Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome

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 ca...

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Autores principales:	Selvin Theodore Jayanth, Partho Mukherjee, Arun Jacob Philip George, J. Chandrasingh, T. J. Nirmal, Rajiv Paul Mukha, Santosh Kumar, Antony Devasia
Formato:	article
Lenguaje:	EN
Publicado:	SpringerOpen 2021
Materias:	VHLS Nephron sparing surgery Cortical sparing adrenalectomy Diseases of the genitourinary system. Urology RC870-923
Acceso en línea:	https://doaj.org/article/8c76da2a126b49138b7efa7f06a6019c
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spelling	oai:doaj.org-article:8c76da2a126b49138b7efa7f06a6019c2021-11-14T12:16:30ZOutcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome10.1186/s12301-021-00253-81110-57041961-9987https://doaj.org/article/8c76da2a126b49138b7efa7f06a6019c2021-11-01T00:00:00Zhttps://doi.org/10.1186/s12301-021-00253-8https://doaj.org/toc/1110-5704https://doaj.org/toc/1961-9987Abstract 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.Selvin Theodore JayanthPartho MukherjeeArun Jacob Philip GeorgeJ. ChandrasinghT. J. NirmalRajiv Paul MukhaSantosh KumarAntony DevasiaSpringerOpenarticleVHLSNephron sparing surgeryCortical sparing adrenalectomyDiseases of the genitourinary system. UrologyRC870-923ENAfrican Journal of Urology, Vol 27, Iss 1, Pp 1-8 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	VHLS Nephron sparing surgery Cortical sparing adrenalectomy Diseases of the genitourinary system. Urology RC870-923
spellingShingle	VHLS Nephron sparing surgery Cortical sparing adrenalectomy Diseases of the genitourinary system. Urology RC870-923 Selvin Theodore Jayanth Partho Mukherjee Arun Jacob Philip George J. Chandrasingh T. J. Nirmal Rajiv Paul Mukha Santosh Kumar Antony Devasia Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome
description	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.
format	article
author	Selvin Theodore Jayanth Partho Mukherjee Arun Jacob Philip George J. Chandrasingh T. J. Nirmal Rajiv Paul Mukha Santosh Kumar Antony Devasia
author_facet	Selvin Theodore Jayanth Partho Mukherjee Arun Jacob Philip George J. Chandrasingh T. J. Nirmal Rajiv Paul Mukha Santosh Kumar Antony Devasia
author_sort	Selvin Theodore Jayanth
title	Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome
title_short	Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome
title_full	Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome
title_fullStr	Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome
title_full_unstemmed	Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome
title_sort	outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of von hippel–lindau syndrome
publisher	SpringerOpen
publishDate	2021
url	https://doaj.org/article/8c76da2a126b49138b7efa7f06a6019c
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Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome

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