Review Article

Adrenal Tumors with Unexpected Outcome: A Review of the Literature

Table 1

Summary of imaging and pathological characteristics of rare adrenal pathologies.

Diagnosis Number of cases Imaging characteristics Pathological characteristicsClinical behavior
CTMRIHistologyImmunohistochemistry +

Primary adrenal lymphoma [815]<200Mostly hypodense tumors, aspect homo- or heterogeneous, slight to moderate contrast enhancement. Iso/hypointense in T1 and hyperintense in T2.Atypical cells, anisokaryosis, hyperchromasia, necrosis.Most common: CD45/CD20/CD40 (B-cell)
CD3/CD30/CD43 (T-cell)
Malignant

Dedifferentiated liposarcoma [1622]>500DDLS: nonlipogenic, heterogeneous node within a well-delineated, lipogenic, and septated mass that is the WDLSWDLS: >75% fat, nonlipomatous components are prominent thick septa. Nodular nonadipose areas may be present.
DDLS within WDLS: low to intermediate on T1 and intermediate to high on T2.
Atypical nonlipogenic stromal cells with hyperchromatic nuclei, scattered in fibrous septa. Cellularity and nuclear atypia increase with dedifferentiation. Mitotic rate typically <8/10HPF.MDM2, CDK4Malignant

Schwannoma [2327]>500Round, well-circumscribed, hypo- or iso-intense compared to muscle, enhancement postcontrast.Intermediate on T1 (isointense to muscle), mared increase on T2. Elongated spindle cells in areas of both high (Antoni A) and low cellularity (Antoni B).NSE, microfilament, S100Benign

Ganglioneuroma [2832]>60Att. > 25 HU, homogeneous aspect, calcifications in 30%–60%.Hypointense on T1, heterogen. hyperintense on T2.Ganglion cells, spindle cells, nerve fibres.NSE, synaptophysin, S100, and CD57.Benign
Rare: transformation to malignant nerve sheath tumor.

Idiopathic adrenal haematoma [3335]>10Variable: homo/heterogeneous depending on lesion’s age.High intensity on T1 in periphery of lesion suggests hemorrhage.Hemorrhage, necrosis and hemosiderin.Benign

Cavernous haemangioma [3639]>60Heterogeneous, central cystic/necrotic components, calcifications, nodular peripheral enhancement postcontrast.Homogeneous on T1, high intensity on T2.Necrosis, cystic components, large vascular spaces, single lining of endothelium.Benign
Risk of spontaneous hemorrhage.
Rare: transformation to angiosarcoma.

Angiomyolipoma [4044]<10Heterogeneous: contains fat, possibly small enhancing foci.Adipose tissue, smooth muscle fibres. HMB45, MART1/MelanA, smooth muscle actin.Benign
Risk of spontaneous hemorrhage.

Epithelioid angiosarcoma [4549]>20Irregular margins, nonhomogeneous density, calcifications.High intensity on T2.Vascular spaces lined by endothelial cells with epithelioid features, possibly pleomorphism.Factor VIII, also CD34 and UEA-1 (less specific).Malignant

Leiomyosarcoma [50, 51]<20Heterogeneous, possibly also liquid components.Spindle-shaped neoplastic cells, nuclear pleomorphism, giant cell formation.Smooth muscle actin.Malignant

Cyst [5257]>600
Pseudocyst 39%Fibrous wall, no endothelial/epithelial lining, dependent on age septations, blood products, fluid-fluid level, or soft tissue component.Intermediate/high density in T1, marked bright up in T2.Usually unilocular, no endothelium. Contains brown/reddish fluid. Connective tissue walls calcificated/hyalinized.Benign
Endothelial 45%Thin wall (≤3.5 mm), smooth borders and pure cystic internal structure. Att. < 20 HU. No contrast enhancement.Smooth endothelial lining, contains clear or milky fluid.D2-40.Benign
Epithelial 9%Lined with cylindrical epithelium.Calretinin and WT-1.Benign
Parasitic 7%Floating membrane or daughter cysts, septal or mural calcifications, coexistent hydatid cysts of other organs. Thick, possibly calcificated walls, parasites within.Benign

DDLS: dedifferentiated liposarcoma, WDLS: well-differentiated liposarcoma, Att: attenuation on unenhanced CT, HU: Hounsfield units, NSE: neuron-specific enolase, UEA-1: Ulex Europaeus Agglutinin 1, WT-1: Wilms tumor protein, and Immunohistochemistry +: Positive immunohistochemical staining.