A rare case report of mixed olfactory neuroblastoma: Carcinoma with review of literature

Abstract

Background:Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Mixed olfactory neuroblastoma which contains areas of divergent differentiation is even rare. Till date, only 4 cases of mixed olfactory neuroblastomas have been reported.Case Description:We report the case of a 46-year-old male who presented with the chief complaints of nasal bleeding and nasal obstruction since 4 months. Radiological imaging was suggestive of a large heterogeneous mass in the left superior nasal cavity with extensions into bilateral maxillary, ethmoidal, and sphenoidal sinuses, as well as into the anterior cranial fossa. Bifrontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities and paranasal sinuses from below was done. Postoperative radiological imaging was suggestive of gross complete excision of the mass. Histopathological diagnosis was “mixed olfactory neuroblastoma-carcinoma (squamous and glandular differentiation) Hyams grade IV.” On immunohistochemistry, the tumor cells were positive for neuron specific enolase (NSE), synaptophysin, chromogranin, and CD56 and peripherally for S100. Because of personal reasons, the patient did not take adjuvant radiotherapy. He presented again after 2 months with a full blown recurrence of esthesioneuroblastoma with similar extensions as before. The patient is now planned for salvage surgery followed by adjuvant chemoradiation.Conclusion:We report the 5th case in the world of mixed olfactory neuroblastoma-carcinoma with squamous and glandular differentiation. From an analysis of the findings in the 5 reported cases of mixed olfactory neuroblastomas, one might infer that a separate subcategory of ONB, i.e., mixed ONB, should be considered because mixed ONBs have an aggressive behavior, high rates of recurrence, and these tumors should be treated aggressively by multimodality treatment.

Olfactory neuroblastoma associated with extensive “in situ” lesion and aberrant glandular and rhabdomyosarcomatous differentiation

“…[1][2][3][4][5] The basal cells or stem cells in the olfactory mucosa 2,6,7 or embryonal olfactory placode 8,9 are considered to be the origin of ONB, but pathological descriptions of the early or preinvasive phase of development or the precursor lesion of ONB are limited. 2,5 To the best of our knowledge, a full documentation of its clinicopathological features has been provided only once, by Attwood et al 10 ONB also rarely shows aberrant differentiation along epithelial 1,[10][11][12][13] and/or mesenchymal lineages, especially to rhabdomyoblasts. 14,15 We report a unique case of ONB associated with a proliferative lesion of primitive-appearing small cells in the overlying olfactory epithelium.…”

Geographic and Socioeconomic Factors on Survival in Esthesioneuroblastoma

“…Possible proposed origins are neuroendocrine cells, the neuroepithelial olfactory cells, or the olfactory epithelium. [2][3][4][5] ENB equally affects both sexes and is most common in the sixth and seventh decades of life. 1,6 ENB spreads to cervical lymph nodes in 10%-33% of cases and can invade local structures, such as the brain and orbit.…”

Radiosurgical Treatment of Recurrent Esthesioneuroblastoma: Case Report

“…Esthesioneuroblastoma is an uncommon neuroectodermal tumor occurring with an incidence of 0.4 per 1 million and constitutes approximately 2-3% of sinonasal malignancies with no gender predilection [1,2]. ENB was first described by Berger et al [3] in 1924 and since then approximately 1200 cases have been reported [3,4]. The tumor arises from basal stem cells of sensory olfactory epithelium locating on superior nasal structures, cribriform plate and anterior skull base [4,5].…”

“…ENB was first described by Berger et al [3] in 1924 and since then approximately 1200 cases have been reported [3,4]. The tumor arises from basal stem cells of sensory olfactory epithelium locating on superior nasal structures, cribriform plate and anterior skull base [4,5]. The most common symptoms are nasal congestion and epistaxis, anosmia, rhinorrhea or lacrimation [6].…”

“…Therefore the primary treatment modality is often a craniofacial resection, adjuvant external beam radiotherapy and chemotherapy [7]. Despite combined treatment the risk of local recurrence is about 20-50% [4][5][6][7]. Appliance of stereotactic radiosurgery (SRS) as a non-invasive treatment modality can be safe and effective in group of patients with local recurrence of ENB after surgery, especially in cases of deep located lesions.…”