Hydrocephalus as Clinical Indicator of Central Nervous System Relapse in Acute Lymphoblastic Leukemia

Acute lymphoblastic leukemia (ALL) is the most common cancer diagnosis in children. While current treatment has greatly improved survival rates, relapse occurs in 15-20% of patients. Signs and symptoms are similar to those found at initial presentation. However, in some patients, relapse can occur in the central nervous system (CNS), even if they did not have previous CNS involvement. Many cases of CNS relapse are clinically silent and are discovered at the time of bone marrow relapse. These patients can be asymptomatic or show signs of mass effect or increased intracranial pressure. Classic MRI findings include signs of leptomengineal disease (LMD) in the ventricles along with hydrocephalus. In this case report, we describe a child with pre-B cell ALL in remission who relapsed with CNS involvement. Unique to this case is that imaging revealed hydrocephalus without definitive evidence of LMD. A ventriculoperitoneal (VP) shunt was placed for the treatment of presumed idiopathic hydrocephalus. CSF analysis revealed leukemic cells. There has been no previous reporting in the literature of hydrocephalus being the only finding on MRI, without the highlighting of LMD. In this case, we had to consider general pediatric causes of hydrocephalus and the final diagnosis was made by examining the cerebral spinal fluid (CSF). This case report points out that hydrocephalus can be an initial indicator for CNS relapse. Citation: Dao JQ, Khatua S, Rytting ME, Sandberg DI, Tran DQ (2015) Prog Science 2(3):e24 | doi: 10.14721/pscience.2015.e24 Academic Editor: Chetan A. Dhamne | National University Health System | Singapore. Reviewer #1: Rajat Bhattacharyya | KK Women’s and Children’s Hospital | Singapore Reviewer #2: undisclosed Received: 12/08/2014 | Revised: 01/31/2015 | Published: 03/17/2015 Copyright: © 2014 Dao JQ et al. Published by TranScience. This is an openaccess article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author(s) and source are credited. Funding: none Competing Interests: The authors have declared that no competing interests exist. Author Contributions: JQD wrote and SK, MER, DIS, DQT revised the manuscript. * Academic E-mail: [email protected] Significance of Report What is already known about this condition? • Acute lymphoblastic leukemia (ALL) is the most common pediatric leukemia with up to 85% achieving long-term cure. • Despite excellent cure rates, 15-20% of patients will relapse and <10% in this group will have CNS disease. • A common MRI finding in CNS relapse is leptomeningeal disease (LMD) in the ventricles. What is unique about this case? • First case report of a child with ALL in remission who has hydrocephalus but MRI lacks LMD until CSF analysis reveals CNS relapse. • This case points out that hydrocephalus can be an indicator for CNS relapse. How will these new findings impact diagnosis, clinical care and research? • This case brings awareness to the importance of considering ALL relapse with CNS involvement in patients with isolated hydrocephalus.