Acta Neurochir (Wien) (2005) 147: 447–448 DOI 10.1007/s00701-004-0479-x
Brief Report of Special Case Primary sellar esthesioneuroblastoma T. Sajko1 , Z. Rumboldt2 , J. Talan-Hranilovic3 , I. Radic1 , and Z. Gnjidic1 1
Department of Neurosurgery, University Hospital ‘‘Sisters of Mercy’’, Zagreb, Croatia Department of Radiology, Medical University of South Carolina, Charleston, USA 3 ‘‘Ljudevit Jurak’’ Department of Clinical Pathology, University Hospital ‘‘Sisters of Mercy’’, Zagreb, Croatia 2
Published online February 24, 2005 # Springer-Verlag 2005
Summary We describe an extremely rare case of a primary intrasellar esthesioneuroblastoma. Keywords: Sellar tumor; esthesioneuroblastoma; MR imaging; transsphenoidal approach; total excision; immunohistochemistry.
Introduction Esthesioneuroblastoma is a well-recognized neuroectodermal neoplasm, which is thought to arise from olfactory receptors of the nasal mucosa. Numerous cases have been reported and intracranial extension of esthesioneuroblastoma has also been well documented in the literature [1, 2]. Primary sellar esthesioneuroblastoma is extremely rare, with only two described cases – one definite [3], and one possible [4]. We report a case of a primary sellar esthesioneuroblastoma without involvement of the olfactory apparatus, because of its unique location, MR imaging features, unusual presentation, and probable complete surgical removal of the neoplasm. Case report A 57-year-old woman was admitted at our institution for further diagnostic evaluation and treatment of a sellar mass lesion. Her medical history revealed a right breast carcinoma, which was resected and postoperatively treated with chemotherapy. Hormone analysis detected mildly increased levels of prolactin (92 mg=l) and follicule-stimulating hormone (FSH) (274 U=l). The rest of her endocrinological status was normal, and there was a left-sided temporal hemianopsia on her ophthalmological examination.
MR imaging showed an intrasellar tumour with suprasellar extension, which was compressing the optic chiasma and the floor of the third ventricle (Fig. 1). The lesion was iso-intense to brain on T1-weighted images, slightly hyperintense and heterogeneous on T2-weighted images, and demonstrated strong homogeneous enhancement. Signs of a possible left cavernous sinus infiltration were present, and there was no evidence of extension into the sphenoid sinus or nasal cavity. Preoperatively, our differential diagnosis was limited to a non-functional pituitary macro-adenoma with hyperprolactinemia due to stalk effect (‘‘pseudoprolactinoma’’), or metastatic breast cancer. The patient underwent a transsphenoidal resection of the pituitary lesion. Upon opening the dura, a soft white-greyish tumorous tissue was encountered. The mass leaked on pressure, giving the appearance of a degenerated macro-adenoma. The entire tumour was resected, including its suprasellar and pericavernous portions, while preserving the adjacent pituitary gland. Immunohistochemical analysis revealed strongly positive reactions to neuron-specific-enolase, synaptophysin, and chromogranin A. Immunohistochemistry was negative for prolactin (PRL), adenocorticotropin hormone (ACTH) and growth hormone (GH) excluding the diagnosis of a pituitary adenoma. Diagnosis of esthesioneuroblastoma was made, corresponding to WHO Stage 3 neoplasm. The patient had an uneventful postoperative recovery followed by the radiotherapy course (TD ¼ 50 Gy).
Discussion In the case reported, thorough evaluation, including ENT examination, neuroradiological studies, and intraoperative exploration of the nasal cavity and sphenoid sinus did not reveal any presence of the tumour in the nasal cavity, sphenoid sinus, or skull base region, nor signs of intracranial metastases. Esthesioneuroblastomas are radiosensitive tumours, and surgical resection followed by radiation therapy is considered the ideal treatment [3, 5]. In the case presented,
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T. Sajko et al.: Primary sellar esthesioneuroblastoma 4. Sarwar M (1979) Primary sellar-parasellar esthesioneuroblastoma. AJR 133: 140–141 5. Chamberlain MC (2002) Treatment of intracranial metastatic esthesioneuroblastoma. Cancer 95: 243–248
Comments This is a short report of a rare case of esthesio-neuroblastoma occuring purely within the sella and managed, seemingly successfully by the trans-sphenoidal route. The patient was thought to have a pituiary adenoma and was managed as such, even though the pathology turns out to be a ‘one off’! As a neurosurgical case, its interest is the neuropathology. I assume that this is cast iron. The operative features and the imaging too (no diagnostic features) are of marginal interest. M. Powell London
Fig. 1. Preoperative MR imaging. Midsagittal T1-weighted image shows an iso-intense intrasellar mass with suprasellar extension, measuring 25 mm in its largest diameter
the fact that the tumour was soft and resectable, enabled us to perform a complete resection. Nevertheless, postoperative irradiation was given. In conclusion, although extremely rare, esthesioneuroblastoma should be considered in the differential diagnosis of sellar lesions. References 1. Dulgeurov P, Allal AS, Calcaterra TC (2001) Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2: 683–690 2. Chirico G, Pergolizzi S, Mazzioti S et al (2003) Primary sphenoid esthesioneuroblastoma studied with MR. Clin Imaging 27: 38–40 3. Roy A, Timothy J, Anthony TR et al (2000) Aesthesioneuroblastoma arising in pituitary gland. Neuropathol Appl Neurobiol 26: 177–179
A well written and well illustrated paper describing the clinical, radiological and histological features of a Primary Sellar Esthesioneuroblastoma. This is a rare location for such a tumour. The illustrations showing MRI and histology of the tumour are clear and appropriate. Roy Weller Southampton This paper describes a case of Esthesioneuroblastoma arising from within the sellar. Although this is not a unique finding, it is certainly rare to be found isolated within the sellar region and without apparent involvement of the olfactory apparatus or pituitary gland. The paper also describes the successful removal of the tumour via a transphenoidal route. The MRI findings demonstrate the tumour well and it does indeed mimic a pituitary adenoma. Although not unique, this paper will certainly alert neurosurgeons of the possibility of this tumour in the differential diagnosis of sellar regions and that the transphenoidal resections of such a tumour in this location is feasible. On the whole, I do feel that neurosurgeons would benefit from such a well written concise case report which includes good radiological and pathological images. Jake Timothy Leeds
Correspondence: Tomislav Sajko, Department of Neurosurgery, University Hospital ‘‘Sisters of Mercy’’, Vinogradska cesta 29, HR 10000 Zagreb, Croatia. e-mail:
[email protected]
