A pseudo-endocrine suprasellar tumour: review of primitive neuroectodermal tumours in adults

Abstract

Primitive neuroectodermal tumours (PNETs) are very rare malignant brain tumours mostly affecting children. We report a 55-year-old female with a PNET, presenting with the clinical picture of a large suprasellar tumour. The patient complained of visual field defects, and had personality changes but no endocrine deficiencies or signs of hypothalamic disturbance. The diagnosis of craniopharyngioma was initially suspected on computerised tomography scan, but was not confirmed by magnetic resonance imaging, because of the presence of some perilesional oedema. The tumour was removed in two phases: first through a right frontal craniotomy to remove the suprasellar part and two weeks later through a transsphenoidal approach to remove tumour tissue from the sella turcica. The pathological examination of the first resected specimen revealed compact fields of small, rounded cells and hyperplasia of the interstitial connective tissue, compatible with an adamantinomatous craniopharyngioma. Pathological investigation after the second operation demonstrated perivascular pseudo-rosettes and immunoreactivity of tumour cells for synaptophysin, favouring the diagnosis of a PNET. After the surgery the patient retained a serious cognitive deficit. Staging examinations were not performed and the intention of systemic therapy was abandoned. She died six months later, in poor general condition, of a respiratory infection. Our case of PNET is the first to be reported with a suprasellar location in an adult patient and we suggest that this diagnosis should be included in the differential diagnosis of a hypothalamic tumour in adults. The epidemiology and therapy of this unusual