Sellar neuroblastoma is hugely rare, only ten cases having been described in the literature. Primary intracranial neuroblastoma mainly occurs in children and arises in the supratentorial parenchyma. These tumors belong to embryonal central nervous system primitive neuroectodermal tumors (PNET). Magnetic resonance imaging (MRI) appearance is non-specific. Management is primary based on surgical resection. Diagnosis relied on histopathological examination. Through this case, it seems relevant to consider neuroblastoma face to MRI atypical aspects of pituitary adenoma.
We report the case of a 58-year-old-man with a 3-year old history of a rapidly growing sellar lesion diagnosed preoperatively as a pituitary non-functioning adenoma. He developed progressive bitemporal hemianopsia and visual field loss. Endocrinal investigations disclosed a functional hyperprolactinemia due to pituitary stalk effect. MRI displayed a sellar and supra-seller mass extension compressing the optic chiasm, with intra-sphenoidal sinus development, skull bone lysis and bilateral cavernous sinus invasion. He underwent near complete surgical removal through an endoscopic transseptal transsphenoidal approach. Histological and immunohistochemical studies yielded a diagnosis of neuroblastoma. Postoperatively his visual deficit improved. Neither endrocinal disturbance nor rhinorrhea occurred. He underwent fractioned radiotherapy a few weeks later.
Primary sellar neuroblastoma could be easily misdiagnosed as pituitary adenoma. Unusual MRI features could lead to consider this diagnosis. The optimal management seems to be surgical resection followed by radiation therapy. Some authors suggested adjuvant chemotherapy in patients with subtotal resection or recurrence however this is no consensual.Le texte complet de cet article est disponible en PDF.