Hemangioblastomas of the central nervous system

1989 ◽  
Vol 70 (1) ◽  
pp. 24-30 ◽  
Author(s):  
Hartmut P. H. Neumann ◽  
Hans R. Eggert ◽  
Klaus Weigel ◽  
Hartmut Friedburg ◽  
Otmar D. Wiestler ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Screening Program ◽  
Family Background ◽  
Renal Cysts ◽  
Pancreatic Cysts ◽  
Content Type ◽  
Von Hippel Lindau ◽  
The Central Nervous System ◽  
Von Hippel Lindau Syndrome

✓ The findings of a 10-year study (1976 to 1986) conducted in southwest Germany on hemangioblastomas (HBL's) of the central nervous system (CNS) are presented. During that period, 47 HBL's were diagnosed and surgically removed in 44 patients, with a good postoperative survival rate and prognosis. The majority (83%) of these tumors were located in the cerebellum. By thorough clinical examination of the patients and careful evaluation of their family background, it was found that 23% of the HBL patients were afflicted with von Hippel-Lindau syndrome. In addition to the CNS tumors, 14 neoplastic or similar lesions were detected in other tissues. These included angiomatosis of the retinae, pheochromocytomas, pancreatic cysts, renal cysts, and renal carcinoma. The diagnosis of von Hippel-Lindau syndrome was thus established in seven families. The authors suggest the need for a screening program for patients with HBL of the CNS which is designed to confirm or exclude ocular or visceral lesions associated with von Hippel-Lindau syndrome.

Hemangioblastomas of the Central Nervous System in von Hippel-Lindau Syndrome and Sporadic Disease

Neurosurgery ◽  
2001 ◽  
Vol 48 (1) ◽  
pp. 55-63 ◽  
Author(s):  
James E. Conway ◽  
Dean Chou ◽  
Richard E. Clatterbuck ◽  
Henry Brem ◽  
Donlin M. Long ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Von Hippel Lindau ◽  
The Central Nervous System ◽  
Sporadic Disease ◽  
Von Hippel Lindau Syndrome

Haemangioblastoma

2019 ◽  
pp. 367-374
Author(s):  
Donald McArthur ◽  
Ammar Natalwala
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Optimal Timing ◽  
Significant Morbidity ◽  
Rapid Disease Progression ◽  
Von Hippel Lindau ◽  
Complete Surgical Resection ◽  
The Central Nervous System ◽  
Benign Tumours ◽  
Von Hippel Lindau Syndrome

Haemangioblastomas are rare and benign tumours which favour the infratentorial structures in the central nervous system. Around 30% of these tumours are associated with Von Hippel-Lindau syndrome, which if the diagnosis is missed can lead to rapid disease progression and significant morbidity and mortality. Complete surgical resection is the main treatment but other techniques including stereotactic radiosurgery are described in this chapter. Screening for Von Hippel-Lindau (VHL), the optimal timing, and mode of treatment remain controversial due to the diversity of clinical presentation in these patients. This chapter also outlines the general principles of managing patients with haemangioblastomas and the factors which impact on prognosis.

scholarly journals Haemangioblastomas of the central nervous system in von Hippel Lindau Syndrome involving cerebellum and spinal cord

2018 ◽  
Vol 32 (1) ◽  
pp. 164-167
Author(s):  
V.A. Kiran Kumar ◽  
Vissa Shanti ◽  
Yaswanth Sandeep ◽  
Jatinder Kumar Mittal ◽  
Amit Agrawal
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Von Hippel Lindau ◽  
The Central Nervous System ◽  
Von Hippel Lindau Syndrome

Clinical and molecular analysis of disseminated hemangioblastomatosis of the central nervous system in patients without von Hippel—Lindau disease

2002 ◽  
Vol 96 (4) ◽  
pp. 775-787 ◽  
Author(s):  
Robert J. Weil ◽  
Alexander O. Vortmeyer ◽  
Zhengping Zhuang ◽  
Svetlana D. Pack ◽  
Nicholas Theodore ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Family History ◽  
Complete Resection ◽  
Comparative Genomic ◽  
Content Type ◽  
Cerebellar Hemangioblastoma ◽  
Von Hippel Lindau ◽  
The Central Nervous System ◽  
Fine Print

✓ Hemangioblastomas of the central nervous system (CNS) may occur sporadically or in association with von Hippel—Lindau (VHL) syndrome. The authors present four patients with no family history or clinical evidence of VHL syndrome in whom extensive, progressive, en plaque coating of the brainstem and spinal cord with hemangioblastomas developed 1 to 8 years after complete resection of a solitary cerebellar hemangioblastoma. Analysis included detailed physical, biochemical, radiological, and pathological examinations in all four patients, combined with family pedigree analysis. In addition, a detailed investigation of the VHL gene was undertaken. Allelic loss, comparative genomic hybridization (CGH), single-stranded conformational polymorphism screening, CpG island methylation status, and X chromosome inactivation clonality analyses were performed. Although there was no evidence of germline alterations in the VHL gene on clinical and radiological examination or in the family history (all four patients) or analysis of peripheral blood (three patients), somatic deletion of one copy of the VHL gene occurred in these tumors. These findings indicate that the multiple, separate deposits of tumors were likely derived from a single clone. Results of CGH indicate that one or several additional genes are probably involved in the malignant behavior of the hemangioblastomas in these patients. Furthermore, the malignant biological and clinical behavior of these tumors, in which multiple sites of subarachnoid dissemination developed 1 to 8 years after initial complete resection, followed by progressive tumor growth and death of the patients, occurred despite a histological appearance typical of benign hemangioblastomas. Malignant hemangioblastomatosis developed 1 to 8 years after resection of an isolated cerebellar hemangioblastoma. Alterations of the VHL gene may be permissive in this setting, but other genes are likely to be the source of the novel biological and clinical presentation of the disseminated hemangioblastomas in these patients. This appears to represent a novel condition in which the product of one or more mutations in several genes permits malignant tumor behavior despite retention of a benign histological picture, a circumstance previously not recognized in CNS tumors.

scholarly journals Neurons Are Host Cells for Mycobacterium tuberculosis

2014 ◽  
Vol 82 (5) ◽  
pp. 1880-1890 ◽  
Author(s):  
Philippa J. Randall ◽  
Nai-Jen Hsu ◽  
Dirk Lang ◽  
Susan Cooper ◽  
Boipelo Sebesho ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Mycobacterium Tuberculosis ◽  
Host Cells ◽  
Productive Infection ◽  
Target Cells ◽  
Dependent Manner ◽  
Content Type ◽  
The Central Nervous System

ABSTRACTMycobacterium tuberculosisinfection of the central nervous system is thought to be initiated once the bacilli have breached the blood brain barrier and are phagocytosed, primarily by microglial cells. In this study, the interactions ofM. tuberculosiswith neuronsin vitroandin vivowere investigated. The data obtained demonstrate that neurons can act as host cells forM. tuberculosis.M. tuberculosisbacilli were internalized by murine neuronal cultured cells in a time-dependent manner after exposure, with superior uptake by HT22 cells compared to Neuro-2a cells (17.7% versus 9.8%). Internalization ofM. tuberculosisbacilli by human SK-N-SH cultured neurons suggested the clinical relevance of the findings. Moreover, primary murine hippocampus-derived neuronal cultures could similarly internalizeM. tuberculosis. InternalizedM. tuberculosisbacilli represented a productive infection with retention of bacterial viability and replicative potential, increasing 2- to 4-fold within 48 h.M. tuberculosisbacillus infection of neurons was confirmedin vivoin the brains of C57BL/6 mice after intracerebral challenge. This study, therefore, demonstrates neurons as potential new target cells forM. tuberculosiswithin the central nervous system.

scholarly journals Adaptation of Cryptococcus neoformans to Mammalian Hosts: Integrated Regulation of Metabolism and Virulence

2011 ◽  
Vol 11 (2) ◽  
pp. 109-118 ◽  
Author(s):  
Jim Kronstad ◽  
Sanjay Saikia ◽  
Erik David Nielson ◽  
Matthias Kretschmer ◽  
Wonhee Jung ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cryptococcus Neoformans ◽  
Central Carbon Metabolism ◽  
Yeast Cells ◽  
Phagocytic Cells ◽  
Content Type ◽  
The Central Nervous System ◽  
Mechanisms Of Adaptation ◽  
Complex Integration

ABSTRACTThe basidiomycete fungusCryptococcus neoformansinfects humans via inhalation of desiccated yeast cells or spores from the environment. In the absence of effective immune containment, the initial pulmonary infection often spreads to the central nervous system to result in meningoencephalitis. The fungus must therefore make the transition from the environment to different mammalian niches that include the intracellular locale of phagocytic cells and extracellular sites in the lung, bloodstream, and central nervous system. Recent studies provide insights into mechanisms of adaptation during this transition that include the expression of antiphagocytic functions, the remodeling of central carbon metabolism, the expression of specific nutrient acquisition systems, and the response to hypoxia. Specific transcription factors regulate these functions as well as the expression of one or more of the major known virulence factors ofC. neoformans. Therefore, virulence factor expression is to a large extent embedded in the regulation of a variety of functions needed for growth in mammalian hosts. In this regard, the complex integration of these processes is reminiscent of the master regulators of virulence in bacterial pathogens.

Cerebral sparganosis

1990 ◽  
Vol 73 (1) ◽  
pp. 147-150 ◽  
Author(s):  
Andrew Mitchell ◽  
Bernd W. Scheithauer ◽  
Patrick J. Kelly ◽  
Glenn S. Forbes ◽  
Jon E. Rosenblatt
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Surgical Removal ◽  
Asian Countries ◽  
Content Type ◽  
Chronic Abscess ◽  
The Central Nervous System ◽  
Cerebral Sparganosis ◽  
Tapeworm Infection ◽  
Fine Print

✓ The tapeworm Spirometra mansonoides infects man worldwide, particularly in Asian countries. Rarely, the central nervous system is involved; such a case is presented here. In the total of 12 reported cases, including the case described, the worm presented clinically as a mass suspicious for neoplasm or chronic abscess cavity. Surgical removal was invariably curative in each case. Although infrequent, the possibility of tapeworm infection should be entertained in the evaluation of intracranial masses in patients who have visited exotic locales.

Central nervous system mesenchymal chondrosarcoma

1984 ◽  
Vol 61 (2) ◽  
pp. 375-381 ◽  
Author(s):  
Griffith R. Harsh ◽  
Charles B. Wilson
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Vascular Tumor ◽  
Mesenchymal Chondrosarcoma ◽  
Content Type ◽  
Meningeal Tumor ◽  
The Central Nervous System ◽  
Initial Resection ◽  
Mesenchymal Chondrosarcomas ◽  
Fine Print

✓ Local recurrence developed 6 years after the initial resection of an intraspinal meningeal tumor that originally was thought to be an angioblastic meningioma. Histological review of the pathology led to a change of that diagnosis to one of mesenchymal chondrosarcoma. The recurrent vascular tumor was embolized, then totally excised. Because this tumor had malignant features, the patient received irradiation and chemotherapy. No evidence of regrowth has been observed during a period of more than 4 years. Mesenchymal chondrosarcomas of the central nervous system and their treatment are reviewed.

Central nervous system leiomyosarcoma in patients with acquired immunodeficiency syndrome

2000 ◽  
Vol 92 (4) ◽  
pp. 688-692 ◽  
Author(s):  
Ann M. Ritter ◽  
Barbara H. Amaker ◽  
R. Scott Graham ◽  
William C. Broaddus ◽  
John D. Ward
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Acquired Immunodeficiency Syndrome ◽  
Content Type ◽  
Barr Virus ◽  
The Central Nervous System ◽  
Histological Characteristics ◽  
Acquired Immunodeficiency ◽  
Epstein Barr ◽  
Immunodeficiency Syndrome

✓ Leiomyosarcomas (LMSs) of the central nervous system are extremely rare; however, they are becoming more prevalent in immunocompromised patients. The authors present the cases of two patients with acquired immunodeficiency syndrome: one with LMS of the thoracic vertebral body and the other with LMS originating from the region of the cavernous sinus. The epidemiological and histological characteristics of LMS and its association with latent Epstein—Barr virus are discussed, as well as the treatments for this neoplasm.

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