Acute Promyelocytic Leukemia Presenting with Cranial Nerve Involvement and Clivus Mass.

Abstract

Introduction: Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia that has become highly curable with advances in targeted therapy. However, central nervous system (CNS) involvement is exceedingly rare in APL and presents significant therapeutic challenges due to the limited penetrance of standard therapies across the blood-brain barrier (BBB). While APL is traditionally managed without chemotherapy, cases with CNS involvement require a multimodal approach for effective disease control.

Case presentation: We present a unique case of a 31-year-old male with de novo APL and CNS involvement at the presentation, including a clival mass. The patient was successfully treated with a combination of systemic chemotherapy, intrathecal chemotherapy, and craniospinal irradiation, leading to durable remission.

Conclusion: This case highlights the rarity of CNS involvement in APL and underscores the importance of a multidisciplinary approach in its management. Additionally, it emphasizes the need to address logistical barriers to treatment to achieve optimal patient outcomes.