دورية أكاديمية
Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome
| العنوان: | Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome |
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| المؤلفون: | Selvin Theodore Jayanth, Partho Mukherjee, Arun Jacob Philip George, J. Chandrasingh, T. J. Nirmal, Rajiv Paul Mukha, Santosh Kumar, Antony Devasia |
| المصدر: | African Journal of Urology, Vol 27, Iss 1, Pp 1-8 (2021) |
| بيانات النشر: | SpringerOpen, 2021. |
| سنة النشر: | 2021 |
| المجموعة: | LCC:Diseases of the genitourinary system. Urology |
| مصطلحات موضوعية: | VHLS, Nephron sparing surgery, Cortical sparing adrenalectomy, Diseases of the genitourinary system. Urology, RC870-923 |
| الوصف: | Abstract Background The management of Von Hippel–Lindau syndrome (VHLS) is multidisciplinary. The urologist is involved in treating the renal, epididymal tumors and often adrenal pheochromocytoma. Preservation of renal and adrenal function is a challenge. We present 17 cases of VHLS in a tertiary care center in South India. Methods A retrospective review of the patients who underwent surgical treatment under urology for VHLS from January 2009 to November 2018 was conducted. The demographic data, the spectrum of manifestation, treatment, change in glomerular filtration rate, adrenal insufficiency, and recurrence-free survival were analyzed. Results There were 17 patients diagnosed with VHLS. The median age of diagnosis was 39 years (range 23–41). The spectrum of clinical manifestation was: multifocal RCC (88%), pancreatic cysts/tumors (70%), cerebellar hemangioblastoma (59%), retinal angiomas (47%), epididymal cysts/tumors (47%), pheochromocytomas (41%), and spinal hemangiomas (30%). There were seven patients with ten pheochromocytoma lesions. Three underwent cortical sparing and seven total adrenalectomies; 13 patients underwent nephron sparing surgery (NSS), of which seven patients had bilateral tumors. The median duration of follow-up was 6.5 years (range 2–12 years). Following NSS, seven patients had a local recurrence, and one developed pancreatic metastasis. Two patients (11%) were lost to follow-up. Renal function was preserved in all patients at the last follow-up, and there was no postoperative adrenal crisis or mortality. Conclusion Nephron sparing surgery and cortical sparing adrenalectomy are the treatment of choice for multifocal RCC and pheochromocytomas in patients with VHLS providing good oncological outcomes and preservation of renal and adrenal function. |
| 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/s12301-021-00253-8 |
| الوصول الحر: | https://doaj.org/article/8c76da2a126b49138b7efa7f06a6019cTest |
| رقم الانضمام: | edsdoj.8c76da2a126b49138b7efa7f06a6019c |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 11105704 19619987 |
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| DOI: | 10.1186/s12301-021-00253-8 |