Isolated intracranial relapse of hodgkin lymphoma in a child

Abstract

Introduction: Although involvement of the central nervous system (CNS) is well described in non­Hodgkin lymphoma (NHL) at diagnosis and/or at relapse, there are few reports in the pediatric literature of primary or relapsed CNS Hodgkin lymphoma (HL). Case Report: We report dural disease as the only site of relapse of HL in an immunocompetent seven­year­old child, presenting with diplopia. Imaging demonstrated a well­circumscribed dural­based mass at the base of the skull involving the clivus and enveloping the pituitary. Restaging did not show disease elsewhere, with normal bone marrow trephine biopsies and cytological examination of the cerebrospinal fluid. Biopsy of the dural­based tumor confirmed classical HL. Eleven months before, he was diagnosed with stage IIIA HL nodular sclerosis subtype and underwent four courses of combination chemotherapy with a complete metabolic response on early response assessment. Forty­ eight months after completing treatment for relapsed HL, he remains well in complete remission, although future relapse of HL cannot be excluded. Conclusion: There were no specific risk factors predictive of relapse in this child. He had responded well to standard chemotherapy as early response assessment after two courses showed complete metabolic response. Treatment of this child at relapse was challenging, since standard salvage chemotherapy regimens comprise drugs not well known to traverse the blood brain barrier. This case highlights the rarity of intracranial HL in children and difficulty with regards to the diagnosis, evidence based treatment and overall prognosis. We suggest that irrespective of any imaging abnormality, histological confirmation should be sought before commencement of definitive treatment.