Article

Outcomes of multidisciplinary management in pediatric low-grade gliomas.

Department of Radiation Oncology, University of Michigan Health System, Ann Arbor, MI, USA.
International journal of radiation oncology, biology, physics (Impact Factor: 4.59). 04/2011; 81(4):e481-8. DOI: 10.1016/j.ijrobp.2011.01.019
Source: PubMed

ABSTRACT To evaluate the outcomes in pediatric low-grade gliomas managed in a multidisciplinary setting.
We conducted a single-institution retrospective study of 181 children with Grade I-II gliomas. Log-rank and stepwise Cox proportional hazards models were used to analyze freedom from progression (FFP) and overall survival (OS).
Median follow-up was 6.4 years. Thirty-four (19%) of patients had neurofibromatosis Type 1 (NF1) and because of their favorable prognosis were evaluated separately. In the 147 (81%) of patients without NF1, actuarial 7-year FFP and OS were 67 ± 4% (standard error) and 94 ± 2%, respectively. In this population, tumor location in the optic pathway/hypothalamus was associated with worse FFP (39% vs. 76%, p < 0.0003), but there was no difference in OS. Age ≤5 years was associated with worse FFP (52% vs. 75%, p < 0.02) but improved OS (97% vs. 92%, p < 0.05). In those with tissue diagnosis, gross total resection (GTR) was associated with improved 7-year FFP (81% vs. 56%, p < 0.02) and OS (100% vs. 90%, p < 0.03). In a multivariate model, only location in the optic pathway/hypothalamus predicted worse FFP (p < 0.01). Fifty patients received radiation therapy (RT). For those with less than GTR, adjuvant RT improved FFP (89% vs. 49%, p < 0.003) but not OS. There was no difference in OS between patient groups given RT as adjuvant vs. salvage therapy. In NF1 patients, 94% of tumors were located in the optic pathway/hypothalamus. With a conservative treatment strategy in this population, actuarial 7-year FFP and OS were 73 ± 9% and 100%, respectively.
Low-grade gliomas in children ≤5 years old with tumors in the optic pathway/hypothalamus are more likely to progress, but this does not confer worse OS because of the success of salvage therapy. When GTR is not achieved, adjuvant RT improves FFP but not OS. Routine adjuvant RT can be avoided and instead reserved as salvage.

0 Bookmarks
 · 
97 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Sellar masses are an heterogeneous group of lesions, both in nature and management. Not all of them require surgery. To describe the presenting symptoms of sellar masses and endocrine abnormalities occurring during follow-up. To emphasize the significance of endocrine assessment, and to identify lesions amenable to hormonal treatment. A retrospective review of the records of children under 14 years of age referred to our center for sellar lesions during 12 years. Data collected included sex, age, nature of lesion, clinical presentation, size, treatment, and endocrine abnormalities. Forty-five patients (25 females) aged 7.2±4.1 years (range 0.25-13.5) were enrolled. Follow-up time was 6.2±3.7 years. Lesion nature was known in 39 cases, 4 of which were successfully treated at the Endocrinology Department: 3 prolactinomas (with dopamine agonist) and one thyrotroph cell hyperplasia (with levothyroxine). The most common presenting symptoms were neurological and/or visual (25/45), followed by endocrine conditions (13/45). Duration of endocrine and neuro-ophthalmic symptoms was 12.6±18.2 months and 2.6±4.9 (P=.012), respectively. Some endocrine condition was found in 24/45 patients at the initial evaluation and in 37/45 patients at the end of follow-up. Management of sellar lesions requires a multidisciplinary effort. Endocrine tests are indispensable to identify lesions amenable to hormonal treatment. Endocrine disorders usually occurred before neurological and ophthalmological symptoms, and their identification may therefore allow for earlier diagnosis. Hormone assessment should be regularly performed during follow-up.
    Endocrinología y Nutrición 03/2014;
  • [Show abstract] [Hide abstract]
    ABSTRACT: Radiation therapy is often considered the treatment of choice for low-grade gliomas. However, given the long-term effects of radiation on the developing brain, the appropriate use of radiation therapy in pediatric patients remains controversial. The purpose of this study was to evaluate progression-free survival (PFS) of pediatric low-grade glioma patients treated with radiation therapy. Data were obtained through a retrospective chart review of patients treated between 1991 and 2008 from a single tertiary care center in the midwest. The study population consisted of 17 patients, of whom 8 (47%) had tumor recurrence after radiation therapy. The median follow-up time was 8.2 years, with a range of 2.3 to 17.2 years. The median age at diagnosis was 5.4 years, and the median age at radiation therapy was 9.4 years. The 3- and the 10-year PFS were 69%±11.7% and 46%±13.3%, respectively. A significant difference in PFS was seen when comparing brainstem tumors with hypothalamic/optic pathway tumors (P=0.019). Differences in PFS based on the age at diagnosis, the extent of initial surgery, and indication for radiation therapy were not significant. A larger multicenter study is needed to better assess PFS in these patients.
    Journal of Pediatric Hematology/Oncology 04/2014; · 0.96 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Neurofibromatosis type 1 is a relatively common inherited disorder. Patients have a high predisposition to develop both benign and malignant tumours. Although many manifestations of neurofibromatosis type 1 affect the nervous system, other organs and tissues can also be affected. Because of the varying features and clinical heterogeneity inherent to this disorder, patients can present to different medical and surgical specialists and, therefore, the association of clinical symptoms with neurofibromatosis type 1 might not be appreciated. Thus, for prompt diagnosis and to provide optimum care for patients with neurofibromatosis type 1, clinicians must be aware of the diverse clinical features of this disorder. We advocate a multidisciplinary approach to care, entailing a dedicated team of specialists throughout the lifetime of the patient. As our understanding of this disorder deepens through basic laboratory and clinical investigations, swift implementation of new effective treatments becomes feasible.
    The Lancet Neurology 08/2014; 13(8):834–843. · 21.82 Impact Factor