NMC Case Report Journal 2015; 2: 57–60 DOI: 10.2176/nmccrj.2014-0091 Case Report Primary Sellar Neuroblastoma in an Elderly Patient: Case Report Shun Yamamuro, Takao Fukushima, Atsuo Yoshino, Kazunari Yachi, Akiyoshi Ogino, and Yoichi Katayama A 71-year-old male presented with an isolated well- Case Report enhanced sellar lesion accompanied by hypopituitarism, A 71-year-old male was admitted to another hospital com- diagnosed preoperatively as a pituitary adenoma, plaining of general fatigue. Laboratory evaluations demon- meningioma, or metastatic brain tumor. However, histo- strated hyponatremia. A computed tomography (CT) scan logical examinations yielded a diagnosis of neuroblas- disclosed a sellar mass lesion. Under a diagnosis of pituitary toma. Primary sellar neuroblastoma in the elderly is very rare. We therefore describe this case of primary sellar tumor, the patient was referred to our hospital. His conscious- neuroblastoma, mimicking common pituitary tumor, ness level was clear and neurological examinations revealed and review the literature. There have so far been only no abnormalities except for bitemporal hemianopsia and right nine reported cases of primary sellar neuroblastoma in oculomotor nerve palsy. Magnetic resonance (MR) imaging the English literature. All reports like the present case, demonstrated a sellar mass with suprasellar extension and demonstrated similar neuroimaging of a “dumbbell- bilateral cavernous sinus invasion. The lesion was isointense shaped extension in the sellar region.” Moreover, the on T1-weighted and T2-weighted MR images; slightly hetero- tumors may exhibit characteristic features, such as rapid geneous enhancement was evident after contrast medium tumor growth, hypopituitarism, or oculomotor nerve administration (Fig. 1). Laboratory and endocrinological palsy, and these findings may represent helpful signs for evaluations revealed hyponatremia and panhypopituitarism the diagnosis of primary sellar neuroblastoma. (Table 1). Hormone replacement therapy was initiated imme- Keywords: elderly case, magnetic resonance imaging, diately after admission. The plasma sodium became normal- neuroblastoma, pituitary tumor, sellar region ized on the eighth day of hospitalization. The patient underwent surgical removal via an endoscopic transsphenoidal approach on the fifteenth day of hospitaliza- Introduction tion. The intraoperative findings indicated that no contact Neuroblastoma arising in the sellar region is extremely existed between the tumor and the floor of the sella turcica rare, with only nine cases having been reported so far in the or bone of the paranasal sinuses. It proved very difficult to English literature.1–9) A limited analysis of the pathogenesis remove the tumor, because it was rich in fiber components, has been given, and no therapeutic strategy has yet been and bled easily. Partial removal of the tumor was therefore established. carried out, excluding the suprasellar part and bilateral cav- We report a case of primary sellar neuroblastoma, which ernous sinus invasion. was first diagnosed as pituitary adenoma. By reviewing the Pathological examinations of the tumor specimen showed literature, we identified characteristic findings among the that the tumor tissue was composed of small blue round cells neuroimaging and clinical symptoms for differentiating arranged in a lobulated or “zellballen” pattern surrounded by sellar neuroblastoma. a vascular connective tissue (Fig. 2a). The tumor cells were Fig. 1 Preoperative magnetic reso- nance (MR) imaging demonstrating a dumbbell-shaped form pituitary tumor with suprasellar extension and invasion of the cavernous sinus. There was het- erogeneous enhancement after contrast medium administration (a: axial view, b: a b c sagittal view, c: coronal view). Department of Neurological Surgery, Nihon University School of Medicine, Tokyo Received: March 8, 2014; Accepted: July 3, 2014 57 S. Yamamuro et al. Table 1 Laboratory data at the time of hospitalization (Normal values) Plasma sodium 109 mEq/l (136–148 mEq/l) Plasma potassium 4.7 mEq/l (3.6–5.0 mEq/l) Plasma chloride 77 mEq/l (98–109 mEq/l) Thyroid stimulating hormone 0.12 µIU/ml (0.34–3.80 µIU/ml) a b Free triiodothyronine 1.45 pg/ml (2.00–3.80 pg/ml) Free thyroxine 1.04 pg/ml (0.8–1.5 pg/ml) Adrenocorticotropic hormone 3.0 pg/ml (7.2–63.3 pg/ml) Prolactin 8.65 ng/ml (3.6–16.3 ng/ml) The laboratory and endocrinological evaluations revealed hyponatremia and panhypopituitarism, at the time of hospitalization. c d e f a b Fig. 2 Photomicrographs obtained by hematoxylin and eosin (H&E) staining. Histological examinations of the tumor revealed that it was composed of small cells with oval-round hyperchromatic nucleoli, accompanied by a surrounding vascular connective tissue (a: original magnification ×100, b: original magnification ×400). g h round with oval-round hyperchromatic nucleoli and a sparse chromophilic, slightly acidophilic cytoplasm (Fig. 2b). Mod- Fig. 3 Photomicrographs of immunohistochemical staining. The erate cellular and nuclear pleomorphism and occasional tumor cells showed positive expression for S-100 protein (a), synapto- physin (b), and neurofilament (c), and diffuse positivity for Ki-67 mitoses were observed in some areas. Immunohistochemi- (MIB-1 labeling index: 12.6%, d); in the same time, the neoplasm was cally, the tumor cells exhibited positive expression for S-100 immunonegative for adrenocorticotropic hormone (e), prolactin (f), epi- protein, neuron-specific enolase, neurofilament, chromo- thelial membrane antigen (g), and glial fibrillary acidic protein (h). granin A, and synaptophysin (Fig. 3a–d), whereas they were Original magnification ×400. immunonegative for epithelial membrane antigen, glial fibrillary acidic protein, and pituitary hormones such as pro- tumor with the olfactory bulbs or ethmoidal aircells. These lactin and adrenocorticotropic hormone (Fig. 3e–h). There operative findings together with the neuroimaging data could was diffuse positivity for Ki-67; the MIB-1 labeling index for exclude a diagnosis of esthesioneuroblastoma growing into the the proliferative activity was 12.6%. Immunostaining for sella. Postoperative MR imaging indicated that the sellar and TP53 was partially positive, although most cells were nega- suprasellar parts of the tumor were mostly removed (Fig. 4a–c). tive. These pathological findings were consistent with Since tumor remained in the cavernous sinus, the patient neuroblastoma. underwent gamma-knife radiosurgery with a marginal isodose The patient was discharged from our hospital on the fif- of 14 Gy, at 28 days after the second operation. Gadolinium teenth day after surgery, and his postoperative course was scintigraphy at 7 months after the gamma-knife radiosurgery uneventful. His oculomotor nerve palsy was improved, and revealed no other region with primary tumor or metastatic visual field deficit underwent slight improvement after the lesions. At 18 months after the second operation, there was no operation, but his panhypopituitarism and bitemporal hemi- apparent evidence of tumor recurrence (Fig. 4d–f). anopsia persisted after the surgery. Laboratory examinations yielded a normal result for urinary vanillylmandelic acid Discussion (VMA): 3.1 mg/day (1.5–4.3 mg/day). A CT scan of the whole Cerebral neuroblastoma, a rare malignant tumor that body revealed no other region with primary tumor or meta- commonly occurs in the cerebral hemisphere or paraventri- static lesions. A second operation was performed at 3 months clar region of children,10,11) belongs to a group of central ner- after the first operation to remove the suprasellar part of the vous system primitive neuroectodermal tumors (CNS tumor via a bi-frontal craniotomy. There was no contact of the PNETs) which represent embryonal tumors composed of 58 Sellar Neuroblastoma in an Elderly Case undifferentiated or poorly differentiated neuroepithelial cells. Primary sellar neuroblastoma is extremely rare. There have so far been only nine reported cases in the English literature (Table 2). Although the origin of primary sellar neuroblastoma remains unknown, several theories have been put forward in previous reports. Lin et al. suggested that diffusely scattered a b c cells of the nervous terminalis, or olfactory placode neu- ronal cells which blocked migration, might represent the origin of the tumor.2) Furthermore, there have been two additional suggestions for the origin of the tumor:neuronal transformation from the pituitary epithelium, and a ganglion of Locy which grows between the olfactory fossa and the telencephalic vesicle.1,4,6,8,9) Oyama et al. reported the case of a 33-year-old male with sellar neuroblastoma who did not d e f display an elevated urinary VMA. In this case, they sug- gested that the primary tumor could have arisen in the Fig. 4 Second postoperative magnetic resonance (MR) imaging. The supradiaphragmatic region and extended into the sellar tur- sellar and suprasellar part of the tumor were mostly removed (a: axial cica, since catecholamine metabolites in the urine are usu- view, b: sagittal view, c: coronal view). MR imaging was performed 1 year after gamma-knife radiosurgery. The tumor parts in the bilateral ally elevated in peripheral neuroblastoma but not within cavernous sinus had become shrunken and there was no recurrence of tumor of the blood-brain barrier: primary cerebral the tumor (d: axial view, e: sagittal view, f: coronal view). neuroblastoma.4) Table 2 Cases of primary sellar neuroblastoma Neuroimaging Authors Age