medical history
Female, 36 years old, accidental physical examination found abnormal lung lesions.
The two HRCT paintings above show random distributions of ground glass nodular foci of varying sizes in both lungs.
Abdominal CT shows ivory osteosclerosis in the lumbar spine and a well-defined low-density nodular foci in the left kidney.
Enhanced scans show fat density in the left renal lesion, lace-like intensification around it, and separation visible within it.
Thoracopic biopsy and pathological diagnosis result in multifocal micronodular cell hyperplasia (MMPH) and left renal lesions as renal vascular leiomyolioma.
discuss
1. Tuberous sclerosis syndrome (TSC), also known as Bourneville-Pringle disease, systemic multi-organ tissue defects and benign connective tissue hamartomas are the histopathological basis of TSC, excluding peripheral neuroskeletal muscle, pineal cones in vitro can affect all tissues and organs, mainly skin nervous system involvement, there is a familial tendency and age genetic effects.
2. Clinical characteristics of TSH:
TSC is more common in children and typically presents with a Vogt triad (epilepsy, mental retardation, sebaceous adenoma).
TSC involvement of kidneys and liver is mostly accidental, so the first clinical diagnosis is easy to misdiagnosis or missed diagnosis, and its symptoms are related to the size and complications of the lesion tumor, which can increase the risk of spontaneous renal bleeding and kidney cancer, and kidney lesions are considered to be the most common cause of death of TSC.
3. Imaging characteristics and pathological basis:
Changes in the brain: The pathology of the neurosclerosis nodule is composed of abnormal glial nerve-forming cells, glialocytes, ganglionocytes, etc., often with calcification.
Imagery is characteristic:
(1) Multiple ependymal nodules, multiple symmetrical distribution, calcified nodules are characteristic;
(2) Cortex or subcortical nodules, most common in the frontal parietal lobe;
(3) Abnormal signals in the white matter area of the brain, characterized by radial, wedge-shaped or amorphous slightly longer T2 signal shadow, T2WI-FLAIR shows obvious nodules showing equal or slightly higher density and T1 slightly longer T2 signal, enhanced scan is not strengthened or strengthened is not obvious, when the nodules are enlarged and significantly strengthened, malignant subependymal giant cell astrocytoma should be considered, the latter is mostly located in the transparent septum and Montessori pore area, CT is the most sensitive examination method for finding subependymal calcification nodules, literature reports, for clinical high suspicion In patients with TSC, CT may be the preferred method of diagnosis for the initial screening of TSC.
Extraencephalic changes:
(1) TSC with hepatic and renal AML: TSC liver and kidney AML are characterized by fat content in the tumor, the solid part of the tumor body is significantly strengthened and the duration of strengthening is longer, the tumor body increases over time, 80% TSC is combined with double kidney multiple AML, and the liver AML and the double kidney AML in TSC have a significant correlation, and the combination of the two suggests TSC.
(2) TSC lung lesions: including MMPH and LAM, MMPH histologically hyperplastic type II alveolar cells, manifested by diffuse distribution of small nodules with increased density shadow with thickening of lobular septum; LAM is characterized by abnormal proliferation of vascular smooth muscle, manifested by thin-walled (<3 mm) clear lung cysts evenly distributed throughout the lung.
(3) TSC Skeletal changes: the pathological mechanism may be related to the fusion of mature bone trabeculae during bone growth and ossification without resorption and molding, resulting in dense bone plaque development abnormalities. Characteristic skeletal imaging with multiple sclerosis nodules in the vertebral body with ivory arcosis; At the same time, it was found that bone changes are more common in the lumbar spine and are first involved, and the first to involve the appendages, the vertebral lesions are mostly located in the posterior half of it, and the bone hardening and volume increase are seen in advanced cases. Skull lesions are predominantly plate obstruction changes, with the proximal medial side being pronounced, some with local volume increase. The results showed that the bone changes in this disease are characterized by multiple bone and progressive exacerbations. This disease needs to be distinguished from renal bone disease, osteogenesis metastatic bone tumor, and candle oil osteoporosis.
TSC has the characteristics of progressive multi-organ involvement, early diagnosis is of great significance, and the first misdiagnosis rate of extraencephalopathy involvement organ lesions is high, clinicians should raise awareness, familiarize themselves with the imaging characteristics of TSC clinical and related affected organs, and detect and treat or intervene in the potential life-threatening comorbidities of the affected organs as early as possible. CT and MRI have great value in the diagnosis and follow-up of TSC, with the role of a weather vane, the intracerebral changes are characteristic and the MRI value is outstanding, and the extra-cerebral changes deserve attention and have certain imaging characteristics and obvious clinical value.
bibliography:
RUAN Zhibing,JIAO Jun,MIN Dingyu,LIAO Xin,WEN Wei. CT and MR manifestations of tuberous sclerosis syndrome[J]. Journal of Clinical Radiology, 2015, v.34; No.30310:1560-1565.
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