Splenic hamartoma in tuberous sclerosis | Figure 1

CincyKidsRad

Cincinnati Children's Radiology

Splenic hamartoma in tuberous sclerosis

Axial T1-weighted (image 1), T2-weighted (image 2), and opposed-phase (image 3) MRI of the spleen in a patient with tuberous sclerosis shows two splenic hamartomas (arrow). Splenic hamartomas are rare tumors associated with tuberous sclerosis. They have a heterogeneous appearance caused by foci of hemorrhage. In the absence of hemorrhage, lesions may be difficult to distinguish from the background spleen on CT and T1-weighted images.

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Axial FLAIR (image 1) and sagittal T1 FLAIR MRI images show a hypothalamic hamartoma (arrow). Patients with these lesions classically present with gelastic or laughing seizures. Hypothalamic hamartomas may also cause symptoms such as precocious puberty or behavioral and cognitive difficulties. On imaging, hypothalamic hamartomas are isointense to gray matter on T1-weighted images and hyperintense on T2-weighted images, without enhancement after contrast administration.

Sagittal (image 1) and axial (image 2) T1-weighted and coronal T1-weighted postcontrast (image 3) MRI show a hypothalamic hamartoma (arrow). The normal pituitary (arrowhead) is shown nearby. Hypothalamic hematomas are nonprogressive tumors that grow in proportion to the normal brain. Symptoms typically occur in childhood and include central precocious puberty or gelastic seizures.

Axial T1 FLAIR MRI shows subependymal gray matter heterotopia (arrowhead), the most commonly observed form of gray matter heterotopia. The differential diagnosis for this finding includes subependymal nodules that occur in tuberous sclerosis complex. Gray matter heterotopia is distinguished from subependymal nodules of tuberous sclerosis by the signal intensity of the nodule; in tuberous sclerosis, the T2 signal of subependymal nodules is higher than that of normal gray matter while nodules in gray matter heterotopia are isointense to gray matter. Patients with tuberous sclerosis may also have additional stigmata such as subcortical tubers and subependymal giant cell astrocytomas.

Axial T2 (image 1) and T1 FLAIR (image 2) MRI in a patient with tuberous sclerosis complex show multiple subependymal nodules (arrowhead) and subcortical tubers (arrow). Subependymal giant cell astrocytomas, affect up to 15% of patients with tuberous sclerosis and can lead to obstructive hydrocephalus. The classic triad of tuberous sclerosis includes seizures, intellectual disability, and adenoma sebaceum.