Answer for BIR CoW 29 Aug 2021
Tuberous sclerosis with subependymal giant cell astrocytoma
Findings
Evidence of T1 isointense , T2 FLAIR hyperintense solid lesion with lobular margins is seen arising from septum pellucidum with extension in to anterior horn of right lateral ventricle and Foramen of Monroe with surrounding edema along with blooming foci corresponding to calcification is seen within the lesion .The lesion shows no diffusion restriction.on contrast administration the lesion shows marked enhancement. Multiple subependymal nodules are seen along the walls of lateral ventricle Evidence of linear streaky cortical bands seen in juxtaventricular white matter
Discussion
Tuberous sclerosis is a rare autosomal dominant neurocutaneous syndrome characterized by the presence of benign congenital tumors in multiple organs. Because the classical triad of epilepsy, mental retardation, and adenoma sebaceum is uncommonly seen at clinical examination, radiologic examinations can play an important role in the diagnosis of tuberous sclerosis and in treatment. Cardiac rhabdomyoma, renal angiomyolipoma, and neurologic involvement encompassing cortical or subependymal tubers and white matter abnormalities are the common radiologic findings. Detection of these entities can be strong evidence for suspecting tuberous sclerosis. Subependymal nodules represent hamartomatous change in subependymal tissue and usually occur as multiple nodules. The prevalence of patients with more than 10 subependymal nodules ranges from 12% to 57% CT is a useful tool for detection of subependymal nodules, since they are associated with calcification far more commonly (88%) than are cortical tubers. Unenhanced CT typically depicts multiple small foci with dense calcification along the lateral ventricles bilaterally . At MR imaging, subependymal nodules are hyperintense on T1-weighted images and iso- to hyperintense on T2-weighted images Subependymal nodules tend to have lower signal intensity on T2-weighted image than do cortical tubers, probably because subependymal nodules have high water content SGCAs are characterized by proliferative astrocytes and giant cells, with a prevalence 1.7%–26% in patients with TS. The typical location of SGCAs is in the foramen of Monro, leading to obstructive hydrocephalus .Typically, the initial symptom of SGCAs is increased intracranial pressure, frequently with acute onset . Different from other cerebral astrocytomas, SGCAs have benign biologic and pathologic features (ie, slow growth, minimal or no attendant brain edema, and minimal invasiveness). It is widely accepted that SGCAs are derived from subependymal nodules; this is supported by the existence of intermediate cells between hamartomatous nodules and SGCAs and by serial CT studies indicating growth of nodules into SGCAs . The peak age of occurrence is 8–18 years, although SGCAs can evolve in utero Radial white matter bands reflect altered development along the migratory pathways of neurons and glial cells. They usually involve cerebral white matter bilaterally (predominantly frontal lobes) and uncommonly cerebellar white matter . At MR imaging, thin straight or curvilinear bands of hyperintensity on T2-weighted images and iso- to hypointensity to normal white matter on T1-weighted images run from ventricular or juxtaventricular white matter to the deep surface of cortical tubers or normal-appearing cortex.
Findings
Evidence of T1 isointense , T2 FLAIR hyperintense solid lesion with lobular margins is seen arising from septum pellucidum with extension in to anterior horn of right lateral ventricle and Foramen of Monroe with surrounding edema along with blooming foci corresponding to calcification is seen within the lesion .The lesion shows no diffusion restriction.on contrast administration the lesion shows marked enhancement. Multiple subependymal nodules are seen along the walls of lateral ventricle Evidence of linear streaky cortical bands seen in juxtaventricular white matter
Discussion
Tuberous sclerosis is a rare autosomal dominant neurocutaneous syndrome characterized by the presence of benign congenital tumors in multiple organs. Because the classical triad of epilepsy, mental retardation, and adenoma sebaceum is uncommonly seen at clinical examination, radiologic examinations can play an important role in the diagnosis of tuberous sclerosis and in treatment. Cardiac rhabdomyoma, renal angiomyolipoma, and neurologic involvement encompassing cortical or subependymal tubers and white matter abnormalities are the common radiologic findings. Detection of these entities can be strong evidence for suspecting tuberous sclerosis. Subependymal nodules represent hamartomatous change in subependymal tissue and usually occur as multiple nodules. The prevalence of patients with more than 10 subependymal nodules ranges from 12% to 57% CT is a useful tool for detection of subependymal nodules, since they are associated with calcification far more commonly (88%) than are cortical tubers. Unenhanced CT typically depicts multiple small foci with dense calcification along the lateral ventricles bilaterally . At MR imaging, subependymal nodules are hyperintense on T1-weighted images and iso- to hyperintense on T2-weighted images Subependymal nodules tend to have lower signal intensity on T2-weighted image than do cortical tubers, probably because subependymal nodules have high water content SGCAs are characterized by proliferative astrocytes and giant cells, with a prevalence 1.7%–26% in patients with TS. The typical location of SGCAs is in the foramen of Monro, leading to obstructive hydrocephalus .Typically, the initial symptom of SGCAs is increased intracranial pressure, frequently with acute onset . Different from other cerebral astrocytomas, SGCAs have benign biologic and pathologic features (ie, slow growth, minimal or no attendant brain edema, and minimal invasiveness). It is widely accepted that SGCAs are derived from subependymal nodules; this is supported by the existence of intermediate cells between hamartomatous nodules and SGCAs and by serial CT studies indicating growth of nodules into SGCAs . The peak age of occurrence is 8–18 years, although SGCAs can evolve in utero Radial white matter bands reflect altered development along the migratory pathways of neurons and glial cells. They usually involve cerebral white matter bilaterally (predominantly frontal lobes) and uncommonly cerebellar white matter . At MR imaging, thin straight or curvilinear bands of hyperintensity on T2-weighted images and iso- to hypointensity to normal white matter on T1-weighted images run from ventricular or juxtaventricular white matter to the deep surface of cortical tubers or normal-appearing cortex.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!