Case report
Sellar neuroblastoma mimicking a pituitary tumour: Case report and review of the literature

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Abstract

Neuroblastomas of the sellar region are exceedingly rare. Only 2 cases have previously been reported. Management of these tumours depends on the tumour's primary site, the patient's age and histopathological features. We are reporting the case of a 43-year-old woman who developed progressive bitemporal hemianopsia and visual loss, accompanied by amenorrhea and hyponatremia. Laboratory findings revealed a slightly elevated prolactin level. Cranial MR-imaging displayed an intrasellar and suprasellar lesion with a maximum diameter of 2.5 cm that was suspicious for a pituitary adenoma or tuberculum sellae meningioma. The tumour was approached via a pterional trepanation. Intraoperatively, the tumour was highly vascularized and adhesive to the optic chiasm, the floor of the third ventricle, the hypothalamus and the hypophyseal stalk. Postoperatively, vision improved and prolactin dropped to normal values, but hyponatremia persisted. Histopathological examination revealed a neuroblastoma with strong positivity for synaptophysin and chromogranin, MAP-2 protein and NeuN-antigen in the immunohistochemistry. No pituitary hormone receptors were expressed. The MIB-1 labelling index was positive in 5% of the cell nucleoli. In the further course, the patient underwent radiotherapy of the neuroaxis.

A brief review of the literature is presented.

Introduction

Neuroblastomas are childhood embryonal tumours of migrating neuroectodermal cells derived from the neural crest and destined for the adrenal medulla and sympathic nerve system [3], [5]. In contrast to olfactory neuroblastomas (also called aesthesioneuroblastoma) which are assumed to originate from olfactory receptor cells high in the nasal cavity [17], primary cerebral neuroblastomas are rare and mainly arise in supratentorial or periventricular locations [1], [8]. CNS neuroblastomas are considered as a subgroup of primitive neuroectodermal tumours of the CNS (CNS PNET) [11]. So far, only 2 primary sellar neuroblastomas have been reported [10], [12].

We are reporting the case of a 43-year-old woman with a primary sellar neuroblastoma mimicking a pituitary adenoma or tuberculum sellae meningioma. The corresponding literature is reviewed.

Section snippets

Clinical report

A 43-year-old woman presented to our department with a 6-month history of progressive visual deterioration. Opthalmological examination revealed bitemporal hemianopsia and reduced visual acuity which was more pronounced in her left eye (visus acuity: right eye 0.8, left eye 0.6). In the last few years she developed secondary amenorrhea. Blood examination showed low sodium levels of 127 nmol/l which persisted under replacement therapy with sodium. Endocrinological evaluation revealed a slightly

Histology

On neuropathological examination the tumour was composed of small packed cells with oval-round hyperchromatic nucleoli (Fig. 3A). In some parts nests of tumour cells were found surrounded by mild till moderate fibrosed blood vessels. There was neither an obvious lobulated growth pattern, so called “zellballen” nor any fibrillary zones within the tumour. Mitosis was very scant. There was no necrosis and only slight cytoplasmic PAS-reaction in tumour cells. Immunohistochemically the tumour cells

Discussion

Cerebral neuroblastomas are rare embryonal neoplasm that generally arise in the supratentorial parenchyma or paraventricular region with a predilection for parietal and frontal lobes. They frequently show haemorrhage, necrosis, calification and cysts [1], [8]. Primary intracranial neuroblastomas mostly occur in children, about thirty percent of the patients are younger than 2 years of age [18]. Although the classification of embryonal CNS tumours is still controversial, cerebral neuroblastomas

Conclusion

Neuroblastomas of the sellar region are extremely rare findings. They are likely to imitate other pathologies that are found more frequently in this region, such as meningiomas or adenomas. Meticulous preoperative evaluation of MR-imaging as well as endocrinological and ophthalmological findings will hopefully help to identify typical features that allow the presumptive preoperative diagnosis. An interdisciplinary therapeutic approach is mandatory to guarantee a favorable outcome. Increasing

Acknowledgements

The authors thank Prof. Pietsch (Brain Tumor Reference Center, University of Bonn, Germany) and Prof. Saeger (reference center on pituitary tumours, Marienkrankenhaus, Hamburg, Germany) for confirmation of our diagnosis.

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