دورية أكاديمية
Bilateral renal angiomyolipomas in tuberous sclerosis
| العنوان: | Bilateral renal angiomyolipomas in tuberous sclerosis |
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| المؤلفون: | Bushra Jawaid, Abdul Hafeez Qureshi, Nadeem Ahmed, Nousheen Yaqoob |
| المصدر: | African Journal of Urology, Vol 27, Iss 1, Pp 1-5 (2021) |
| بيانات النشر: | SpringerOpen, 2021. |
| سنة النشر: | 2021 |
| المجموعة: | LCC:Diseases of the genitourinary system. Urology |
| مصطلحات موضوعية: | Angiomyolipoma, Tuberous sclerosis, Bilateral renal angiomyolipoma, MTOR inhibitors, Diseases of the genitourinary system. Urology, RC870-923 |
| الوصف: | Abstract Background Tuberous sclerosis complex (TSC) is a rare genetic disorder characterized by benign hamartomas in multiple organs of the body. Renal angiomyolipomas (AML) are commonly associated with TSC. They are mostly asymptomatic. But large and rapidly growing AMLs with the presence of an aneurysm cause symptoms and pose a life-threatening risk for hemorrhage. Case presentation Our patient is a 25-year-old female who presented to us as an undiagnosed case of tuberous sclerosis having a large abdominal mass. She fulfilled the clinical criteria required for the diagnosis of TSC. The CT scan revealed an 18 × 13 × 33 cm fat-containing lesion in the right kidney with an adjacent aneurysm measuring around 16 cm in diameter. Due to the large size of the AML and associated aneurysm, surgical exploration was mandated. On the contralateral kidney, multiple contrast-enhancing soft-tissue densities were present that appeared suspicious on radiology. So a percutaneous biopsy of those lesions was done. Fortunately, it had the same histopathology as an Angiomyolipoma. Nephrectomy of the right-sided kidney with AML has been done. The left-sided lesions that are less than 2 cm and asymptomatic are kept on close surveillance. Any change in size will prompt therapy with mTOR inhibitors. Conclusion When dealing with bilateral renal AML, it is important to adopt a conservative approach. When intervention is indicated, the least invasive strategy should be sought and enacted. Radical surgery should be the last resort. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 1110-5704 1961-9987 |
| العلاقة: | https://doaj.org/toc/1110-5704Test; https://doaj.org/toc/1961-9987Test |
| DOI: | 10.1186/s12301-021-00161-x |
| الوصول الحر: | https://doaj.org/article/37ff7b597911437fa72a8c4a5189a48aTest |
| رقم الانضمام: | edsdoj.37ff7b597911437fa72a8c4a5189a48a |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 11105704 19619987 |
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| DOI: | 10.1186/s12301-021-00161-x |