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 slidesThe virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1168853011139286.
Highlights
Esthesioneuroblastoma (ENB) is a rare and complex tumour, representing the most undifferentiated end of the spectrum of neuroendocrine tumours
ENB is characterized by slow progression and locally aggressive behaviour, which lead to long-term survival but very frequent late local recurrence
The aggressiveness of ENBs is partly due to their complex anatomical location, close to vital structures, which is associated with non-specific symptoms that lead to delay in the patient seeking diagnosis [1,5,6]
Summary
Esthesioneuroblastoma (ENB) is a rare and complex tumour, representing the most undifferentiated end of the spectrum of neuroendocrine tumours. This study highlights the characteristics of clinical, histopathological and immunohistochemical features of ENB showing unusual oral involvement and its importance in the differential diagnosis of sinonasal neuroendocrine malignancies and maxillary neoplastic lesions. Conventional x-rays and computed tomography (CT) revealed osteolytic lesion with imprecise limits (± 3.0 cm) determining resorption of alveolar process and palatal bone, soft tissue invasion and floating teeth features. It extended into the nasal cavity, with partial destruction of the nasal septum and inferior nasal turbinate (Figures 1B-D and 2). The histological similarities to malignant small round cell tumours (e.g. neuroendocrine carcinoma) and basaloid squamous cell carcinoma required the use of a broad immunohistochemical panel as an adjuvant technique in the differential diagnosis. The patient refused further surgery and palliative chemotherapy was initiated
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