neurofibromatosis type 1 (NF1) shows a 10-cm, cystic and solid right adrenal mass <img src='img/arrows/CO.png'/>, surgically proven to be a pheochromocytoma. ~ 1-6% of patients with NF1 develop pheochromocytoma. Note IVC filter <img src='img/arrows/WC.png'/>.*
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*Axial T2 FS MR in an 80-year-old man with sporadic pheochromocytoma shows a rounded right adrenal mass <img src='img/arrows/CO.png'/> with heterogeneous signal. Sporadic pheochromocytoma occurs more often in older patients. Note incidental hepatic cyst <img src='img/arrows/WS.png'/>.*
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*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 <img src='img/arrows/CO.png'/> with internal areas of hyperintensity <img src='img/arrows/WC.png'/> suggesting cystic degeneration or necrosis.*
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*Axial T1 C+ FS MR in the same patient shows heterogeneous enhancement of the left adrenal mass <img src='img/arrows/CO.png'/> with cystic degeneration or necrosis <img src='img/arrows/WC.png'/>. Surgical pathology showed pheochromocytoma, which was sporadic in this patient with no family history.*
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*Coronal CECT shows a heterogeneous right adrenal mass <img src='img/arrows/CC.png'/> 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.*
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*Axial CECT in a 44-year-old man with hypertension shows an incidental right adrenal mass <img src='img/arrows/CS.png'/> following motor vehicle collision. The mass was resected via right posterior retroperitoneoscopic adrenalectomy and pheochromocytoma was confirmed.*
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*Axial T2 FS MR in a 66-year-old woman with ↑ 24-hour metanephrines shows a heterogeneous mass in the hepatorenal space <img src='img/arrows/CO.png'/>. Heterogeneous T2 signal is often seen due to varying degrees of hemorrhage and necrosis.*
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*Axial T1 FS C+ MR in the same patient shows heterogeneous enhancement of the surgically proven pheochromocytoma <img src='img/arrows/CO.png'/>. The patient was treated with alpha-blockade (phenoxybenzamine) for 1 month prior to intervention.*
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*Axial CECT in a 79-year-old man with locally recurrent pheochromocytoma shows an irregular, enhancing mass in the left adrenal fossa <img src='img/arrows/WS.png'/> abutting the anterior left kidney <img src='img/arrows/CS.png'/>. Note incidental chronic aortic dissection <img src='img/arrows/BS.png'/>.*
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*Axial Ga-68 DOTATATE PET/CT in the same patient shows avid tracer uptake in the left adrenal fossa <img src='img/arrows/WS.png'/>, corresponding to the enhancing mass on CT and consistent with locally recurrent pheochromocytoma.*
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*Axial CECT shows bilateral, centrally necrotic adrenal masses <img src='img/arrows/BS.png'/> 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.*
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*Axial Cu-61 DOTATATE PET/CT in the same patient shows minimal peripheral uptake by the bilateral adrenal masses <img src='img/arrows/WS.png'/> due to extensive necrosis. Note physiologic uptake in the left kidney <img src='img/arrows/BS.png'/>.*
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*Axial T2 FS MR in a 21-year-old man with neurofibromatosis type 1 (NF1) shows a heterogeneous right adrenal mass <img src='img/arrows/CS.png'/> proven to be pheochromocytoma. While classically described as "light bulb bright," the T2 signal of this neoplasm is highly variable.*
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*Axial CECT shows a heterogeneously enhancing right adrenal mass <img src='img/arrows/CC.png'/> in a 55-year-old man with elevated 24-hour urine metanephrine and pheochromocytoma shown at surgical pathology.*
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*Transverse US of the RUQ for pleuritic chest pain in a 20-year-old man shows a round mass <img src='img/arrows/CO.png'/> posterior to the right hepatic lobe <img src='img/arrows/WS.png'/> with centrally decreased echogenicity <img src='img/arrows/WC.png'/> suggesting necrosis.*
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*Axial CECT in the same patient shows a centrally necrotic right adrenal mass <img src='img/arrows/CO.png'/>, surgically proven pheochromocytoma. Multiple pancreatic cysts <img src='img/arrows/BS.png'/> are also noted, and the diagnosis of familial VHL syndrome was subsequently confirmed.*
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### Additional Images
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*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 <img src='img/arrows/WS.png'/>, consistent with recurrent pheochromocytoma.*
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*Axial CECT in a 36-year-old woman shows a 7-cm, heterogeneous left adrenal mass <img src='img/arrows/WC.png'/>. The differential includes adrenal carcinoma, pheochromocytoma, and metastasis. Urinary metanephrines were ↑, and laparoscopic resection (after α and β blockade) confirmed hemorrhagic pheochromocytoma.*
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*Axial T2 MR in the same patient shows a hyperintense ("light bulb") left adrenal lesion <img src='img/arrows/WC.png'/>. 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.*
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*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 <img src='img/arrows/WS.png'/>.*
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*Axial T2 MR in the same patient shows a slightly intense right adrenal lesion <img src='img/arrows/WS.png'/> and adjacent renal/hepatic cysts <img src='img/arrows/WC.png'/>. Elevated 24-hour urinary metanephrines indicated (sporadic) unilateral pheochromocytoma, although the imaging appearance is nonspecific.*
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