case_images: PMC11788273_01
case_images: Major clinical and imaging findings in a 26-year-old woman with TSC. (A) Facial photograph demonstrates multiple angiofibromas on her face. (B) A shagreen patch on the right waist. (C) Subungual fibromas affecting toenails. (D,E) Echocardiogram showing a BAV with lobe thickening and severe aortic valve insufficiency. (F) Head CT demonstrating bilateral calcifications in the lateral ventricles and a nodular calcification. (G) Brain T2-weighted FLAIR sequence MR showing multiple cortical and subependymal nodules. (H) Chest CT revealing multiple cystic lesions and a well-circumscribed nodule in the right middle lobe. (I) Epigastrium postcontrast CT suggesting rapid arterial phase enhancement and delayed washout in liver S4 and fatty nodule without enhancement in S6. Both kidneys are enlarged (left more prominent) with multiple fat-containing lesions. (J) MIP image from a CT angiogram of the left kidney demonstrating numerous tortuous, narrowed, and dilated arterioles.
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case_images: F1
case_images: Physical examination revealed fibrous plaques on the forehead, multiple facial angiofibroma (Figure 1A), a shagreen patch on the right waist (Figure 1B), and subungual fibromas on both hands and feet (Figure 1C). Leaflet thickening was observed during systolic phase, and moderate to severe AR was present during diastolic phase (Figure 1D,E). Left ventricular diameter increased and aortic ring diameter was 28 mm. Head CT revealed bilateral calcifications in the lateral ventricles and a 13-mm nodular calcification within the right frontal horn (Figure 1F). Brain T2-weighted FLAIR sequence MRI further delineated multiple cortical and subependymal nodules (Figure 1G). Chest CT demonstrated lymphangioleiomyomatosis in both lungs, characterized by multiple cysts (the largest measuring approximately 22 mm) and a well-circumscribed nodule in the right middle lobe (Figure 1H). A fatty nodule without enhancement in S6 was consistent with an AML (Figures 1I). Left renal CT angiogram revealed multiple tortuous, narrowed, and dilated arterioles within the angiomyolipomas, with several aneurysmal dilations, the largest measuring 27 mm (Figure 1J).
case_images: Histopathological and postoperative imaging findings. (A) Resected aortic valve demonstrating fibrous tissue hyperplasia with hyaloid and mucoid degeneration (hematoxylin-eosin stain, x100). (B) Postoperative mediastinum CT revealing a large pleural effusion and pericardial effusion in the left thorax and pericardium.
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case_images: F2
case_images: Pathological examination showed fibrous tissue hyperplasia with hyaline and myxoid degeneration (Figure 2A). Chest CT eleven days post-surgery revealed large left-sided pleural and pericardial effusions (Figure 2B), requiring thoracentesis and pericardiocentesis, which drained chylous fluid and hematoma, respectively.
case_images: Timeline of diagnosis and treatment for case of TSC.
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case_images: F3