Management Of Renal Cell Carcinoma In Von Hippel–Lindau Disease

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Contemporary Management of Renal Masses Final - Handout

Disease Gene (chromosome) Histology Frequency von Hippel-Lindau VHL (3) Clear Cell 75% HLRCC* FH (1) Papillary Type 2 10% Birt-Hogg-Dube BHD (17) Chromophobe/Onc ocytoma 10% Hereditary papillary RCC Met (7) Papillary Type 1 5% Hereditary RCC *HLRCC = Hereditary Leiomyomatosis Renal Cell Carcinoma RCC occurs in 50% of VHL patients ‒Males

Von Hippel-Lindau disease - Genomics Education Programme

VHL disease most frequently affects the eyes, cerebellum, kidneys, spinal cord, adrenal gland or pancreas. Retinal angiomas are often the initial manifestation of VHL disease and can cause vision loss if not treated. Renal cell carcinoma occurs in about 70% of individuals with VHL, necessitating surveillance.

Renal cancer - The Lancet

markers in the same cohort. 24,25 Hereditary forms of renal cell carcinoma include von Hippel-Lindau syndrome (VHL 3p25 26),26 hereditary papillary renal cell carcinoma (MET 7q31 34),27 Birt-Hogg-Dubé syndrome (FLCN 17p11), 28 hereditary leiomyomatosis (FH 1q42 43), 29 and tuberous sclerosis (TSC1 9q34 or TSC2 16p13).30 Diagnosis

Genotype phenotype correlation in von Hippel‐Lindau disease

visual impairment in patients with von Hippel-Lindau (VHL) disease. Identifi-cation of genotype phenotype correlation is an important prerequisite for better management, treatment and prognosis. Methods: Retrospective, single-centre cohort study of 200 VHL patients. Genetic data and date of onset of RH, central nervous system haemangioblas-

Role of Von Hippel-Lindau gene mutation in Renal Cancer

management of this disease. Keywords: renal cell carcinoma. Von Hippel-Lindau gene, vascular endothelial growth factor. *Corresponding Author: Tapan Behl, Department of Pharmacology, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi. [email protected] 1. Introduction Von Hippel-Lindau Syndrome (VHL) is a

The role of VHL in clear-cell renal cell carcinoma and its

KEYWORDS: clear-cell renal cell carcinoma; targeted therapy; tyrosine kinase inhibitor; von Hippel Lindau There were an estimated 54,390 newly diagnosed cases of kidney cancer in 2008 and over 13,000 deaths.1 For many patients, the disease is localized when it is found, and for them, surgical excision remains the standard of care and

Imaging Features of von Hippel Lindau Disease

Abbreviations: CNS central nervous system, MIBG metaiodobenzylguanidine, RCC renal cell carcinoma, VHL von Hippel Lindau RadioGraphics 2008; 28:65 79 Published online 10.1148/rg.281075052 Content Code: 1From the Department of Radiology, Guy s and St Thomas Hospitals, St Thomas Street, London SE1 7EH, United Kingdom. Presented as an edu-

Metastatic Renal Cell Carcinoma Management

Results: Progress in understanding the molecular basis of renal cell carcinoma, especially related to genetics and angio-genesis, has been achieved mainly through of the study of von Hippel-Lindau disease. A great variety of active agents have been developed and tested in metastatic renal cell carcinoma (mRCC) patients.

ISSN: 2151-6200 Open Access Renal Cell Carcinoma and Genomics

Renal cell carcinoma (RCC), accounts for 2% to 3% of all adult malignancies, and is the most lethal of all the common urologic cancers.Approximately 73,750 new cases of RCC are diagnosed each year in the United States alone, and 14,830 patients are reported to die of the disease.

Advancing the Science and Management of Renal Cell Carcinoma

2. Renal Cell Carcinoma Incidence and Histology Renal cell carcinomas are a broad classification referring to tumors originating from the renal pelvis and medulla. The incidence of RCC is increasing, with an estimated 73,750 newly diagnosed cases in 2020 in the United States [10]. The disease now accounts for 5% of cancer diagnoses in

Von Hippel Lindau (VHL) disease: A case report

Von- Hippel Lindau is a rare disease which is inherited in autosomal dominant fashion and results in development of hemangioblastoma of central nervous system (CNS), retinal hemangioblastoma, renal cell carcinoma or renal cyst, neuroendocrine tumours and cyst of pancreatic gland, pheochromocytoma, epididymal cyst adenoma.

Von Hippel-Lindau Disease: An Overview Continuing Nursing

Von Hippel-Lindau disease is a rare, familial disease consisting of multiple tumors, which can present in the eyes, brain, adrenal gland, pancreas, liver, spinal cord, kid-neys, or other areas of the body. The renal tumors are primarily renal cell carcinoma. Renal involvement may be the primary manifestation, but most frequently it is a sec-

Ocular Findings in von Hippel Lindau Disease

Ocular Findings in von Hippel Lindau Disease RETINA OPHTHALMIC PEARLS V on Hippel Lindau (VHL) disease is a tumor syndrome that affects the central nervous system (CNS), retina, and visceral organs. Inherited in an autosomal dominant manner, it arises from germ-line mutations in the VHL gene.1 The syndrome is rare, with an incidence

Management of Locally Advanced Renal Cell Carcinoma

Mar 01, 2006 Management of Locally Advanced Renal Cell Carcinoma Alejandro Rodriguez, MD, and Wade J. Sexton, MD Background:Renal cell carcinoma accounts for approximately 3% of adult malignancies and over 90% of primary renal tumors. Recurrence rates for patients with locally advanced renal cell carcinoma (LARCC) remain high.

Tumors in von Hippel Lindau Syndrome: From Head to Toe

Tumors in von Hippel Lindau Syn-drome: From Head to Toe Com-prehensive State-of-the-Art Review1 Von Hippel Lindau syndrome (VHL) is an autosomal-dominant he-reditary tumor disease that arises owing to germline mutations in the VHL gene, located on the short arm of chromosome 3. Patients with

Precision Surgery and Kidney Cancer: Knowledge of Genetic

Feb 11, 2021 with a focus on the surgical management of these tumors. Keywords: renal cell carcinoma; kidney cancer; precision surgery; genetic alterations; Von Hippel-Lindau; hereditary leiomyomatosis and renal cell cancer; Birt-Hogg-Dubé 1. Introduction In the United States, renal cell carcinoma (RCC) diagnoses are expected to reach over

Oncology Agents, Renal Cell Carcinoma

RCC.15 There are also hereditary types of RCC; von Hippel-Lindau (VHL) disease predisposes patients to the development of kidney cancer.16 In the US, kidney cancer death rates are highest among American Indians/Alaska natives.17 Approximately 85% of all kidney tumors are RCC, and 70% of all RCCs have


extraocular clinical findings of von hippel-lindau disease VHL is an autosomal dominant neoplastic disorder in which multiple benign or malignant tumors and cysts with specific histopathologic features develop in the tissues of the central nervous system, including brain, spinal cord, inner ear, and retina, and in

Radiology and Von Hippel-Lindau Disease

Renal Cell Carcinoma (RCC) 30-40% of VHL patients 70% have renal cell carcinoma by age 60 Cancer of the kidney, risk of metastasis Treatment is surgical removal or ablation, typically when > 3cm Must balance risk of metastasis with accumulating loss of kidney function Dialysis 42% five year survival

Von Hippel-Lindau Disease Testing - eviCore

Von Hippel-Lindau (VHL) syndrome is a hereditary cancer syndrome whose main clinical features include hemangioblastomas of the central nervous system (CNS) and retina, renal cysts and renal cell carcinoma, pheochromocytoma, and

Kidney Cancer Early Detection, Diagnosis, and Staging

Nov 21, 2019 People who have certain inherited conditions2, such as von Hippel-Lindau disease, have a higher risk of kidney cancer. Doctors often recommend that these people get regular imaging tests such as CT, MRI, or ultrasound scans at younger ages, to look for kidney tumors. Kidney cancers that are found early with these tests can often be cured.

Renal cell carcinoma

The investigation and management of renal cell carci-noma (RCC) has undergone an unprecedented period of change. This is largely due to advances in genomics and biological discoveries that can be successfully targeted to benefit patients. Pivotal to these advances was the recog - nition of mutations of the von Hippel-Lindau gene, and

Management of advanced renal cancer

cancer syndromes. Von Hippel-Lindau disease (VHL) is an autosomal-dominant, multi-organ, familial can-cer syndrome characterized by the development of cerebellar/spinal hemangioblastomas, retinal angiomas, pheochromocytomas, and renal cysts/tumors. Renal cell cancer develops in 40% to 60% of VHL patients, and is

Understanding the Importance of Smart Drugs in Renal Cell

surgical management of renal cell carcinoma (RCC) that have occurred in the past 20 years [1], dramatic progress has been made in understanding the genetic basis of RCC. Specific genes responsible for von Hippel-Lindau disease (VHL), as well as for european urology 49 (2006) 633 643 available at

Systemic therapy in the management of localized and locally

therapy in localized and locally advanced renal cell carcinoma. Key words: adjuvant, immune checkpoint inhibitor, neoadjuvant, renal cell carcinoma, tyrosine kinase inhibitor. Introduction RCC is the 17th most common cancer, globally contributing 2.2% of the total number of all cancers diagnosed in 2018.

Hereditary Renal Tumor Syndromes: Imaging Findings and

propriate management and follow-up. von Hippel Lindau Syndrome The most common cause of heredi-tary RCC is von Hippel Lindau syndrome (VHL). It is an autosomal dominant disor-der of variable expressivity caused by a mu-tation in the VHL tumor suppressor gene on chromosome 3. It is not rare, with incidence of approximately 1 case/36,000 live

Gynecology and Reproductive Health Issues in von Hippel

Nov 01, 2015 Familial von Hippel-Lindau Disease Type 1 low risk of pheochromocytoma Retinal angioma, CNS hemangioblastoma, renal cell cancer, pancreatic cysts, and neuroendocrine tumors Type 2 high risk of pheochromocytoma Type 2A hemangioblastomas and pheochromocytoma Type 2B hemangioblastomas, pheochromocytoma and renal cell cancer

Risk factors for metachronous bilateral renal cell carcinoma

a genetic cancer syndrome such as Von Hippel-Lindau disease.4 Understanding these risks may determine appropriate surveillance following treatment and help guide therapy. Advocating for partial nephrectomy as management can be-come challenging if a contralateral tumor develops in a solitary kidney. In these instances, further surgical therapy may

Hereditary Renal Cancer Syndromes: An Update of a Systematic

Hereditary leiomyomatosis renal cell carcinoma Hereditary papillary renal cell carcinoma Kidney cancer Renal cell carcinoma Tuberous sclerosis complex von Hippel-Lindau disease Abstract Context: Hereditary renal cancers (HRCs) comprise approximately 3 5% of renal cell carcinomas (RCCs).

Imaging and Management of Intrathoracic Renal Cell Carcinoma

von Hippel Lindau tumor suppressor gene; in 10 20% of cases, the von Hippel Lindau gene is inactivated [12]. Histologically, clear cell RCC consists of cells with clear cyto-plasm, although eosinophilic cytoplasm may also be present [11]. Papillary RCC is the second most preva-lent carcinoma of the renal tubular epithe-

Von Hippel-Lindau Disease Testing - eviCore

Von Hippel-Lindau (VHL) syndrome is a hereditary cancer syndrome whose main clinical features include hemangioblastomas of the central nervous system (CNS) and retina, renal cysts and renal cell carcinoma, pheochromocytoma, and

Manejo interdisciplinario del tumor renal en la enfermedad de

with Von Hippel-Lindau disease, evaluated by the Von Hippel-Lindau disease interdisciplinary management group of a Latin American hospital, noting the management and behavior of renal cell carcinoma associated with this syndrome. Materials and methods: A retrospective observational descriptive study was conducted.

Renal Cell Carcinoma: Recent Advances in Genetics and Imaging

Renal cell carcinoma is now known to be a polymorphic malignant neoplasm consisting of several histologic subtypes demonstrating different biological profiles. Clear cell renal carcinoma, the most common histologic subtype, is predominantly associated with mutations involving the von Hippel-Lindau gene and elaboration of vascular and somatic

Managing Renal Cell Carcinoma Associated Paraneoplastic

Oncology Managing Renal Cell Carcinoma Associated Paraneoplastic Syndrome with Nephron-sparing Surgery in a Patient with von Hippel-Lindau John M. DiBiancoa, Julie Y.Anb,*, Sally Tanakchic, Zachary Stanikb, Aidan McGowanb,

Ashouri et al. Von Hippel-Lindau and Renal Cell Carcinoma

treatments, and prognosis of renal tumors in this disease. Data for this review was acquired using combinations of the following terms: von Hippel-Lindau syndrome, von Hippel-Lindau disease, von Hippel-Lindau, renal cell carcinoma, radiology, screening, treatment, nephrectomy, VHL, VHL gene, HIF, metastatic, nephron sparing surgery, ablation.


progenitors in von Hippel-Lindau disease and renal cell carcinoma. British Journal of Cancer 2011, 105: 112 117 4. Jilg C, Neumann H, Glasker S, Schafe O, Ardelt P, Schwardt M, et al. Growth Kinetics in Von Hippel-Lindau-Associated Renal Cell Carcinoma. Urologia Internationalis 2012, 88: 71 78 5. Maher Eamonn R, Neumann Hartmut

Survivorship Kidney Cancer Page 1 of 5

1 Von Hippel-Lindau disease (VHL) is a hereditary condition associated with tumors arising in multiple organs 2 Category 1: Pathologic T1a, T1b (tumor less than or equal to 7 cm) limited to kidney; transition to survivorship 3 years after completion of treatment and NED

Pazopanib in the treatment of renal cell carcinoma

cell renal cell carcinoma (RCC) [1]. Increasing knowledge of RCC molecular biology over the last two decades has given insight into the molecular pathogenesis that drives tumor angiogenesis in this deadly cancer [2]. In the 1990s, the tumor-suppressor gene, von Hippel Lindau (VHL), was found to be frequently mutated or silenced in sporadic

Renal Cell Carcinoma: Diagnosis and Management

Feb 01, 2019 of these is von Hippel-Lindau disease which leads to the development of clear cell renal cell carcinoma through the activation of vascular endothelial growth factor (VEGF).

Von Hippel-Lindau disease - The Lancet

management of haemangioblastomas in the CNS, renal cell carcinoma tends to be the major cause of death and von Hippel-Lindau disease is now recognised as the main cause of adult inherited renal cancer.2 The disease is caused by germline mutations in the VHL tumour-suppressor gene; the same gene is