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 Endocrine-Related Cancer (1998) 5 303-310 type of tumour is reviewed in detail.

Hemorrhagic Suprasellar Central Nervous System Embryonal Tumor in an Adult: Uncommon Features of an Extremely Rare Neoplasm

“…Sellar embryonal tumors presenting in the adult population have been reported in English-written literature only 12 times before, defining those lesions as extremely rare tumors (►Table 1). [19][20][21][22][23][24][25][28][29][30][31][32] The pathologic diagnoses of previously published cases were 10 cases of primary sellar neuroblastoma and 2 cases reported as suprasellar PNETs according to the previous versions of the WHO classification of CNS tumors.…”

“…Sellar embryonal tumors presenting in the adult population have been reported in English-written literature only 12 times before, defining those lesions as extremely rare tumors ( Table 1 ). 19 20 21 22 23 24 25 28 29 30 31 32…”

“…Only 12 cases describing embryonal lesions in the sellar and parasellar area occurring in adults were reported (►Table 1). [19][20][21][22][23][24][25][28][29][30][31][32]…”

“…3,[5][6][7][8][9][10][11][12][13][14][15] Onset with intracranial hemorrhage is further unusual and it has been previously reported in only three cases, one of which was fatal. [16][17][18] Most interestingly, suprasellar embryonal tumors may behave as highly infiltrative tumors toward the surrounding neurovascular structures (cavernous sinus) and paranasal sinuses (sphenoidal sinus) [19][20][21][22][23][24][25] ; white matter fiber diffusion has never been previously described.…”

“…Most interestingly, suprasellar embryonal tumors may behave as highly infiltrative tumors toward the surrounding neurovascular structures (cavernous sinus) and paranasal sinuses (sphenoidal sinus) 19 20 21 22 23 24 25 ; white matter fiber diffusion has never been previously described.…”