Bilateral renal angiomyolipomas in tuberous sclerosis
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 an...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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SpringerOpen
2021-04-01
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| Series: | African Journal of Urology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12301-021-00161-x |
| _version_ | 1818415226164346880 |
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| author | Bushra Jawaid Abdul Hafeez Qureshi Nadeem Ahmed Nousheen Yaqoob |
| author_facet | Bushra Jawaid Abdul Hafeez Qureshi Nadeem Ahmed Nousheen Yaqoob |
| author_sort | Bushra Jawaid |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-14T11:31:37Z |
| format | Article |
| id | doaj.art-37ff7b597911437fa72a8c4a5189a48a |
| institution | Directory Open Access Journal |
| issn | 1110-5704 1961-9987 |
| language | English |
| last_indexed | 2024-12-14T11:31:37Z |
| publishDate | 2021-04-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | African Journal of Urology |
| spelling | doaj.art-37ff7b597911437fa72a8c4a5189a48a2022-12-21T23:03:16ZengSpringerOpenAfrican Journal of Urology1110-57041961-99872021-04-012711510.1186/s12301-021-00161-xBilateral renal angiomyolipomas in tuberous sclerosisBushra Jawaid0Abdul Hafeez Qureshi1Nadeem Ahmed2Nousheen Yaqoob3The Indus Hospital KarachiThe Indus Hospital KarachiThe Indus Hospital KarachiThe Indus Hospital KarachiAbstract 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.https://doi.org/10.1186/s12301-021-00161-xAngiomyolipomaTuberous sclerosisBilateral renal angiomyolipomaMTOR inhibitors |
| spellingShingle | Bushra Jawaid Abdul Hafeez Qureshi Nadeem Ahmed Nousheen Yaqoob Bilateral renal angiomyolipomas in tuberous sclerosis African Journal of Urology Angiomyolipoma Tuberous sclerosis Bilateral renal angiomyolipoma MTOR inhibitors |
| title | Bilateral renal angiomyolipomas in tuberous sclerosis |
| title_full | Bilateral renal angiomyolipomas in tuberous sclerosis |
| title_fullStr | Bilateral renal angiomyolipomas in tuberous sclerosis |
| title_full_unstemmed | Bilateral renal angiomyolipomas in tuberous sclerosis |
| title_short | Bilateral renal angiomyolipomas in tuberous sclerosis |
| title_sort | bilateral renal angiomyolipomas in tuberous sclerosis |
| topic | Angiomyolipoma Tuberous sclerosis Bilateral renal angiomyolipoma MTOR inhibitors |
| url | https://doi.org/10.1186/s12301-021-00161-x |
| work_keys_str_mv | AT bushrajawaid bilateralrenalangiomyolipomasintuberoussclerosis AT abdulhafeezqureshi bilateralrenalangiomyolipomasintuberoussclerosis AT nadeemahmed bilateralrenalangiomyolipomasintuberoussclerosis AT nousheenyaqoob bilateralrenalangiomyolipomasintuberoussclerosis |