2006
DOI: 10.1200/jco.2005.03.8349
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Second Primary Tumors in Neurofibromatosis 1 Patients Treated for Optic Glioma: Substantial Risks After Radiotherapy

Abstract: There is a significantly increased risk of second nervous system tumors in those NF1 patients who received radiotherapy for their OPGs, especially when treated in childhood. Thus radiotherapy should only be used if absolutely essential in children with NF1.

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Cited by 333 publications
(173 citation statements)
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“…This is in contrast to the sporadic PA that are normally formed in the cerebellum [21]. The OPG remain asymptomatic in the majority of patients but can cause visual loss in some patients [22][23][24]. When symptomatic, tumors have been treated with surgery, chemotherapy and radiotherapy.…”
Section: Cns Tumorsmentioning
confidence: 99%
See 1 more Smart Citation
“…This is in contrast to the sporadic PA that are normally formed in the cerebellum [21]. The OPG remain asymptomatic in the majority of patients but can cause visual loss in some patients [22][23][24]. When symptomatic, tumors have been treated with surgery, chemotherapy and radiotherapy.…”
Section: Cns Tumorsmentioning
confidence: 99%
“…When symptomatic, tumors have been treated with surgery, chemotherapy and radiotherapy. The use of radiotherapy is avoided if possible, based on increased risk of developing secondary high grade gliomas [22]. Studies indicate that OPG patients receiving radiotherapy have a three-fold increased risk of developing secondary gliomas, and a nine-fold increased risk of developing an MPNST [22].…”
Section: Cns Tumorsmentioning
confidence: 99%
“…There is also concern about late vascular events: the incidence of occlusive vasculopathy at the circle of Willis in children with brain tumors is highest among those with OPTs, especially in younger children (29). There is also a heightened concern regarding the risk of second malignancy in patients with NF1 (30). The Toronto group has uniquely reported a 10% incidence of second malignant neoplasms after irradiation for OPTs; of interest, a series from Children's Hospital of Los Angeles showed the same rate of anaplastic degeneration in JPA after surgery alone (31,32).…”
Section: Radiation Therapymentioning
confidence: 94%
“…Gross total resection (GTR) is the treatment of choice for accessible lesions. GTR has been documented in 20-25% of all cases, apparently more often in children and adolescents (30). The OS rate at 10 years after total excision is reported to be about 60% (38).…”
Section: Oligodendrogliomamentioning
confidence: 99%
“…Unnecessary radiation exposure should be avoided, especially in individuals with nf1, because of the risk of secondary malignancy 24 and other potential complications 25 seen with higher doses of radiation therapy. However, fdg-pet might be useful for the assessment of malignant potential (as in our case 1) and for clinical and surgical planning of unusual presentations (our cases 1 and 2).…”
Section: Figure 1 Case 1 (A) Initial Coronal and Axial Magnetic Resomentioning
confidence: 99%