PMC8063773_01
Grey scale (a) and Colour Doppler ultrasound (b) of the left kidney shows a large hetero-echoic left renal mass with intense vascularity at the periphery of the mass and a central avascular component (white arrow heads).
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The lesion demonstrated intense internal vascularity and a central avascular hyperechoic area (Figure 1).
The axial pre-contrast (a) and post-contrast (b-d) and coronal reformatted pre-contrast (e) and post-contrast (f-h) computed tomography (CT) images demonstrating renal and hepatic angiomyolipomas (AMLs). Multiple bilateral renal AMLs have small foci of fat attenuation within (white arrows; a, e). There is heterogeneous enhancement of these renal masses with multiple dilated vascular channels (black arrows) within the left renal mass suggestive of intra-lesional aneurysms and dilated draining venous channels at the periphery. A large hyperdense non-enhancing intra-tumoural haematoma (black arrowheads) is seen within the largest left-sided AML (measuring 17 cm in the largest dimension), which had ruptured. A thin perinephric haematoma (white arrowheads; c, h) was seen on the left. Free fluid was also seen in the pelvis (yellow asterisks; d, h). Well-defined fat attenuated round lesions scattered in the liver suggestive of lipid-rich hepatic AMLs (bent arrow; e-f).
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Similar, but smaller lesions were also seen in the right kidney along with fat-containing lesions in the liver (Figure 2).
The serial contrast-enhanced computed tomography (CT) brain images demonstrate coarsely calcified cortical tubers in both cerebellar hemispheres (arrowheads, a), multiple calcified subependymal nodules along the walls of both lateral ventricles (thick black arrows, b) with a subependymal giant cell tumour (SEGCT) and associated peri-tumoural cyst in relation to the frontal horn of the right lateral ventricle, extending to the white matter of the right frontal lobe (thick white arrows, b) No hydrocephalus is seen as the tumour is away from the foramen of Monro, the usual location of SEGCT. Multiple hypodense non-calcified cortical tubers were also seen in the cerebral parenchyma (thin white arrow, c). The axial (d) and coronal (e) CT thorax lung windows show multiple small thin-walled cysts scattered diffusely throughout the lung parenchyma without any zonal predilection, suggestive of lymphangioleiomyomatosis (LAM). A few randomly distributed nodules are also seen in both upper lobes (black arrows) representing multifocal micronodular pneumocyte hyperplasia (MMPH).
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A CECT scan of the brain revealed multiple cerebral and cerebellar calicified, as well as non-calcified cortical tubers, subependymal calcified nodules and a large cystic lesion with an enhancing mural nodule in the subependymal region of the frontal horn of the right lateral ventricle, consistent with a subependymal giant cell tumour (Figure 3). TSC, revealing multiple, round, thin-walled cysts and a few small randomly distributed nodules uniformly scattered across both lungs, without any pneumothorax or chylous effusions. The findings were consistent with lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (Figure 3).
Bone manifestations of tuberous sclerosis complex. Reformatted sagittal computed tomography (CT) in bone window (a) shows patchy and round sclerotic lesions (white arrows) involving the posterior elements and vertebral bodies at multiple levels in the thoracolumbar spine. The axial bone window of the CT head (b) demonstrates a diffusely thickened calvarium with patchy sclerosis. An oblique radiograph of the left hand (c) shows typical intra-osseous cysts in the terminal phalanges (white arrows). The left thumb and second digit (b) additionally show multiple cortical defects or scalloping (arrow heads) and periostitis with associated soft-tissue components, likely representing large cutaneous angiofibromas or hamartomas.
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Digital subtraction angiography images of the selective renal embolisation procedure: (a) catheter angiogram of the left main renal artery shows a solitary aneurysm (solid white arrow) and multiple clustered aneurysms in the lower pole (open arrow), suggestive of abnormal intra-tumoural vascularity in a renal angiomyolipoma. (b) Super-selective angiogram using a microcatheter (red arrow) indicates tumoural hypervascularity and clustered small aneurysms in the lower pole of the kidney (encircled). (c) Super-selective angiogram reveals the rounded area of absent parenchymal blush (star) suggestive of haematoma. There is hypervascularity in the surrounding parenchyma. (d) Post-embolisation catheter angiogram from the main renal artery demonstrated the de-vascularised arterial stump (white arrow). Embolised tumoural vessels with glue cast and alcohol (encircled). The upper pole of the kidney is spared and shows normal enhancement.
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A 5Fr-renal double curve catheter (Cook Medical, Bloomington, USA) was used to obtain an angiogram of the left main renal artery which revealed massive enlargement of the renal outline, hypertrophy of the dorsal branch of the renal artery supplying the AML, multiple aneurysms at the lower pole and an associated bizarre pattern of tumoural hypervascularity (Figure 5a). Using a 2.7Fr-microcatheter, super-selective angiograms were obtained from multiple segmental branches of the dorsal branch of left renal artery, which revealed tumoural hypervascularity, multiple clustered aneurysms (Figure 5b) and a hypo-vascular region in the centre of the renal outline, splaying the vasculature and corresponding to the intra-tumoural hematoma (Figure 5c). The post-embolisation angiogram revealed complete occlusion of the abnormal tumoural vessels with preservation of the normal renal parenchyma (Figure 5d).