Diagnostic and therapeutic approach to esthesioneuroblastoma: case report

Isabela Porto Silva Costa,Ivelise Theresa Araujo Balby,Gustavo Henrique Campos De Sousa,Talita Trindade França,Luis Alves De Pinho Segundo

doi:10.51723/hrj.v2i12.162

Isabela Porto Silva Costa, Ivelise Theresa Araujo Balby + Show 3 more

Open Access

https://doi.org/10.51723/hrj.v2i12.162

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Abstract

Esthesioneuroblastoma is a rare neoplasm of the nervous system that appears in the paranasal cavities derived from the olfactory neuroepithelium, located in the cribriform region of the nasal fossa, and can invade the sinuses of the face, skull base and orbital region. Kadish classification is the most widely used to stage it according to its extension. The symptomatology is unspecific, and it may present progressive unilateral nasal obstruction, epistaxis, anosmia, headache, sinusitis, diplopia, and orbital proptosis. The diagnosis is based on the lesion biopsy, immunohistochemistry, and ultrastructural findings. Excision of the tumor is described as the treatment of choice that may be associated with radiotherapy and chemotherapy. This is a case report of an olfactory esthesioneuroblastoma in a patient admitted to the hospital complaining of orbital proptosis who, after a computed tomography of the skull, showed a suspicious lesion. After the biopsy and the anatomopathological study, a protocol with neoadjuvant chemotherapy was initiated, with the subsequent excision of the tumor. Despite the lack of other symptoms, an advanced stage of the neoplasm was identified, which reflects the importance of investigating less common complaints and thus facilitating the diagnosis and early treatment.

Highlights

Esthesioneuroblastoma or olfactory neuroblastoma – a neoplasm of the sympathetic nervous system – is part of the main group of primitive neuroectodermal tumors (PNET)[1] and a rare malignant tumor that appears in the paranasal cavities of the olfactory neuroepithelium, which is located in the cribriform region of the nasal fossa[2]
The Classification of Kadish and the International TNM Classification of Malignant Tumors[9] are the most commonly used parameters to describe this tumor, there are more recent proposals such as Dulguerov's10, which is based on the extension of the tumor, Hyams[11], which is based on histological criteria, and Biller's12, which takes into account the tumor size and presence of metastasis
This led to a late diagnosis with poor prognosis for the patient because when he arrived at the service he already presented an advanced stage of neoplasia

Summary

INTRODUCTION

Esthesioneuroblastoma or olfactory neuroblastoma – a neoplasm of the sympathetic nervous system – is part of the main group of primitive neuroectodermal tumors (PNET)[1] and a rare malignant tumor that appears in the paranasal cavities of the olfactory neuroepithelium, which is located in the cribriform region of the nasal fossa[2] This tumor can invade the sinuses of the face, brain’s base and orbital region[1], and accounts for 4% to 6% of malignant sinus tumors[3,4]. On skull magnetic resonance imaging (MRI), an expansive voluminous lesion, with a lobulated contour and intense contrast enhancement was identified, measuring about 8.3 x 6.9 x 6.4 cm, the origin presumed to be from ethmoid sinuses This lesion extended through the cribriform plate, cranial cavity, nasal cavities, orbits and nasopharynx. The chemotherapy was changed from Cisplatin to Carboplatin and neoadjuvant treatment continued in order to perform a further excision of the tumor

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CONCLUSIONS