Case reportSellar neuroblastoma mimicking a pituitary tumour: Case report and review of the literature
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.
References (21)
- et al.
Primary cerebral neuroblastoma in an adult man
Surg Neurol
(1981) - et al.
Neuroblastoma and other neuroendocrine tumours
Semin Nucl Med
(2007) - et al.
Primary cerebral neuroblastoma: long-term follow-up review and therapeutic guidelines
J Neurosurg
(1983) - et al.
The biological behavior of primary cerebral neuroblastoma: a reappraisal of the clinical course in a series of 70 cases
Ann Neurol
(1984) - et al.
Allelic loss of chromosome 1p as a predictor of unfavorable outcome in patients with neuroblastoma
N Engl J Med
(1996) - et al.
Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a Childrens Cancer Group randomized trial
J Clin Oncol
(1995) - et al.
Olfactory neuroblastoma
- et al.
Primitive neuroectodermal tumours of the cerebrum: pathology and treatment
J Neuro-Oncol
(1985) - et al.
Primary cerebral neuroblastoma A clinicopathological study of 35 cases
Brain
(1976) - et al.
Supratentorial primitive neuroectodermal tumours: diffusion-weighted MRI
Neuroradiology
(2000)