Spinal hemangioblastoma containing metastatic renal cell carcinoma in von Hippel—Lindau disease

2005 ◽  
Vol 3 (6) ◽  
pp. 495-500 ◽  
Author(s):  
Meric A. Altinoz ◽  
Carlo Santaguida ◽  
Marie-Christine Guiot ◽  
Rolando F. Del Maestro
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Metastatic Renal Cell Carcinoma ◽  
Renal Cell ◽  
Electron Microscopic ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Spinal Hemangioblastoma ◽  
Vhl Disease ◽  
Fine Print

✓ The authors describe the case of a patient with von Hippel—Lindau (VHL) disease in which a spinal hemangioblastoma contained metastatic renal cell carcinoma (RCC). The literature on tumor-to-tumor metastasis associated with VHL disease of the central nervous system (CNS) is reviewed. Midthoracic back pain developed in this 43-year-old man with a left-sided radicular component 2 years after he underwent resection of a left RCC. Radiological findings demonstrated a T6–7 intradural intramedullary lesion. A T5–8 laminectomy and gross-total resection of the spinal cord mass were performed. Light and electron microscopic examination showed features of hemangioblastoma, which contained metastatic foci of RCC. Genetic analysis demonstrated the presence of a deleting mutation in the first exon (nt. 394–406) of the VHL locus, truncating 16 amino acids (N61–77) from the first beta sheet in the VHL protein. A review of the literature revealed that RCC-to-CNS hemangioblastoma is the second most common donor—recipient tumor association among the tumor-to-tumor metastases.

Gamma knife radiosurgery for renal cell carcinoma brain metastases

2002 ◽  
Vol 97 ◽  
pp. 489-493 ◽  
Author(s):  
Laura Hernandez ◽  
Lucia Zamorano ◽  
Andrew Sloan ◽  
James Fontanesi ◽  
Simon Lo ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Brain Metastases ◽  
Cell Carcinoma ◽  
Gamma Knife ◽  
Median Survival ◽  
Renal Cell ◽  
Univariate Analysis ◽  
Gamma Knife Radiosurgery ◽  
Content Type ◽  
Fine Print

Object. The purpose of this study was to clarify the effectiveness of gamma knife radiosurgery (GKS) in achieving a partial or complete remission of so-called radioresistant metastases from renal cell carcinoma (RCC) and to propose guidelines for optimal treatment Methods. During a 5-year period, 29 patients (19 male and 10 female) with 92 brain metastases from RCC underwent GKS. The median tumor volume was 4.7 cm3 (range 0.5–14.5 cm3). Fourteen patients (48%) also underwent whole-brain radiotherapy (WBRT) before GKS, and two patients (6.8%) after GKS. The mean GKS dose delivered to the 50% isodose at the tumor margin was 16.8 Gy (range 13–30 Gy). All cases were categorized according to the Recursive Partitioning Analysis (RPA) classification for brain metastases. Univariate analysis was performed to determine significant prognostic factors and survival. The overall median survival was 7 months after GKS treatment. Age, sex, Karnofsky Performance Scale score, and controlled primary disease were not predictors of survival. Combined WBRT/GKS resulted in median survival of 18, 8.5, and 5.3 months for RPA Classes I, II, and III, respectively, compared with the median survival 7.1, 4.2, and 2.3 months for patients treated with WBRT alone. Conclusions. These results suggest that WBRT combined with GKS may improve survival in patients with brain metastases from RCC. Furthermore, this improvement in survival was seen in all RPA classes.

scholarly journals Hereditary renal cell carcinoma associated with von Hippel–Lindau disease: a description of a Nova Scotia cohort

2013 ◽  
Vol 3 (1) ◽  
pp. 32 ◽  
Author(s):  
Shannon Bradley ◽  
Nadine Dumas ◽  
Mark Ludman ◽  
Lori Wood
Keyword(s):  
Renal Cell Carcinoma ◽  
Nova Scotia ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Prise En Charge ◽  
Mortality Data ◽  
Malignant Tumours ◽  
Multiple Sources ◽  
Von Hippel Lindau ◽  
Vhl Disease

Background: von Hippel–Lindau (VHL) disease is an autosomaldominant condition characterized by the development of benignand malignant tumours, including cases of renal cell carcinoma(RCC). Early detection of RCC through routine surveillance canlead to decreased morbidity and mortality. Data on the numberof patients in Nova Scotia (NS) who have VHL disease, diseasemanifestations and the frequency and mode of the surveillancehave not previously been collected or reported. This project wasdesigned to obtain that information.Methods: The number and management of patients with VHL diseasewas determined by multiple sources: the Maritime MedicalGenetics Service, patient charts, and pathology, radiology and laboratorydata. The actual surveillance being performed was comparedwith that recommended in the literature.Results: Twenty-one patients from 11 families in NS were identified.Manifestations included cases of RCC (31.6%), central nervoussystem (CNS) hemangioblastoma (73.7%), retinal hemangioma(47.4%), renal cyst (47.4%) and pheochromocytoma (10.5%).Of the 6 patients with RCC, 4 had bilateral tumours, 2 requiredkidney transplants and 1 developed metastatic disease. Routinesurveillance was being done for the CNS in 62.5% of patients,retina in 47.4%, abdomen in 43.8% and urine catecholaminesin only 10.5%. Only 1 of the 6 patients who developed RCCwas undergoing routine abdominal imaging. Surveillance investigationswere ordered by a number of different specialists.Conclusion: Patients with VHL disease in NS have a number of manifestationsassociated with their disease, including RCC, in a similarfrequency to that reported in the literature. The surveillanceof these patients is suboptimal in frequency and coordination.von Hippel–Lindau disease is a complex condition that requiresa coordinated approach to care to ensure proper surveillance andtreatment. Our study highlights current deficiencies and offersan enormous opportunity for improvement.Généralités : La maladie de von Hippel-Lindau (VHL) est une maladieà transmission autosomique dominante caractérisée par la formationde tumeurs bénignes et malignes, dont l’hypernéphrome.Le dépistage précoce de l’hypernéphrome par des examens régulierspeut amener une réduction de la morbidité et de la mortalité. Onne sait pas combien de personnes sont atteintes de VHL enNouvelle-Écosse, quelles sont les manifestations de la maladie chezces patients et quels tests de dépistage sont effectués et à quellefréquence. Le projet décrit ici visait à obtenir ces renseignements.Méthodologie : Le nombre et la méthode de prise en charge despatients atteints de VHL ont été établis à l’aide de plusieurs sources :la Clinique de génétique médicale des Maritimes, des dossiers depatients, des rapports de pathologie et de radiologie et des analysesde laboratoire. Les méthodes de surveillance mises en placeont été comparées aux méthodes recommandées dans la littératuremédicale.Résultats : Vingt et un patients de 11 familles de Nouvelle-Écosseont été cernés. Les manifestations incluaient : hypernéphrome(31,6 %), hémangioblastomes siégeant au niveau du SNC (73,7 %),hémangiomes rétiniens (47,4 %), kystes rénaux (47,4 %) etphéochromocytomes (10,5 %). Sur les six patients porteurs d’unhypernéphrome, 4 avaient des tumeurs bilatérales, 2 ont eu besoind’une transplantation rénale et un patient a présenté des métastases.De tous les patients atteints de VHL, 62,5 % ont subi destests réguliers de dépistage au niveau du SNC, 47,4 %, au niveaude la rétine, 43,8 %, au niveau de l’abdomen, et seulement 10,5 %des patients ont subi des tests réguliers de dépistage des catécholaminesurinaires. Sur les 6 cas d’hypernéphrome, un seulementsubissait des épreuves régulières d’imagerie au niveau del’abdomen. Les tests de dépistage avaient été prescrits par différentsspécialistes.Conclusion : Les cas de VHL en Nouvelle-Écosse présentent un certainnombre de manifestations liées à cette maladie, dont l’hypernéphrome,à une fréquence proche de celle mentionnée dansla littérature. La fréquence et la coordination des épreuves dedépistage sont sous-optimales. La maladie de VHL est une affectioncomplexe nécessitant une bonne coordination des soinsafin d’assurer une surveillance et un traitement adéquats. Cetteétude montre les lacunes actuelles et pointe vers des améliorationssubstantielles.

Repeated gamma knife surgery for multiple brain metastases from renal cell carcinoma

2002 ◽  
Vol 97 (4) ◽  
pp. 785-793 ◽  
Author(s):  
Berndt Wowra ◽  
Michael Siebels ◽  
Alexander Muacevic ◽  
Friedrich Wilhelm Kreth ◽  
Andreas Mack ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Brain Metastases ◽  
Cell Carcinoma ◽  
Gamma Knife ◽  
Renal Cell ◽  
Radiation Toxicity ◽  
Content Type ◽  
Multiple Brain Metastases ◽  
Fine Print

Object. The aim of this study was to evaluate the therapeutic profile of repeated gamma knife surgery (GKS) for renal cell carcinoma that has metastasized to the brain on multiple occasions. Methods. Data from this study were culled from a single institution and cover a 6-year period of outpatient radiosurgery. A standard protocol for indication, dose planning, and follow up was established. In cases of distant or local recurrences, radiosurgery was undertaken repeatedly (up to six times in one individual). Seventy-five patients harboring 350 cerebral metastases were treated. Relief from pretreatment neurological symptoms occurred in 72% of patients within a few days or a few weeks after the procedure. The actuarial local tumor control rate after the initial GKS was 95%. In patients free from relapse of intracranial metastases after repeated radiosurgery, long-term survival was 91% after 4 years; median survival was 11.1 ± 3.2 months after radiosurgery and 4.5 ± 1.1 years after diagnosis of the primary kidney cancer. Survival following radiosurgery was independent of patient age and sex, side of the renal cell carcinoma, pretreatment of the cerebrum by using radiotherapy or surgery, number of brain metastases and their synchronization with the primary renal cell carcinoma, and the frequency of radiosurgical procedures. In contrast, survival was dependent on the patient's clinical performance score and the extracranial tumor status. Tumor bleeding was observed in seven patients (9%) and late radiation toxicity (LRT) in 15 patients (20%). Treatment-related morbidity was moderate and mostly transient. Late radiation toxicity was encountered predominantly in long-term survivors. Conclusions. Outpatient repeated radiosurgery is an effective and only minimally invasive treatment for multiple brain metastases from renal cell cancer and is recommended as being the method of choice to control intracranial disease, especially in selected patients with limited extracranial disease. Physicians dealing with such patients should be aware of the characteristic aspects of LRT.

Radiosurgery in patients with renal cell carcinoma metastasis to the brain: long-term outcomes and prognostic factors influencing survival and local tumor control

2003 ◽  
Vol 98 (2) ◽  
pp. 342-349 ◽  
Author(s):  
Jason P. Sheehan ◽  
Ming-Hsi Sun ◽  
Douglas Kondziolka ◽  
John Flickinger ◽  
L. Dade Lunsford
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Cell Cancer ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Content Type ◽  
Extracranial Disease ◽  
The Brain ◽  
Fine Print

Object. Renal cell carcinoma is a leading cause of death from cancer and its incidence is increasing. In many patients with renal cell cancer, metastasis to the brain develops at some time during the course of the disease. Corticosteroid therapy, radiotherapy, and resection have been the mainstays of treatment. Nonetheless, the median survival in patients with renal cell carcinoma metastasis is approximately 3 to 6 months. In this study the authors examined the efficacy of gamma knife surgery in treating renal cell carcinoma metastases to the brain and evaluated factors affecting long-term survival. Methods. The authors conducted a retrospective review of 69 patients undergoing stereotactic radiosurgery for a total of 146 renal cell cancer metastases. Clinical and radiographic data encompassing a 14-year treatment interval were collected. Multivariate analyses were used to determine significant prognostic factors influencing survival. The overall median length of survival was 15 months (range 1–65 months) from the diagnosis of brain metastasis. After radiosurgery, the median survival was 13 months in patients without and 5 months in those with active extracranial disease. In a multivariate analysis, factors significantly affecting the rate of survival included the following: 1) younger patient age (p = 0.0076); 2) preoperative Karnofsky Performance Scale score (p = 0.0012); 3) time from initial cancer diagnosis to brain metastasis diagnosis (p = 0.0017); 4) treatment dose to the tumor margin (p = 0.0252); 5) maximal treatment dose (p = 0.0127); and 6) treatment isodose (p = 0.0354). Prior tumor resection, chemotherapy, immunotherapy, or whole-brain radiation therapy did not correlate with extended survival. Postradiosurgical imaging of the brain demonstrated that 63% of the metastases had decreased, 33% remained stable, and 4% eventually increased in size. Two patients (2.9%) later underwent a craniotomy and resection for a tumor refractory to radiosurgery or a new symptomatic metastasis. Eighty-three percent of patients died of progression of extracranial disease. Conclusions. Stereotactic radiosurgery for treatment of renal cell carcinoma metastases to the brain provides effective local tumor control in approximately 96% of patients and a median length of survival of 15 months. Early detection of brain metastases, aggressive treatment of systemic disease, and a therapeutic strategy including radiosurgery can offer patients an extended survival.

scholarly journals Von Hippel-Lindau Tumor; Radiographic Images

2017 ◽  
Vol 2 (1) ◽  
pp. 30
Author(s):  
Reza Bidaki ◽  
Azam Ghanei ◽  
Seyed Mehdi Hosseinizade ◽  
Mohammad Ebrahim Ghanei
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Renal Cysts ◽  
Cell Renal Cell Carcinoma ◽  
Radiographic Images ◽  
Von Hippel Lindau ◽  
Malignant Lesions ◽  
Vhl Disease ◽  
Renal Computed Tomography

The patient is a 34-year-old patient with abdominal pain, gross hematuria with anxiety and worries about it from 5 months ago. The physician requested renal computed tomography (CT) without and then with contrast for rule out of renal stone. However, he found multiple lesions in kidneys. The laboratory tests were normal except hematuria. He was a candidate for surgery. The pathologist reported clear red cell renal cell carcinoma. He was referred to a radiologist for staging. Von Hippel – Lindau (VHL) disease is an inherited and rare disease that is characterized by a variety of benign and malignant lesions (1). It preval ence is 1 in 31,000 -53,000 (2,3). Previous studies shown 59 – 63% of patients have renal cysts and 24 - 45 % renal cell carcinoma (4), and in 75 % of cases ,the lesions are bilateral (4, 5). Involvement of pancreas includes simple cysts (50 – 91%), serous m icrocystic adenomas (12%) and adenocarcinoma (7%) (2, 4).

Stereotactic radiosurgery for spinal metastases from renal cell carcinoma

2005 ◽  
Vol 3 (4) ◽  
pp. 288-295 ◽  
Author(s):  
Peter C. Gerszten ◽  
Steven A. Burton ◽  
Cihat Ozhasoglu ◽  
William J. Vogel ◽  
William C. Welch ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Tumor Progression ◽  
Cell Carcinoma ◽  
Stereotactic Radiosurgery ◽  
Renal Cell ◽  
Spinal Metastases ◽  
External Beam Radiation ◽  
Content Type ◽  
Single Fraction ◽  
Fine Print

Object. The role of stereotactic radiosurgery in treating renal cell carcinoma (RCC) metastases to the spine has previously been limited. In this study the authors evaluated the clinical outcome in patients with spinal RCC who underwent single-fraction radiosurgery. Methods. Forty-eight patients with 60 RCC metastases to the spine (six cervical, 26 thoracic, 18 lumbar, and 10 sacral) were treated with a single-fraction radiosurgery technique and were followed for a period of 14 to 48 months (median 37 months). All patients were successfully treated in an outpatient setting. The tumor volume ranged from 5.5 to 203 cm3 (mean 61.9 cm3). Forty-two of the total 60 lesions had been previously treated with external-beam radiation therapy (EBRT). The maximum tumor dose was maintained at 17.5 to 25 Gy (mean 20 Gy). The volume of the spinal cord exposed to greater than 8 Gy ranged from 0.01 to 3 cm3 (mean 0.64 cm3); the volume of the spinal canal at the cauda equina level exposed to greater than 8 Gy ranged from 0.01 to 2.2 cm3 (mean 0.65 cm3). No radiation-induced toxicity occurred during the follow-up period. Axial and radicular pain improved in 34 (89%) of 38 patients who were treated primarily for pain. Tumor control was demonstrated in seven of eight patients treated primarily for radiographically documented tumor progression. In time six patients required open surgical intervention for tumor progression that had caused neurological dysfunction after radiosurgery. Conclusions. Spinal radiosurgery can be a successful therapeutic modality for the delivery of large-dose single-fraction radiation to RCC spinal metastases that are often poorly controlled with conventional EBRT modalities.

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