Defining the best available treatment for neurocytomas in children

Abstract

BACKGROUND.In children, neurocytomas are extremely rare tumors in the central nervous system. Since this entity was introduced in 1982, approximately 60 cases have been reported among patients age Յ ϭ18 years of age. The current analysis was performed to define the best available neurocytoma therapy in children. METHODS.All reported neurocytoma cases were reviewed for age, extent of resection, radiotherapy, radiotherapy dose, local control, and survival. Data were obtained from the literature and the authors. Statistical analysis was performed with the Kaplan-Meier method and log-rank test. RESULTS.Fifty-nine children were categorized by therapy: complete tumor resection (CTR; n ϭ 20), complete tumor resection plus radiotherapy (CTR-RT; n ϭ 11), incomplete tumor resection (ITR; n ϭ 14), and incomplete tumor resection plus radiotherapy (ITR-RT; n ϭ 14). Local control rates were better after CTR, CTR-RT, and ITR-RT than after ITR, at 5 years (86%, 100%, and 100% vs. 60%; P Ͻ 0.001) and at 10 years (86%, 100%, and 100% vs. 45%; P Ͻ 0.001). The 5-year and 10-year survival rates were 100% after CTR, 100% after CTR-RT, 100% after ITR-RT, and 93% after ITR (P ϭ 0.4). In the ITR-RT group, no difference was observed between doses Յ 50 gray (Gy) and Ն 54 Gy when compared for local control (P ϭ 1.0) and survival rates (P ϭ 1.0). Radiotherapy-related psychomotor retardation or secondary brain tumors were not reported. CONCLUSIONS.The prognosis of children with neurocytomas is extremely good.CTR was associated with better local control and survival rates than ITR. After ITR, radiotherapy improves local control, but not survival. If postoperative radiotherapy is considered, a dose of 50 Gy was appropriate for long-term local control in children, whereas higher doses were required in adults.

Extraventricular neurocytoma in the left temporal lobe: A case report and review of the literature

“…Complete surgical excision is currently considered as the optimal treatment for EVN, but is often limited by tumor proximity to eloquent areas ( 20 ). In cases of incomplete resection, available data suggests that radiotherapy offers the same tumor control rates for EVN ( 11 ).…”

“…Post-operative radiation therapy may be effective in reducing tumor size and recurrence rates in cases of aggressive atypical neurocytoma; the recommended dose of 50 Gy is considered to be the most appropriate for long-term local control in children ( 20 ). Additional Gamma Knife stereotactic radiosurgery may be performed post-surgery in order to obtain improved local control and a higher survival rate for cases that cannot be surgically resected ( 7 ).…”

Orbital Meningoencephalocele Due to Extraventricular Neurocytoma: Case Report

“…The patient, a 31-years old female, started to complain of mild headache and paroxysmal diplopia about one month before admission. Therefore, she underwent brain Magnetic Resonance Imaging (MRI) scan that showed the presence of a Complete surgical resection is currently the "gold standard" treatment [10], in cases of sub-total removal, radiotherapy may be a valid alternative to control tumor growth [11]. To date, no supported data can be found about radiosurgery rate of activity on EVNs.…”

Gamma knife radiosurgery for cranial neurocytomas

“…Gross-total resection can be achieved in 60 to 100% of patients and recurrence is seen in up to one third of patients after subtotal resection [4,5,30,31]. Rades et al [32] found that radiotherapy after subtotal tumor removal significantly improved local tumor control but had no effect on survival of children. However, control of tumor growth is an important determinant of prognosis, because neurocytomas may have malignant behavior, for example craniospinal dissemination or intraventricular hemorrhage, at the time of recurrence.…”