Treatment options and prognosis for multicentric juvenile pilocytic astrocytoma

Abstract

Little is known about the risk of developing multicentric disease in patients with juvenile pilocytic astrocytoma (JPA), and even less about its prognosis. Only five cases have been reported. Between 1986 and 1992, the authors treated 90 patients with either primary or recurrent JPA, 11 of whom developed multicentric spread. Ten patients had primary tumors in the hypothalamic region, eight were under 4 years of age at initial diagnosis, all had initially undergone a subtotal resection or biopsy, and 10 received postoperative multiagent chemotherapy or irradiation for residual disease. Multicentric spread was discovered immediately to 108 months after initial diagnosis; nine patients were asymptomatic at the time. Most patients received chemotherapy for the multicentric disease, which was found throughout the craniospinal axis. During 21 to 148 months of follow-up monitoring, seven patients had stabilization or regression of multicentric disease and four died. Patients with hypothalamic region tumors were 23 times more likely to develop multicentric spread than were those with primary tumors located elsewhere (p < 0.001). Based on this review, it is concluded that multicentric spread of JPA occurs more frequently than was previously recognized. In patients with subtotally resected JPA and several years of follow-up review via magnetic resonance imaging, the incidence of recurrence in a site different from the original was 12%. Patients with subtotally resected JPA in the hypothalamic region should be considered to be at high risk for developing multicentric spread. Chemotherapy appears useful in stabilizing multicentric disease. Earlier detection and intervention may result in longer disease-free survival in patients with multicentric spread of JPA.

Leptomeningeal dissemination of pilocytic astrocytoma at diagnosis in childhood: two cases report

“…Pilocytic astrocytoma (PA) is a benign subset of gliomas with an excelent prognosis 1 and that rarely spread along the neuraxis. Leptomeningeal dissemination (LD) of primary central nervous system (CNS) tumors in children has been reported mainly in ependymomas, germ-cell tumors, primitive neuroectodermal tumors, high grade gliomas [2][3][4][5][6][7][8][9] .…”

“…Leptomeningeal dissemination (LD) of primary central nervous system (CNS) tumors in children has been reported mainly in ependymomas, germ-cell tumors, primitive neuroectodermal tumors, high grade gliomas [2][3][4][5][6][7][8][9] . Low grade gliomas dissemination has been documented in few cases 1,2,5,6,9 . Leptomeningeal dissemination of pilocytic astrocytoma in children is much more uncommon with 32 cases reported in the literature 1,2,6,7,[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] .…”

“…Low grade gliomas dissemination has been documented in few cases 1,2,5,6,9 . Leptomeningeal dissemination of pilocytic astrocytoma in children is much more uncommon with 32 cases reported in the literature 1,2,6,7,[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] . These include only five with LD at diagnosis 13,[17][18][19] .…”

Leptomeningeal dissemination of pilocytic astrocytoma in a 17-year-old boy

“…The exact incidence still remains unclear (6,12). Previous studies have demonstrated that leptomeningeal dissemination occurs in approximately 4 to up to 12% of patients with low grade gliomas or low grade astrocytomas (9,11). It seems that with the growing use of MRI as a routine investigative tool for the diagnosis of brain tumors will increase the number of these patients are being identified (5).…”

Differential imaging characteristics and dissemination potential of pilomyxoid astrocytomas versus pilocytic astrocytomas

“…Furthermore, experiences from earlier reports of successful management of disseminated, aggressive PAs may help the management of children with PMA (Mamelak et al, 1994;McCowage et al, 1996). The emerging understanding of the genetic and molecular background of tumorigenesis, such as the recently recognized importance of the MAPK pathways, may allow us to develop improved treatments for PA and PMA in the future (Aktas et al, 2014).…”