Ali Mazloom MD, Presenter: Nothing to Disclose

Joseph Hodges MD, MBA, Abstract Co-Author: Nothing to Disclose

Bin Sing Teh MD, Abstract Co-Author: Nothing to Disclose

E. Brian Butler MD, Abstract Co-Author: Nothing to Disclose

Arnold C. Dela Paulino MD, Abstract Co-Author: Nothing to Disclose

Purpose: Juvenile pilocytic astrocytoma (JPA) with leptomeningeal dissemination (LMD) is a rare occurrence for which patient and tumor characteristics, treatments and outcomes have not been well defined. The purpose of this study is to review the existing literature in order to gain more understanding of this disease. Methods: A PubMed search of English-language articles with radiation, juvenile pilocytic astrocytoma, low grade glioma and leptomeningeal dissemination used as key words in the search engine yielded 27 articles dealing with JPA and LMD from 1976-2005. Results: There were 59 patients with a median age of 72 months (range, 3.5 to 840 months) at diagnosis of JPA and LMD. Gender distribution was 24 (41%) boys, 18 (30%) girls, and 17 (29%) unknown. JPA and LMD was diagnosed either synchronously (N=22) with the primary, subsequently (N=26) after primary diagnosis, or at an unknown time (N=11). For cases with metachronous spread to the leptomeninges, the median time to LMD was 41 months (range, 4 to 420 months). The primary site was reported in 49 cases and was located in the hypothalamic/chiasmic region in 28 (57%), cerebellum in 16 (33%), and the third ventricle in 5 (10%) patients. Initial treatment for the primary tumor in cases with metachronous spread was surgery alone in 10 (39%), surgery with radiotherapy (XRT) in 11 (42%), chemotherapy (CTX) with or without surgery and/or XRT in 4 (15%) and VP shunt alone in 1 (4%). LMD site was reported in 53 cases with involvement of the brain and spine in 22 (41%), brain alone in 11 (21%) and spine alone in 20 (38%). Overall, radiotherapy (XRT) was given to 23 (39%) while chemotherapy (CTX) to 28 (48%) for LMD. The 5-year overall (OS) and progression-free survival (PFS) rates for all patients after diagnosis of LMD were 55.5% and 41.6%, respectively. Age, gender, primary site, LMD site, timing of LMD (synchronous vs. metachronous) and treatment for LMD did not influence PFS. The 5-year PFS for patients receiving XRT vs. no XRT was 41% and 30% (p=0.99) while for those receiving CTX vs. no CTX it was 29% and 35% (p=0.41). For XRT patients receiving craniospinal irradiation (CSI) vs. no CSI, the 5 year PFS was 70% and 30% (p=0.29). Conclusions: At 5 years, about half of patients are alive after diagnosis of JPA and LMD. PFS was not improved by use of XRT, although many patients were treated with less than craniospinal fields. While the 5- year PFS of 70% is encouraging with use of CSI, the patient numbers are small, and a conclusion that CSI is beneficial for JPA and LMD cannot be made based on this review.

Mazloom, A, Hodges, J, Teh, B, Butler, E, Paulino, A, Juvenile Pilocytic Astrocytoma with Leptomeningeal Dissemination.  Radiological Society of North America 2009 Scientific Assembly and Annual Meeting, November 29 - December 4, 2009 ,Chicago IL. http://archive.rsna.org/2009/8501587.html