Radiat Oncol J.  2022 Jun;40(2):162-168. 10.3857/roj.2021.01074.

Craniospinal irradiation for respiratory failure secondary to central nervous system Erdheim-Chester disease

• Prasad RN
• Kobalka PJ
• Perlow HK
• Prevedello DM
• Blakaj DM
• Raval RR
• Palmer JD

• KMID: 2532067
• DOI: http://doi.org/10.3857/roj.2021.01074

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis typically featuring lower extremity osteosclerosis (96%) from Langerin-negative histiocytes with fibrosis. Central nervous system (CNS)-only disease is extremely rare, and particularly difficult to diagnose and manage. Neurologic complaints may be refractory to systemic therapy (ST), and the role of radiation therapy (RT) is undefined. We present a patient with ECD of the medulla complicated by respiratory failure and strength deficits with disseminated leptomeningeal disease (LMD) but not systemic disease, representing the first report of CNS-limited ECD with LMD. He received upfront craniospinal irradiation (CSI), representing a rare account of CSI for ESD, with marked clinical improvement resulting in extubation and improved strength. CSI facilitated excellent preservation of quality of life, and no treatment-related toxicity was observed prior to eventual, unrelated cardiopulmonary arrest. Thus, palliative CSI may augment ST by safely offering improved local control and symptomatic relief for CNS ECD.

Keyword

Craniospinal irradiation; CSI; Erdheim-Chester disease; Leptomeningeal disease; Radiation therapy; Stereotactic radiotherapy