Primary sellar neuroblastoma mimicking invasive pituitary adenoma: a systematic review

INTRODUCTION: to report an extremely rare case of sellar neuroblastoma, which mimicked invasive pituitary adenoma with supra- and para-sellar extensions, treated through endoscopic endonasal approach. A systematic review of the literature on diagnosis and management of patients affected by sellar neuroblastomas has been performed. EVIDENCE ACQUIITION: A literature search according to the PRISMA statement was conducted using MEDLINE, Google Scholar, and EBSCO databases, searching for the following MeSH terms: (sellar OR parasellar OR suprasellar OR sphenoidal) AND (neuroblastoma OR extranasal esthesioneuroblastoma OR central neuroblastoma). EVIDENCE SYNTHESIS: Sixty-eight studies were identified. We included 16 papers in our systematic review, comprising a total of 16 patients, 37.5% males and 62.5% females, with a mean age of 47.2 years. Visual disturbances were found in 56.2% of them, hyperprolactinemia in 43.7%, panhypopituitarism in 12.5%, normal pituitary function in 6.25%, SIADH in 25%, but no data is available in 25% of cases. Brain computed tomography and magnetic resonance imaging showed suprasellar and parasellar extension in 31.2% of patients, isolated suprasellar extension in 50%, isolated parasellar extension only in 6.25%, while in 1 case involvement of the petrous apex was described. Transcranial surgery (TCS) was performed for the removal in 31.2% of patients, transsphenoidal surgery (TSS) in 43.7%, a combined approach in 12.5%, and in two cases surgery was not performed due to poor general patient conditions. Adjuvant treatment with conventional radiotherapy (CRT) was performed in 62.5% of cases, gamma knife surgery (GKS) in 18.7%; in 12.5% it was not carried out, while in one case there was no data available. Absence of recurrence was documented in 50% of patients, recurrence that required further treatments in 25%, while in 25% there was no data available. CONCLUSIONS: Primary sellar neuroblastoma is an extremely rare entity with high propensity to recur; whole body scintigraphy is recommended to search for extracranial locations, for optimum management of the disease. Special attention should be paid to endocrinological evaluation and management.