Opsoclonus‐myoclonus syndrome associated with neuroblastoma: Insights into antitumor immunity

Abstract

Opsoclonus‐myoclonus syndrome (OMS) is a rare neurological disorder. Half of these cases occur in children with neuroblastoma. Neuroblastoma patients with OMS usually have better oncological outcomes than those without OMS even after stratification by tumor stage and age, indicating that factors mediating OMS may also inhibit tumor cell proliferation. Although the mechanisms underlying OMS remain undefined, the cytokines and lymphocytes alterations in the cerebrospinal fluid support the concept that it is a pattern of neuroinflammation due to an autoimmune effect. The presence of lymphoid follicles consisting of follicular dendritic cells, CD20+ B lymphocytes, CD3+ T lymphocytes, and CD68+ macrophages in the tumor microenvironment in OMS‐associated neuroblastoma support the autoimmune nature of this disorder. This review focuses on the clinical and genetic features of OMS‐associated neuroblastoma, and we update readers on immune features of neuroblastoma with or without OMS to gain insights into antitumor immunity as it relates to tumor biology and prognosis.

Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

“…Despite this well described association between OMS and neuroblastoma, a specific associated antibody has yet to be elucidated. However, a neuroinflammatory process is suspected, given the cytokine and lymphocyte alterations in the cerebrospinal fluid (CSF) of these patients and the presence of CD20+ B lymphocytes and CD3+ T lymphocytes in the tumor microenvironment of OMS-associated neuroblastomas [47]. Additional suspected causes of pediatric OMS include para-infectious (e.g., varicella, influenza, human herpesvirus 6, SARS-CoV-2) inflammatory syndromes as well as familial/genetic neuro-inflammatory syndromes such as Aicardi-Goutières syndrome [43,[48][49][50].…”