Pediatric suprasellar primary CNS lymphoma with cavernous sinus invasion and ischemic stroke: a case report and systematic review.

完整摘要 · 英文原文

BACKGROUND: Primary central nervous system lymphoma (PCNSL) is an exceptionally rare pediatric malignancy, accounting for less than 1% of childhood central nervous system tumors. Suprasellar involvement is particularly uncommon and frequently mimics more prevalent lesions, including germinoma and Langerhans cell histiocytosis, often resulting in delayed diagnosis. CASE PRESENTATION: We report the case of a 12-year-old immunocompetent girl with a history of Epstein-Barr virus infection and a previously resected atypical smooth muscle tumor of the ileum who presented with high-grade fever, severe headache, and diplopia. She rapidly deteriorated neurologically, and magnetic resonance imaging demonstrated a rim-enhancing sellar-suprasellar lesion invading the cavernous sinus, causing critical internal carotid artery stenosis with bilateral watershed cerebral infarctions. Emergency endoscopic endonasal transsphenoidal decompression was performed. Histopathological examination confirmed anaplastic diffuse large B-cell lymphoma (DLBCL) of the non-germinal center B-cell subtype. Despite prompt surgical intervention and initiation of systemic chemotherapy, the patient developed central diabetes insipidus, severe hemodynamic instability, and ultimately died within few months. METHODS: A systematic review was conducted in accordance with the PRISMA 2020 guidelines. PubMed, Ovid, and Scopus were searched from database inception to 31 May 2026 to identify published cases of pediatric suprasellar primary central nervous system lymphoma (PCNSL). RESULTS: Five studies comprising seven previously reported pediatric patients met the inclusion criteria. Six of seven historical patients (85.7%) achieved complete radiological remission, Diffuse large B-cell lymphoma (DLBCL) or large-cell B-cell lymphoma was reported in four of seven patients (57.1%). Most patients achieved complete remission following intensive CNS-directed chemotherapy, although persistent endocrine dysfunction remained common. In contrast to previously reported cases, our patient presented with fulminant cavernous sinus invasion, critical internal carotid artery stenosis, and ischemic watershed cerebral infarctions, representing an exceptionally aggressive clinical phenotype. CONCLUSION: Pediatric suprasellar PCNSL is a rare but highly aggressive disease that should be considered in children presenting with diabetes insipidus, pituitary stalk lesions, or rapidly progressive suprasellar masses. Progressive radiologic evolution or atypical vascular complications should prompt early histopathological confirmation to facilitate timely diagnosis and initiation of treatment.