Leptomeningeal dissemination of low‐grade gliomas in childhood

Abstract

Although leptomeningeal spread (LMS) of primary CNS tumors in children has been well documented in the literature, it has rarely been reported in children with low-grade gliomas. Between 1975 and 1985, 6 of 162 children (3.7%) with low-grade gliomas treated at Children's Hospital of Philadelphia had LMS. LMS was present at diagnosis of the original tumor in one patient, was the first sign of relapse in one patient, occurred simultaneously with local relapse in two patients, and after local relapse in two patients. Pathology of the original tumor was low-grade astrocytoma in five and low-grade oligodendroglioma in one. Primary tumor site was cervical cord in three, chiasm in one, frontal lobe in one, and cerebellum in one. All of the children with LMS had undergone surgical treatment at the time of diagnosis of the primary tumor; four had total resections at some point in their course. Three of the six patients died; three are still alive after treatment with radiation therapy and/or chemotherapy. The longest survival to date has been 3 1/2 years after diagnosis of LMS. We compared clinical characteristics of these six patients with 131 children with low-grade tumors without dissemination treated at our institution during the same time period. LMS, although relatively infrequent, does occur in children with low-grade gliomas, especially spinal cord tumors. LMS may occur at any time during illness and diagnosis may be difficult unless LMS is suspected. Treatment, at times, results in clinical improvement and considerable disease control.

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

“…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 .…”

Chemotherapy for unresectable and recurrent intramedullary glial tumours in children

“…At the outset of chemotherapy, three of our patients had leptomeningeal metastases; this is a rare event in benign CNS tumours in children (Hardison et al, 1987;Bell et al, 1988;Civitello et al, 1988), being more frequent with intramedullary tumours and malignant tumours (Chamberlain, 1995). Chemotherapy induced two PR and one SD in our three children with leptomeningeal dissemination.…”

“…They had various primary tumours and were treated with RT and intrathecal chemotherapy. Two of the three patients reported by Civitello et al (1988) were alive 9 and 44 months after relapsing. They both received chemotherapy, and one also had craniospinal radiotherapy (csRT).…”

“…If complete resection is impossible, or if the tumour is inoperable, radiation therapy is usually recommended (Lunardi et al, 1993;O'Sullivan et al, 1994). Metastatic tumours (with leptomeningeal spread) and relapses have a poor prognosis when treated with the standard approach, and may require other forms of treatment (Bell et al, 1988;Civitello et al, 1988;Chamberlain, 1995). In the case of holocord tumours and cases involving young children, radiotherapy (RT) can have very severe sequelae (spinal deformities, growth deficits, etc.)…”

Intractable vomiting from glioblastoma metastatic to the fourth ventricle: Three case studies

“…Distant metastases of cerebral glioma to the spinal cord via CSF pathways have also been reported (Nakagawa et al, 1990); however, they respond poorly to spinal irradiation (Delattre et al, 1989). Secondary gliomatosis arises rarely in patients with low-grade glioma (Civitello et al, 1988). Secondary gliomatosis may be the presenting symptom of glioma in rare cases (Davila et al, 1993;Herman et al, 1995).…”