Atypical esthesioneuroblastoma invading oral cavity: a case report and review of the literature

Abstract

Esthesioneuroblastoma is an uncommon tumour of neuroectodermal origin. The authors describe a rare presentation of an atypical esthesioneuroblastoma invading oral cavity. The clinical presentation, aetiology, diagnosis, and management of this condition are discussed. The patient developed significant swelling in the right anterosuperior alveolar mucosa and had moderate tooth mobility. Conventional x-rays and computed tomography revealed a large osteolytic lesion, with imprecise limits. Histological findings along with immunohistochemical staining results and clinical features led to the diagnosis of high-grade esthesioneuroblastoma. Local recurrences and neck metastasis were detected. The rare oral findings produced delayed in diagnosis which may lead to a compromise in planning and execution of further radical management and thus a poor prognosis.

Virtual slides: The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1168853011139286.

Figure 1

Clinical and radiographic features. (A) Intraoral appearance of the neoplasm at first consultation. (B, C, D) Panoramic, occlusal and periapical radiographs revealed a radiolucent lesion extending from the upper right first molar to the upper left canine, with imprecise limits, suggesting involvement of the floor of the nasal cavity and ipsilateral maxillary sinus. Extensive bone resorption gave the appearance of floating teeth.

Figure 2

Tomographic characteristics. Axial scans (A1, B1) and coronal reformatted images (C1, D1) in bone window, and the same axial (A2, B2) and coronal (C2, D2) slice levels in contrast-enhanced soft tissue window. (A, B) The views show that the right inferior nasal concha was moderately increased in volume and tissue proliferation was observed in its anterior portion. (C, D) The nasal septum was partially destroyed. The lesion had invaded the alveolar process and soft tissue. Contrast enhancement was moderate and homogenous.

Figure 3

Histopathological and immunohistochemical findings. (A) Low-powered photomicrograph showing proliferation of islands and cords composed of small cells displaying either basaloid or fusiform morphology, a thick collagenized cellularized stroma with little inflammatory infiltrate. (B) In detail, neoplastic cells with pleomorphic and hyperchromatic nuclei. The cells in the tumour islands and cords were strongly positive for enolase (NSE), synaptophysin (SYN) and vimentin (VIM3B4). Some positive expression was also observed in the stromal cells. (Haematoxylin-eosin, original magnification x20[A], x100[B]; Immunohistochemical staining, original magnifications x40).