Unusual Presentations of Acute Lymphoblastic Leukemia in Children: A Study of 9 Patients

Abstract

Background: Acute lymphoblastic leukemia is the most common malignancy in children below 15 years of age. Bone marrow, CNS and the gonads are the three major sites of failure or relapse in acute lymphoblastic leukemia. Occasionally, leukemic cells can infiltrate the unusual sites and present with some unusual manifestations either at initial (diagnosis) presentation or during the course of disease. This study presents 9 such rare cases of unusual manifestations of acute lymphoblastic leukemia. Aim: To report the unusual presentation and management of acute lymphatic leukemia in children either at initial diagnosis or during the course of disease. Methods: Between 2000-2012, 1800 patients with acute lymphoblastic leukemia were treated at the Gujarat Cancer & Research Institute, Ahmedabad, a tertiary cancer care center and one of the largest regional cancer center of India. Among these patients a total of 317 cases were found to develop relapse at bone marrow, CNS, testicles etc. Out of these patients, a total of 9 patients were detected to have very unusual manifestations of the disease, either at initial presentation or during the course of their disease. These patients had parotid salivary gland involvement (1), maxillary sinus (2), bilateral blindness due to retinal detachment (1), bilateral facial nerve palsies (1), multiple symmetrical involvement of bones of both upper and lower extremities (1), small bone of the hand (4th metacarpal bone) (1), massive splenomegaly (1) and torsion of the testicle (1). The manifestations of such unusual clinical presentations, their evaluation, management and ultimate outcome is being presented in this presentation along with review of the literature. Results: Acute lymphoblastic leukemia can present with very unusual manifestation during initial diagnosis or later. Conclusion: ALL may occasionally present in some unusual way. Such uncommon presentation can create a diagnostic dilemma as well as difficulty in management. All such presentations require special attention from the treating consultant and team for the better outcome. We strongly recommend that all such rare presentations must be reported to enhance the understanding of the disease and enhance the literature.