scholarly journals Optic pathway gliomas associated with neurofibromatosis type I in children

2019 ◽  
Vol 18 (4) ◽  
pp. 28-38
Author(s):  
E. F. Valiakhmetova ◽  
N. A. Mazerkina ◽  
O. A. Medvedeva ◽  
Y. Y. Trunin ◽  
E. M. Tarasova ◽  
...  
Keyword(s):  
Survival Rate ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Low Grade ◽  
Type I ◽  
Optic Pathway ◽  
Neurofibromatosis Type I ◽  
National Medical ◽  
Optic Pathway Gliomas ◽  
Visual Disturbances

Neurofibromatosis type I (NFI) is one of the most common brain tumor predisposition syndromes. Children with NFI are prone to develop a low grade gliomas, which can be localized in various areas of the brain, however, most of them occur in the structures of the optic pathway: optic nerves, chiasm, tracts and optic radiations – that is, are optic pathway gliomas (OPG). This retrospective study included children with newly diagnosied low grade glioma of the optic pathway at the age from 0 to 18 years with NFI, who underwent medical examination and / or treatment at the Burdenko Neurosurgery Institute from January 1, 2003 till December 31,2015. Atotal from 264 patients 42 (16%) had clinical manifestations of NFI. The ratio of boys and girls was 1:1. The median age was 4.25 years (range 4.5 months – 17 years). Visual disturbances were the most frequent clinical manifestation of the tumor. Surgical resection was performed in 18 patients. The remaining 24 patients OPG were diagnosed based on clinical and radiological findings: 9 patients were in observation group, 11 patients chemotherapy was carried out, three were given radiation therapy, and spontaneous regression of the tumor was recorded in 1 patient. Progression of the disease was observed in 14 patients in our cohort. The overall survival rate in patients with NFI was 98 ± 2% at 5 years. Event free survival rate was 68 ± 7% at 5 years.The study was approved by the Independent Ethics Committee of N.N. Burdenko National Medical Research Center of neurosurgery Ministry of healthcare ofRussian Federation.

scholarly journals Optic pathway gliomas associated with neurofibromatosis type I in children

2019 ◽  
Vol 18 (4) ◽  
pp. 29-38
Author(s):  
E. F. Valiakhmetova ◽  
N. A. Mazerkina ◽  
O. A. Medvedeva ◽  
Y. Y. Trunin ◽  
E. M. Tarasova ◽  
...  
Keyword(s):  
Survival Rate ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Low Grade ◽  
Type I ◽  
Optic Pathway ◽  
Neurofibromatosis Type I ◽  
National Medical ◽  
Optic Pathway Gliomas ◽  
Visual Disturbances

Neurofibromatosis type I (NFI) is one of the most common brain tumor predisposition syndromes. Children with NFI are prone to develop a low grade gliomas, which can be localized in various areas of the brain, however, most of them occur in the structures of the optic pathway: optic nerves, chiasm, tracts and optic radiations – that is, are optic pathway gliomas (OPG). This retrospective study included children with newly diagnosied low grade glioma of the optic pathway at the age from 0 to 18 years with NFI, who underwent medical examination and / or treatment at the Burdenko Neurosurgery Institute from January 1, 2003 till December 31,2015. Atotal from 264 patients 42 (16%) had clinical manifestations of NFI. The ratio of boys and girls was 1:1. The median age was 4.25 years (range 4.5 months – 17 years). Visual disturbances were the most frequent clinical manifestation of the tumor. Surgical resection was performed in 18 patients. The remaining 24 patients OPG were diagnosed based on clinical and radiological findings: 9 patients were in observation group, 11 patients chemotherapy was carried out, three were given radiation therapy, and spontaneous regression of the tumor was recorded in 1 patient. Progression of the disease was observed in 14 patients in our cohort. The overall survival rate in patients with NFI was 98 ± 2% at 5 years. Event free survival rate was 68 ± 7% at 5 years.The study was approved by the Independent Ethics Committee of N.N. Burdenko National Medical Research Center of neurosurgery Ministry of healthcare ofRussian Federation.

Vision Outcomes for Pediatric Patients With Optic Pathway Gliomas Associated With Neurofibromatosis Type I

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Vineeth M. Thirunavu ◽  
Laila M. Mohammad ◽  
Viswajit Kandula ◽  
Molly Beestrum ◽  
Sandi K. Lam
Keyword(s):  
Pediatric Patients ◽  
Neurofibromatosis Type ◽  
Type I ◽  
Optic Pathway ◽  
Neurofibromatosis Type I ◽  
Optic Pathway Gliomas

Low grade fibromyxoid sarcoma and neurofibromatosis type I

2008 ◽  
Vol 50 (4) ◽  
pp. 938-938 ◽  
Author(s):  
Vasiliki Vlacha ◽  
Maria Repanti
Keyword(s):  
Neurofibromatosis Type ◽  
Low Grade ◽  
Type I ◽  
Neurofibromatosis Type I ◽  
Fibromyxoid Sarcoma

Optic pathway gliomas: a review

2007 ◽  
Vol 23 (5) ◽  
pp. E2 ◽  
Author(s):  
Mandy J. Binning ◽  
James K. Liu ◽  
John R. W. Kestle ◽  
Douglas L. Brockmeyer ◽  
Marion L. Walker
Keyword(s):  
Progressive Disease ◽  
Clinical Course ◽  
Mass Effect ◽  
Neurofibromatosis Type ◽  
Low Grade ◽  
Optic Pathway ◽  
Intracranial Tumors ◽  
First Line ◽  
Optic Pathway Gliomas

✓Optic pathway gliomas represent approximately 3–5% of childhood intracranial tumors. They usually occur in children during the first decade of life and are seen in 11–30% of patients with neurofibromatosis Type 1 (NF1). Although these tumors are typically low-grade gliomas, the clinical course and natural history are highly variable, making treatment paradigms difficult. Overall, however, they are often indolent tumors that can be observed over time for progression without initial treatment, especially in patients with NF1. Chemotherapy is the first-line treatment for progressive tumors, and radiation therapy is reserved for patients with progressive disease who are older than 5–7 years. Surgery is reserved for large tumors causing mass effect or hydrocephalus and tumors confined to the orbit or unilateral optic nerve.

scholarly journals PEDIATRIC OPTIC PATHWAY GLIOMA: DIENCEPHALIC SYNDROME

2021 ◽  
Vol 20 (1) ◽  
pp. 34-45
Author(s):  
A. F. Valiakhmetova ◽  
N. A. Mazerkina ◽  
L. I. Papusha ◽  
O. I. Bydanov ◽  
E. M. Tarasova ◽  
...  
Keyword(s):  
Clinical Course ◽  
Neurofibromatosis Type ◽  
Optic Pathway Glioma ◽  
Type I ◽  
Optic Pathway ◽  
National Medical ◽  
Number Of Patients ◽  
Diencephalic Syndrome ◽  
Pilocytic Astrocytomas ◽  
Aggressive Clinical Course

Diencephalic cachexia (DC ) is a metabolic disorder characterized by a decrease in body weight. DC usually occurs in the presence of glioma brain tumors extended into the optic pathway. These tumors are very aggressive and have poor prognosis.Objective: to analyze the clinical course of optic pathway gliomas (OPG s) in patients with and without DC .Material and Methods. The study included 264 patients aged 0 to 18 years with an initial diagnosis of OPG s registered in the N.N. Burdenko National Medical Research Center of neurosurgery from 01/01/2003 to 12/31/2015. Patients were divided into two groups: without DC (204 people) and with DC (60 children). Results: neurofibromatosis type I (NFI) was much more common in children without DC , and pilomyxoid histology was much more prevalent in children with DC . Five-year overall survival (OS ) and event-free survival EFS were significantly lower in children with DC than in children without DC (82 ± 5 % and 96 ± 1 %, respectively versus 37 ± 7 % and 62 ± 3 %, respectively). It was found that in the DC group, the OS and EFS rates were significantly lower in girls, in children without NFI, in children without histological verification and in children with pilocytic astrocytomas. It was also found that in the DC group, OS rates were significantly lower in children under 1 year, and EFS rates were significantly lower in children aged more than 12 months. The number of patients without events were significantly higher in the group without DC (p=0.001). The number of deaths in the postoperative period was significantly higher in children with DC (p<0.001). Diabetes insipidus and hyponatremia were significantly more common in patients with diencephalic cachexia, and vision improvement after treatment was significantly more likely to occur in patients without DC .Conclusion. OPG s in patients with DC have a more aggressive clinical course, which requires more careful treatment and observation. 

scholarly journals Sepsis from Neurofibromatosis

2006 ◽  
Vol 33 (3) ◽  
pp. 328-328
Author(s):  
Navdeep Tangri ◽  
Shireen Sirhan ◽  
Gordon Crelinsten
Keyword(s):  
Autosomal Dominant ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Large Mass ◽  
Type I ◽  
Neurofibromatosis Type I ◽  
Autosomal Dominant Disorder ◽  
Chest Wound ◽  
History Of ◽  
The Right

Neurofibromatosis Type I or von Recklinghausen’s neurofibromatosis is an autosomal dominant disorder with a high index of spontaneous mutations and extremely varied and unpredictable clinical manifestations. We present a case of sepsis secondary to an infected hematoma, enclosed within a massive neurofibroma.A 42-year-old man presented to the emergency department with a one week history of fever and chills. He reported an increase in pain and size of a growth near his chest. The patient was noted to be febrile on arrival. On physical examination, a very large neurofibroma was seen extending from the right upper chest. Wound and blood cultures were obtained. Computed Tomography (CT) of the thorax revealed a hematoma contained within the large mass.

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