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International Archives of Otorhinolaryngology

Print version ISSN 1809-9777On-line version ISSN 1809-4864

Int. Arch. Otorhinolaryngol. vol.19 no.1 São Paulo Jan./Mar. 2015

http://dx.doi.org/10.1055/s-0033-1353368 

Case Reports

Isolated Schwannoma of the Olfactory Groove: A Case Report

Luiz Augusto Nascimento 1  

Flávio Aurélio Parente Settanni 1  

José Franscisco de Góis Filho 2  

Isabela Naria Dias Sanchez 3  

Bruna Barros Cavalcante 4  

João Noberto Stávale 5  

1Division of Skull Base Surgery, Department of Otolaryngology-Head and Neck Surgery, Universidade Federal de São Paulo, São Paulo/SP, Brazil

2Department of Head and Neck Surgery, Instituto do Cancêr Arnaldo Vieira de Carvalho, São Paulo/SP, Brazil

3Department of Oncology, Universidade Federal de São Paulo, São Paulo/SP, Brazil

4Department of Head and Neck Surgery, Universidade de Brasília, Brasília/DF, Brazil

5Department of Pathology, Universidade Federal de São Paulo, São Paulo/SP, Brazil


ABSTRACT

Introduction

Schwannoma of the olfactory groove is an extremely rare tumor that can share a differential diagnosis with meningioma or neuroblastoma.

Objectives

The authors present a case of giant schwannoma involving the anterior cranial fossa and ethmoid sinuses.

Case Report

The patient presented with a 30-month history of left nasal obstruction, anosmia, and sporadic ipsilateral bleeding. Computed tomography of the paranasal sinuses revealed expansive lesion on the left nasal cavity extending to nasopharynx up to ethmoid and sphenoid sinuses bilaterally with intraorbital and parasellar extension to the skull base. Magnetic resonance imaging scan confirmed the expansive tumor without dural penetration. Biopsy revealed no evidence of malignancy and probable neural cell. Bifrontal craniotomy was performed combined with lateral rhinotomy (Weber-Ferguson approach), and the lesion was totally removed. The tumor measured 8.0 4.3 3.7 cm and microscopically appeared as a schwannoma composed of interwoven bundles of elongated cells (Antoni A regions)mixed with less cellular regions (Antoni B). Immunohistochemical study stained intensively for vimentin and S-100.

Conclusion

Schwannomas of the olfactory groove are extremely rare, and the findings of origin of this tumor is still uncertain but recent studies point most probably to the meningeal branches of trigeminal nerve or anterior ethmoidal nerves.

Key words: paranasal sinuses; skull base; neurilemmoma; olfactory nerve; head and neck neoplasms; neoplasms; nerve tissue

Introduction

Schwannoma of the olfactory groove is extremely rare. So far only 35 cases have been reported in the literature when we searched the PubMed database. Schwannoma had already been described at the beginning of the 19th century, but Virchow was the first to recognize them for what they were. Such tumors account for ∼8% of all primary intracranial tumors.1 The most common arises from the vestibular portion of the eighth nerve and, less commonly, the fifth, ninth, tenth, and twelfth cranial nerves.123456 They have been described as resembling subfrontal or olfactory groove schwannomas, which reflects their enigmatic origin.12345678910111213141516171819202122232425

Case Report

The patient was a 39-year-old man with 30-month history of left nasal obstruction, anosmia, and sporadic ipsilateral bleeding. His physical examination showed a mass in the left nasal cavity extending to the nasopharynx. No café au lait spots or other signs of von Recklinghausen neurofibromatosis were observed.

Computed tomography of paranasal sinuses revealed expansive lesion on the left nasal cavity extending from the nasopharynx to ethmoid and sphenoid sinuses bilaterally with intraorbital and parasellar extension to the skull base. T2-weighted magnetic resonance imaging confirmed a heterogeneous hyperintense tumor occupying the anterior cranial fossa without dural penetration (Fig. 1). Biopsy by nasal endoscopy revealed no evidence of malignancy and probable neural cell. Cerebral angiography showed a large tumoral blush with pathologic vascularization of the displaced left maxillary artery and left ophthalmic artery without sufficient diameter for embolization.

Fig. 1  Coronal magnetic resonance imaging showing extensive mass growing down from cribriform plate. 

The bilateral supraorbital approach was applied by making a coronal skin incision and retracting the scalp flap forward, and bilateral frontal craniotomy was performed with the John-Jane technique for excellent access to the floor of the anterior fossa and the superior orbits with less brain retraction. The orbital roof was partially removed and the dura was elevated on both sides of the crista galli. The tumor was seen arising from the base of the anterior cranial fossa and attached to the dura at level of the cribriform plate up to anterior clinoid process.

The lesion was well delineated, involving the anterior cranial fossa and parasellar region and extending down to the nasal cavity, nasopharynx, and ethmoid sinuses with intraorbital parasellar extension. The tumor was completely excised through the bifrontal craniotomy approach combined with lateral rhinotomy (Weber-Ferguson approach). The tumor measured 8.0 × 4.3 × 3.7 cm and was firm in consistency. Microscopically, the tumor appeared as a Schwannoma composed of interwoven bundles of elongated cells (Antoni A regions) mixed with less cellular regions (Antoni B). Immunohistochemical study stained intensively for vimentin and S-100 (Fig. 2). The resected area of the dura and tumor was repaired with part of the temporal bone and galea–periosteum aiming to minimize the possibility of fistula and infection. The patient remains free of disease with an excellent functional and cosmetic result. He is in good health with a residual sense of smell.

Fig. 2  Neopasm consisting of elongated spindle cells with poorly defined, palely eosinophil cytoplasm, sometime with nuclear palisading. Immunohistochemically, glial fibrillary acidic protein (GFAP) was negative and vimentin and S-100 protein stained intensively. 

Discussion

Isolated schwannoma of the olfactory groove are rare tumors.131435 According to a review by Ulrich et al in 1978,36 three of the previous cases had been reported in association with von Recklinghausen neurofibromatosis. Husain et al reported a case of a tumor arising from the base of the right anterior cranial fossa,13 and Sabel and Teepen referred to a case of tumor of the anterior cranial fossa where the olfactory bulb and nerve were not seen.14

There are a few descriptions of intracerebral schwannomas.2341737383940 To date there are 35 cases of schwannoma of the anterior cranial fossa described in the literature.741

The origin of intracranial Schwannoma is intriguing. There are some hypotheses concerning their origin. Developmental theories hold that these lesions primarily arise from aberrant Schwann cells in the central nervous system.14151733 Russell and Rubinstein advocate that mesenchymal pial cells may transform into ectodermal Schwann cells, explaining the occurrence of intraparenchymal schwannomas. Other theories suggest an origin from multipotent mesenchymal cells or displacement of neural crest cells forming the focus of Schwann cells within brain parenchyma (schwannosis).35

The developmental theories can explain the origin of intraparenchymal schwannomas,14151833 but the subtype of schwannomas located in the anterior skull base intimately related to the olfactory groove seems to be an extra-axial tumor.153133

Adachi et al reviewed the literature in 2007 about the origin of schwannoma from the fila olfactoria.15 They reported that embryonic nerve, the plexus of dural vessels, and the fila olfactory that acquire Schwann cells have been considered as possible origins of these tumors.1519 However, others authors have pointed that the olfactory schwannomas originate from the olfactory bulb and nerve.8141833 A kind of cell that expresses phenotypic features of both astrocyte and Schwann cell is present.19

To assess the possibility of schwannomas originating from a real peripheral nerve, we need to know which nerve, present near the olfactory bulb and first cranial nerve, could be the origin of the tumor growth. The anterior ethmoidal nerve and the ramus meningeus of the trigeminal nerve are possibilities131442 In most vertebrates, including man, a terminal nerve, which is part of an accessory olfactory system, is thought to be present. It is possible that this nerve is the source of an olfactory schwannoma.

The descriptions of the origin of this tumor are intriguing and enigmatic. Most descriptions point to a lesion intimately related to the anterior skull base, particularly the region of the olfactory groove.

Conclusion

Schwannomas represent ∼8% of all intracranial tumors. Schwannomas of the olfactory groove are extremely rare. The present case represents the 36th case described in the literature. The origin of this tumor is still uncertain but recent studies point most probably to the meningeal branches of trigeminal nerve or anterior ethmoidal nerves.

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Received: February 06, 2013; Accepted: July 01, 2013

Address for correspondence Luiz Augusto Nascimento, MD, PhD, R. Estado de Israel, 493, apto 184, São Paulo/SP, CEP: 04022001, Brazil (e-mail: luizaugusto.hns@gmail.com).

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