International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 153–155

Case report

Primary paranasal sinus meningioma of a 1.7-year-old boy

Mu Xian, Bing Zhou *

Beijing Tong Ren Hospital, Capital Medical University, Key Laboratory of Otolaryngology Head and Neck Surgery (Capital Medical University), Ministry of Education, China

A R T I C L E I N F O

Article history:

Received 16 May 2010

Accepted 19 June 2010

Available online 18 July 2010

Keywords:

Ectopic meningioma

Paranasal sinus neoplasm

Endoscope

Child

A B S T R A C T

A 1.7-year-old boy was admitted to our hospital with the first impression of ossified fibroma in the right

ethmoid sinus. However, histopathological examination of the surgical specimen turned out to be

meningioma. The mass was completely removed endoscopically assisted by image-guided navigation

system. This is the youngest ectopic meningioma case reported. The clinic figure and treatment of

primary paranasal sinus meningioma is briefly discussed. Considering the young age and small blood

volume of this patient, careful preoperative preparation and the cooperation of multi disciplines were of

vital importance.

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International Journal of Pediatric OtorhinolaryngologyExtra

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1. Introduction

Meningioma of the paranasal sinus is an uncommon tumor. Thefirst description of paranasal sinus meningioma was by Shaheen[1] in 1931. Here we report a 1.7-year-old boy with a primaryectopic meningioma in the right ethmoid sinus. This is theyoungest patient of all reported primary meningioma cases of thesinonasal tract [2–4] ranging from 4 to 88 years old.

Treatment of paranasal sinus meningiomas is similar to othertumors originating in the sinuses and is, most commonly, surgical[5]. The surgical approach is chosen depending on the location, thesinus involved and the extensions of the tumor. Frontal cranioto-my, rhinotomy and endoscopic surgery are commonly used.However, the surgery of paranasal sinus meningioma for youngchildren was rarely reported in the literature. This report presentsa case that the tumor was successfully removed endoscopicallyassisted by image-guided navigation system.

2. Case report

A 1.7-year-old boy was admitted to our hospital with ahistory of persistent nasal obstruction and intermittent epistaxisof the right side for 5 months. Decongestant treatment forrhinitis failed.

On endoscopic examination, there was a pink, hard mass withsmooth surface occupying the right nasal cavity. The massextended to the concha and septum occluding deep structures.

* Corresponding author at: No. 1 Dong Jiao Min Xiang, Dong Cheng District,

Beijing 100730, China. Tel.: +86 10 58269206; fax: +86 10 85115988.

E-mail address: entzhou@263.net (B. Zhou).

1871-4048/$ – see front matter � 2010 Published by Elsevier Ireland Ltd.

doi:10.1016/j.pedex.2010.06.003

CT scan showed an oval mass lesion of 3.79 cm � 2.22 cm with acomplete bony boundary and mixed density in the right ethmoidsinus (Fig. 1). The mass expanded into the right orbital area, leftethmoid sinus and both nasal cavity. The boundary between thelesion and right lamina papyracea was obscure. The mass was firstconsidered as ossified fibroma.

Image-guided endoscopic nasal surgery was performed. Themass filling the right ethmoid sinus and fusing the middleturbinate was completely removed endoscopically with itscapsule. In order to dissect the tumor thoroughly, it is quiteimportant to identify and cut along the edge. The image-guidednavigation system allowed for the identification of the true edgeof the tumor as well as adjacent vital structures including laminapapyracea, cribriform plate etc. during the procedure. Theposterior one-third of the lamina papyracea fusing with themass was drilled with diamond bur and then dissected. Theperiorbital fascia was intact. The biopsy was cellular, spongy bonelike lesion with savage bleeding. Three nutrient arteries werefound, followed, and identified as main branches of ethmoidartery. About 1500 ml bleeding was replenished with 800 mlblood transfusion. The middle concha was retracted laterally tosupport the orbit. Considering the difficulty of redressing for sucha young child, bio-degradable fragmentable foam, Nasopore, wasdressed.

Histopathological examination of the surgical specimenshowed meningioma. Microscopically, the tumor was proliferativediffusely in propria lamina under the lamina epithelialis, whichwas composed of a mixed population of immature bone trabeculasurrounded by abundant polygonal to spindled tumor cells (Fig. 2).Tumor exhibited a meningotheliomatous pattern (Fig. 3), charac-terized by lobules of cell with whorl formation, indistinct cellborders, and bland nuclei with delicate chromatin. The nuclei were

Fig. 1. (A) axial CT and (B) coronal preoperative CT image.

Fig. 3. The tumor exhibited a meningotheliomatous pattern (HE 400�).

Fig. 4. Immunohistochemical staining for vimentin was positive (IHC 400�).

M. Xian, B. Zhou / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 153–155154

ovoid, with uniform size and shape. Nuclear chromatin was blandwith inconspicuous nucleoli. Typical intranuclear pseudoinclu-sions and psammoma body was absent. Immunohistochemically,the tumor cells expressed vimentin (Fig. 4), and EMA (Fig. 5).

The patient was transferred to IUC for 1 day postoperatively.The tampon was removed 2 days after the operation. Thepostoperative course was uneventful. Before the patient wasdischarged on the 4th postoperative day, another CT scan wasperformed. The mass was completely removed (Fig. 6).

At the first follow up 6 months after the surgery, no sign ofrecurrence or inflammation was found.

Fig. 2. The tumor was composed of a mixed population of immature bone trabecula

surrounded by abundant polygonal to spindled tumor cells (HE 100�).

Fig. 5. Immunohistochemical staining for EMA was positive (IHC 200�).

3. Discussion

Although increasing evidence supports the development ofectopic meningiomas from ectopic arachnoid cell clusters, the ageof primary sinonasal meningiomas averaged 42.4 years [2]. The

Fig. 6. Postoperative CT scan.

M. Xian, B. Zhou / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 153–155 155

reluctance of performing CT examination on young children andslow development of the tumor might partially explain thediscrepancy.

Daneshi et al. [6] described three criteria to differentiateparanasal sinus meningiomas from metastatic meningioma orthose extending from intracranial tumors: (1) the bony wall of thesinus is intact on radiological imaging or on inspection duringsurgery. (2) The absence of a simultaneous intracranial meningio-ma on imaging or at surgery. (3) Expansion of the sinus walltowards the cranium rather than to the opposite direction. Thepresent case met all these criteria.

The radiographic result of ectopic meningioma was lessspecific, but nasal cavity or paranasal sinus opacification by amass lesion, accompanied by bony erosion and sclerosis orhyperostosis [7,8]. The tumor might expand into the base of theskull and/or the orbit. If contrast-enhanced CT scan wereperformed, there would be intense enhancement of meningiomasin consistent with the abundant blood supply. In the present case,because of the egg shell like expression of the neoplasm (Fig. 1),the preoperative CT is very confusing with osteofibroma. Thedifferential diagnoses of paranasal sinus meningiomas includeossifying fibroma, osteofibrous dysplasia, sinus mucocoele ormucopyelocoele, follicular dendritic cell tumor/sarcoma, osteo-blasoma and low-grade osteosarcoma [3,4]. The diagnose can onlybe made upon pathology examination.

Like their intracranial counterparts, sinonasal tract meningio-mas may exhibit a variety of different histologic patterns [2].However, most cases were typical meningotheliomatous menin-giomas composed of lobules of cells with indistinct borders andpossessing generally bland nuclei with delicate chromatin. Theimmunohistochemical profile of sinonasal tract meningiomas wasindistinguishable from intracranial lesions, with all tumors testedexpressing epithelial membrane antigen and vimentin immuno-reactivity.

The aim of the surgery is total excision. Where this is notpossible and for recurrent tumors, radiotherapy can be considered.In the present case, the image-guided navigation system helped todissect the tumor thoroughly and safely. The image-guidednavigation system assisted the identification of the true edge ofthe tumor as well as adjacent structures including laminapapyracea, cribriform plate etc. As for young patients, thoughtfulpreoperative preparation and cooperation with multi disciplineswere of vital importance. In this case, 800 ml packed red blood cellwas prepared considering the risk of life threatening blood loss.Pediatric, anesthetic, and ICU specialties were consulted preoper-atively. While the difficulty of endoscopic redressing for such asmall patient must also be considered, a bio-degradable fragmen-table foam, Nasopore, was dressed.

In general, the prognosis of primary meningioma of the sinonasaltract appears to be excellent [9]. There was little difference betweenthe 5-year and 10-year disease-free survival rates, indicating thatonce the patients survived disease-free for 5 years, they wereunlikely to die with tumor [2]. When recurrences developed, theyusually arise in the same anatomic site as the primary lesion andprobably represent residual disease rather than recurrent tumor.Additional surgery, if clinically feasible, is advisable, becauseradiation therapy does not always result in a clinical response[10]. If death with tumor does result, it is usually the result ofinvolvement of the vital structures of the midfacial region orcomplications of the surgery rather than the aggressive nature of thetumor.

4. Conclusion

Although rare, primary paranasal sinus meningioma does occurin very young children. The symptom, sign and CT examination arenot specific comparing with other paranasal mass. The diagnosecan only be made based on pathology result. Because of the benignbehavior of the tumor, the treatment may be difficult but worthy.Thoughtful preoperative preparation is of vital importanceespecially for the very young children.

References

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[9] K.L. Ho, Primary meningioma of the nasal cavity and paranasal sinuses, Cancer 46(1980) 1442–1447.

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